January 26th - LBJ - Nephrotic Syndrome

Today at LBJ, a case of nephrotic syndrome was presented.  Although the exact diagnosis for this patient is not known (biopsy results pending), for boards you should know the basic causes of nephrotic syndrome - as well as further work-up and treatment










Lab findings/Diagnosis: The classic necessary finding in nephrotic syndrome is proteinuria (usually at least 3+ on urine dipstick, but a 24-hr urine protein of >3grams or a spot Urine Protein/Cr ratio >3 is more specific). Patients will also have hypoalbuminemia, dyslipidemia (LDL and TG), HTN and hypogammaglobulinemia. There is usually not as much inflammation as in patients with nephrotic syndromes, with some exceptions - but in most patients there will not be hematuria or dysmorphic RBCs/RBC casts.  While pending biopsy, serologic workup can be performed (you'll see why below) including HIV, ANA, anti-DS DNA, Complement Levels, RPR, Hepatitis Panel and Serum Protein/Urine Protein Electrophoresis with IF. Remember to get a good review for all OTC medications taken as well. Workup in most adult patients will require a biopsy for ultimate diagnosis unless the cause is obvious (i.e. diabetic). 


Clinical:  Patients can present with HTN, Foamy Urine, Edema (starts in dependant areas - such as periorbital, scrotum and legs - but patients can eventually get anasarca/pleural effusion/ascites), infections (due to low gamma globulins), and thrombosis (Lower extremity, pulmonary embolism and renal ven thrombosis due to loss of AT-3).  How does renal vein thrombosis present? -Acute abdominal pain + sterile pyuria should clue you in to this entity


Like the causes of glomerulonephritis, one can think of causes of nephrotic syndrome in two categories: Those that are systemic diseases, and those that cause more specific changes on biopsy


The systemic diseases that cause nephrotic syndrome:
1) Diabetes Mellitus - the most common!  Remember the A1c can be normal in those patients that have developed some element of renal insufficiency.  These patients usually do not need a renal biopsy to diagnose, and this is usually a clinical diagnosis.  Patients should be placed on an ARB/ACEI and a low protein diet.  Patients that do not have good glycemic control, uncontrolled HTN, and keep spilling protein will get progressive decrease in GFR. This is usually not too rapid, and occurs over a number of years. 


2) SLE - Can also present with a more "nephritic" component. Remember, SLE can affect the kidney in a variety of ways...


3) Multiple Myeloma - Patients will present clinically with: high globulin gap, bone pain, anemia, renal failure, hypocalcemia.  They might not have frank nephrotic syndrome on presentation, and might just has +1 protein in their urine. Still, a work-up for this must be done for patients with nephrotic syndrome (SPEP/UPEP with IF)


4) Amyloidosis - usually caused by light chain deposition, or from AL forms.  On the boards, these patients will have other clues/findings (such as restrictive heart failure, neuropathy, etc)


The Renal "Causes" of nephrotic syndrome
1) Minimal Change Disease - In adults, the causes can be Idiopathic or caused by NSAIDS or  Hodgkin's Lymphoma.  Biopsy shows "effacement of foot processes" Treat with steroids +/-  cytotoxic agents (such as cyclophosphamide) 


2) Membranous Nephropathy - There are MULTIPLE causes for this on biopsy. 
    Drugs: NSAIDS, Gold
    Autoimmune Disease: SLE
    Cancer: Solid tumors, especially HCC, RCC, Colon and Lung
    Infections (HCV, HBV, HIV, Malaria)
       These patients usually have the underlying condition treated/removed first, and if
     severe disease, get  steroids +/- cytotoxic agents


3) Focal Segmental Glomerulosclerosis - has MULTIPLE causes as well
     Idopathic (esp with h/o HTN), Sickle Cell Disease, Heroin use, Obesity.
     Steroids are usually given to treat, but most end up needing Dialysis


Overall, patients with nephrotic syndrome should be placed on ACEI/ARB and diuretics as needed.  Good BP control is essential.  Many patients need to be on medications for dyslipidemia.  A word about anticoagulation - these patients are "hypercoaguable", so do they all need treatment?  Usually when inpatient - only DVT prophylaxis is given.  Life-long treatment is usually given to patients with one thrombotic event. Prophylactic treatment to prevent an occurance of a thrombosis is not usually done, except in some cases of membranous nephropathy.

January 25th - MHH - Scleroderma

At MHH, a case of CREST Syndrome was presented - here is a brief overview of scleroderma - subtypes, lab findings, and management. 

For internal medicine boards, you should be familiar with the systemic forms of scleroderma (as opposed to those which only dermatologists treat)




The first is Limited Systemic Sclerosis (used to be called "CREST" syndrome - which is a good way to remember the salient features)

These patients will have findings that go along with the CREST letters - Calcinosis, Raynaud's phenomenon, Esophageal Dysmotility, Sclerodactyly and Telangiectasias.  These patients usually only have skin changes in their extremities distal to the wrist, and mostly in the upper extremities.

Other findings and complications in these patients include:
-Hyper/hypo pigmentation of skin
-Tight skin around face/paucity of wrinkles/"mouse facies"
-Digital ulcerations and ischemia
-Arthritis
-Lung disease (pulmonary HTN)
-"Watermelon Stomach"/Gastric vascular antral ectasia and GI Bleeding. Bacterial overgrowth and malabsorbtion can occur as well

Lab findings: ANA (>90%)and  anti-centromere staining pattern of ANA (50%)

Treatment:
Raynaud's: Warming of fingers, calcium channel blockers
Digital ulcers: warming of extremities, aspirin, topical nitrates and prostacyclins
Esophageal Dysmotility: Almost all patients are on PPIs. May need Feeding tube placed
Pulmonary HTN: Supplemental Oxygen, Calcium channel blockers, bosenten, prostacyclins

Other category is Diffuse Systemic Sclerosis:

Clinically these patients have skin involvement more proximal to the wrist, and also have involvement of the torso/face.  Although these patients will have some overlap features of the CREST syndrome (especially telangiectasias, "mouse facies" and Raynaud's), in general organ invovlement is more prevalent:

MS: Abnormal nailfold capillaries, arthritis may be present, tendon friction rubs,
        myositis and myalgias
Lungs: Patients can develop interstitial lung disease and pneumonitis
Renal: Scleroderma Renal Crisis (HTN, Renal Failure and hemolytic anemia)
GI: Malabsorbtion, bacterial overgrowth and primary biliary cirrhosis
CV: Fibrosis, Myocarditis, CHF, Pericardial Effusions

Labs: ANA (>90%) and scl-70 (30%)

Treatment:  Patients get treated for systemic sclerosis with steroids when organs are affected (i.e. Heart and Lung).  Cyclophosphamide is used for intersititial lung disease, and other cytotoxic agents are added with heart involvement.   Scleroderma renal crisis is treated with ACEIs.  Arthritis is usually treated with NSAIDS and Acetaminophen.  Remember, steroids are not given to all patients - only when there is some element of systemic involvement, or myositis.

January 22nd - MHH - Lung Cancer

Today at morning report, a 47 year old AAF with significant tobacco use presented with 2 months of worsening dyspnea on exertion.  Although the final pathology was not back, it is likely small-cell lung cancer.

On the boards, the info you'll need to know for Lung Cancer is broad, so here is a quick review.  I would be familiar with staging - although the boards will not likely ask you to stage, they may ask a questions regarding treatment modality. To know this, you need to know the stage.




Risk Factors: Smoking (increases risk about 15x normal. Even 2nd hand smoke exposure increases risk about 2x), asbestos exposure, other heavy metal exposure

Clinical Presentation:  Over 90% of patients with a diagnosis of lung cancer have some sort of symptoms - either from local involvement, paraneoplastic syndromes or metastatic disease.  Local symptoms depend on the location of the lesion.  So the more "central" cancers (i.e. small cell and squamous cell) present with bronchial obstruction, pneumonia, cough, hemoptysis and dyspnea.  The more "peripheral" cancers can present with pleurisy, chest wall pain and pleural effusion. Obviously either can cause dyspnea as well as cough/hemoptysis, and there is some overlap.  Think of lung cancer in a patient with a history of COPD that presents with an "exacerbation" that is different than their usual exacerbation (i.e. more severe, or lasting longer). Also consider with new onset COPD symptoms in an older patient.  Other signs/symptoms include change in voice, Horner's Syndrome, arm paresthesias, SVC syndrome, clubbing, temporal wasting and lymph node enlargement.  Metastatic disease can present with bony pain, headache/AMS, or hepatomegaly.  We'll go over paraneoplastic syndromes in a bit as well.

Diagnosis:  CXR for initial suspicion, followed by CT Scan.  You ideally want to biopsy any lymph nodes first, but can also biopsy the mass.  Bronchoscopy can be used for central lesions that are >4cm.  Open biopsy or IR-guided biopsy if the lesion is peripheral or small.

Classification/Pathology and Subtypes:
Small-Cell Carcinoma
Non-Small Cell Subtypes (includes squamous cell, large cell and adenocarcinoma)
Others: carcinoid and rare pathology (carcinoid is reviewed in one of my earlier posts)

Small Cell Carcinoma
Usually metastasize early, as compared to the non-small cell types. Located more "central" Paraneoplastic syndromes include SIADH, Eaton-Lambert and ectopic ACTH production. 

Staging for Small Cell:
Limited: Can fit within one radiation therapy Port (Confined to one Lymph Node, the Mediastinum and A Supraclavicular LN)

Extended: If it does not fit into one port

Treatment for Small Cell:
Limited: Cisplatin + Etoposide with Radiation

Extensive:  Cisplatin + Etoposide or Carboplatin + Etoposide
If Brain Metastases are Present - Radiation and Steroids (Some give prophylactic cranial irradiation for both stages, although it is more controversial for Limited-Stage Disease)

Non-Small Cell Subtypes:
Squamous Cell - Most likely to cavitate.  Central Lesion. Hypercalcemia
Large Cell - Peripheral. Mets to CNS
Adenocarcinoma - Peripheral.  Seen 50% of the time in non-smokers. Bronchoalveolar carcinoma is a subtype of adenocarcinoma that looks like pulmonary edema/diffuse interstitial infiltrates

Staging for Non-Small Cell:
Stage I: <3cm and no LAD or Mets
Stage II: Hilar LAD or Chest Wall
Stage III: Mediastinal LAD
Stage IV: Mets

Further Workup for Non-Small Cell:
PET Scan
Mediastinoscopy for LN >1cm
CT and MRI of Head usually needed
Bone Scan if symptoms of bone pain or increased Alk Phos
Treatment for Non-Small Cell:
Stage I and II: Sx and Chemo (Debate if radiation is added to this – it may increase mortality).

Stage III: Chemo and Radiotherapy
Stage IV: Chemo for patients with good Performance status

Chemotherapy for Non-Small Cell usually consists of:
Paclitaxel+Carboplatin or

Cisplatin+Etoposide
Adenocarcinoma:  Oral Gefitinib used at times

Here is a chart I made comparing "high-yield" basics for the 4 types:

Paraneoplastic syndromes - the boards like these, so know them cold!
SIADH - Hyponatremia (what are the labs with SIADH? UNa>50 and Uosm>200) - Small Cell
Hypercalcemia and it's multiple symptoms - usually squamous cell
Acromegaly - GH secretion usually from small cell subtype
Cushing's Syndrome - ACTH secretion from small cell subtype
Hypertrophic Pulmonary Osteoarthropathy - New periosteal bone formation - usually adenocarcinoma
Lambert Eaton Syndrome - Proximal Muscle Weakness, autonomic dysfunction (impotence and dry mouth) with areflexia and sparing cranial nerves  The amplitude of action potentials on EMG, and symptoms, improve with repetitive stimulation. This usually occurs with Small Cell Subtypes.  (Remember that Myasthenia has cranial nerve involvement, normal reflexes, and worsening with repeated motion)

January 22nd - MHH - All about the Pituitary

Today at Morning Report a case of a piuitary mass in a patient with history of treated prolactinoma was presented.  Here is an overview of pituitary masses, pituitary disorders, and more on prolactinomas









Pituitary Mass:

Clinical Symptoms: Pituitary masses cause one of two major categories of symptoms - those of mass effect and those of either hypersecretion or hyposecretion of hormones.  Mass Effect from tumor growth can cause headache, seizures, diploplia, blurry vision and/or visual deficits.

When you see a pituitary mass, you should order the following:
TSH and FT4
Prolactin
Alpha Subunit (marker produced by GH) and IGF-1
FSH/LH
Cortisol
Estradiol in Females
Testosterone in Men

Specific Symptoms: Will depend on hormone effects, some of which include:
Patients can get S/Sx of hyper/hypothyroidism
Patients may get cushing's disease or adrenal insufficiency (the former is more common)
Acromegaly may result as well
Prolactinoma symptoms (more below)

Treatment: Depends on treating the causes of hormone excess or deficiency.  Surgery is usually used in these cases as well. 


Prolactinoma:


Classified as microadenomas (<1cm) or macroadenomas (>1cm).  Usually macroadenomas cause visual field defects, but microadenomas are more common overall

Symptoms:  In Men - Loss of libido, impotence, loss of peripheral body hairDiagnosis: Imaging (MRI) and Prolactin Levels. You should work these patients up for "pituitary masses" as stated above - importantly obtaining a TSH (to rule out secondary elevated PRL caused by an elevated TRH level OR concurrent hypothyroidism) and cortisol levels, in addition to other hormones. 

                      In Women - Amenorrhea, galactorrhea and hirsutism
                      If the tumor is large enough, compression of nearby cells can lead to deficiency  
                                of other hormones - commonly TSH, GH and ACTH.                   
                      And of course, mass effect symptoms depending on size of tumor
                 

So, in morning report there was some discussion was raised about levels of Prolactin. What is normal? How high of a levels leads you to suspect prolactinoma? What else raises levels of PRL?

Normal:  <25ng in females, <20ng in Males and Children
40-85 ng: Craniophayngomas, Hypothyroidism and Drugs (esp anti-emetics, antipsychotics and TCAs
~50ng: 25% chance of pituitary tumor and ~100ng: 50% chance of pituitary tumor
>150ng: Usually a prolactinoma, and if the level is >200 it is about 100% chance of being a prolactinoma

Other causes of increased prolactin include:
Any pituitary lesion
Hypothalamic Lesions (histiocytosis X, sarcoid, TB, glioma)
Endocrine: Hypothyroidism, Addison's Disease, Glucortocoid Excess, COS
Ectopic production: Bronchogenic carcinoma, RCC
Neurogenic: Post-Seizure, Spinal Cord Lesion, Nursing
Stress (includes vigorous exercise and post-op)
Pregnancy
Chronic Kidney Disease
Drugs (antiemetics, antipsychotics, opiates)

Treatment of Prolactinoma:Medical treatment with dopamine agonists - bromocriptine and cabergoline.  Bromocriptine is not well tolerated due to side effects, including orthostasis, nausea and dizziness. Bromocriptine, however, is the choice of treatment in pregnant patients.  Nonetheless, both medications can both reduce tumor size and prolactin levels. Pergolide was previously used, but withdrawn due to it's association with valvular disease in patients. 


Surgical treatment is done if drug therapy does not help or is poorly tolerated.  Still, drugs are usually given post-operatively.   


Irradiation is given usually only if the lesion is very large or medical/surgical therapy doesn't help much

Other Pituitary Disorders:


Empty Sella Syndrome - This occurs when the CSF compresses the pituitary.  Mostly present in a multiparous women. Can be secondary to irradation or trauma.  Sometimes this  is benign and does not require treatment, other times hormone replacement is needed. 

Pituitary Apoplexy - Infarct and hemorrhage into the pituitary.  Patients will present acutely with signs of meningiitis (i.e. meningismus and headache) as well as nausea/vomiting, altered mental status, visual changes and vertigo.  If you suspect this, order imaging (MRI is better than CT) and if it is present, neurosurgery needs to be called ASAP!  Sometimes steroids are used for minor cases, but patients with severe symptoms need surgical decompression.

Sheehan Syndrome - Postpartum infarct of pituitary with usual decreases in almost all hormones - TSH, ACTH, GH, LH, FSH as well as sometimes causing DI.  Hormone replacement is the treatment of choice. 

January 15th - LBJ - ASD and Congenital Heart Disease

Atrial Septal Defects are a commonly tested question on boards, and are a very complicated topic.  However, here are the basics you'll need to know for boards. 


Even though it is a congenital heart defect, the various types of ASDs can be asymptomatic until adolescence or adulthood.  Therefore, physical exam features are clues into the presence of the defect before symptoms develop








In general, patients can present with symptoms attributable to atrial fibrillation, right-sided heart failure, and rarely Pulmonary HTN. 




There are 4 types of ASD, each with subtle features to know.  You'll most likely be given a patient with an ostium secundum defect, so know this one well.  A lot of the concepts are also relevant to the other types of ASD, so just remember what makes the others different (which I'll list)


Ostium Secundum - The most common Type of ASD, and the 2nd most common congenital abnormality in adults (the most common being a bicuspid aortic valve)


Exam: Fixed Split S2 and systolic murmur at LUSB. Loud P2 if patient get Pulmonary HTN
EKG: Right Axis Deviation and RBBB
CXR:  Enlarged Right Ventricle, prominent pulmonary vasculature
Diagnosis: Echocardiogram
Treatment: Surgical Closure of the ASD.  This is done in symptomatic patients, and in asymptomatic patients that a have pulmonary: system shunt ratio >2:1. If there is pulmonary HTN, surgical closure usually cannot be done, but percutaneous closure can be done. 
Complications:Patients can get TIA, Strokes, and Eisenmenger's Syndrome


Ostium Primum
Exam: Pansystolic Murmur of MR or TR
EKG: First Degree AV Block, Left Axis Deviation and RBBB
Treatment: Treatment of choice is percutaneous closure


Sinus Venosus - Know patients have anomalous pulmonary venous return, treat with surgery


Coronary Sinus - Know that patients usually have a Left SVC, treat with surgery


Okay, so what else do you need to know for boards regarding congenital heart disease? Here are the very basic minimums to know about most of the congenital diseases in adults that you will encounter on boards:


PFO:
Diagnose with agitated saline and TTE
Evidence to suggest there is an association b/w PFO and migraines and cryptogenic stroke
Some advocate closure of a PFO in those with cryptogenic stroke, but this is controversial


VSD: 
Exam with holosystolic systolic murmur at LLSB, Prolonged Split of S2, as well as murmurs of AR/TR

Diagnose with Echo (suggestive) but need cath to tell difference in O2 Sat b/w Right Atrium and Ventricle

Patients with large VSDs, Significant Shunt, Progressive AR and Volume Overload undergo closure

If left untreated, patients will develop Eisenmenger Syndrome


PDA: 
More common in females, may have history of congenital Rubella infection.

Can present with symptoms of heart failure, and can get pulmonary HTN

Exam wth a Machine-like murmur at LUSB. May have bounding peripheral pulses

Interestingly, if Einsenmeger's occurs there is "differential cyanosis" - only in the lower extremities

Diagnose with Echo, Cath can reveal difference in O2 sat b/w Right Ventricle and Pulmonary Artery

Treatment is closure if the patient has symptoms (but not if the patient has with Pulmonary HTN!)


Coarction of Aorta:
Usually associated with Bicuspid Aortic Valve, and patients can get Mitral Valve disease, as well as LVH

Patients can present with HTN, symptoms of CHF and leg claudication

Exam with a delayed pulse between radial/brachial and femoral areas. Femoral Pulse can be absent. HTN can be present in the Upper Extremities compared to Lower Extremities. Patients usually also have a systolic murmur

CXR with Rib Notching (due to collateral vessels) and a "Figure 3 Sign" (dilated aorta above and below coarcted area)

EKG - LVH

Diagnosis - MRI can visualize best, cath usually done to measure degree and gradient

Treatment - Can do balloon with stenting or surgical repair. Usually done in younger patients, or those with an increased gradient

Outcome - patients have risk for aortic dissection and advanced HTN

January 14th - MHH - HOCM/IHSS

Background: Hypertrophic Cardiomyopathy is inherited about half the time (autosomal dominant). It is these patients with inherited disease that have a high risk of ventricular arrythmias and sudden cardiac death. Annual Mortality in Adults is around 2%

Pathophysiology:  Patients can get asymmetrical septal hypertrophy, concentric hypertropy, and other areas of the heart may be involved. Due to this hypertrophy, a narrow outflow tract occurs and there is a pressure gradient that occurs. 

 


Clinical:
Patients can present along the spectrum of disease - syncope, dizziness/presyncope, signs of diastolic dysfunction (dyspnea, PND, orthopnea), myocardial ischemia (angina), palpitations (arrythmias), and sudden cardiac death.  However, most are asymptomatic early in the disease course, and remember that sudden cardiac death can be the first clinical feature.  The Exam will reveal a mid-systolic murmur in the RUSB that increases with Valsalva. An S4 is usually present.  Not uncommonly a double (and sometimes triple) apical impule is felt. Carotid pulse will be brisk, and is sometimes bifid. 

EKG and CXR:

Some may have deep T-wave inversions in the inferior and anterior leads of the EKG, some patients may have inferio-lateral Q-waves.  Both the EKG and CXR can reveal LVH and LAE.

Diagnosis:
Echocardiogram is diagnostic.  There are multiple findings, but a septum that is too thick (and thicker than the posterior wall) is usually present.  Outflow tract obstruction is usually seen, as well as abnormal motion of the anterior mitral leaflet.   Most patients should have a Holter Placed. Cardiac cath is not usually done on these patients unless surgical intervention is planned. 

Treatment:
Beta Blockers are the treatment of choice. These are given to those with a family history of sudden cardiac death, and in patients with symptoms.  Long-acting Calcium Channel Blockers are used as well (esp Verapamil). If patient has volume overload, give small doses of diuretics, as patients are preload dependant and low volume is dangerous.  If patients have outflow tract obstruction, septal myectomy is performed.  Septal ablation with ethanol can also be performed.   AICDs are placed in those at high risk for Sudden Cardiac Death.  Remember: Avoid aggressive diuresis as well as digoxin and diuretics.

January 11th - LBJ - Polymyositis/Dermatomyositis and Rheum Labs

Today at LBJ a case of Polymyositis was presented

Here is an overview of the inflammatory myopathies and some rheum labs to know for boards








The 3 Inflammatory Myopathies you should know about for boards are Polymyositis, Dermatomyositis and and Inclusion Body Myositis

Clinical
Polymyositis and Dermatomyositis: Proximal Muscle Weakness.  Classically they have difficulty "rising from a chair." Also they can have trouble lifting their head up and trouble climbing stairs. 
Pain is not frequently present in these patients (myalgias in less than 30%), it is more muscle weakness.  Arthralgias may be present, and pharyngeal weakness can lead to dysphagia/aspiration. 
Dermatomyositis is essentially Polymyositis with a rash, but with more to know on this in a bit

Diagnostic Criteria:
1. Symmetrical weakness of limb girdle muscles
2. Muscle Biopsy showing necrosis and perifasicular atrophy
3. Elevated Muscle Enzymes (Can be any - CK, Aldolase, LDH and AST are the most often used)
4. EMG with small action potentials, sharp waves and high frequency discharges

Definite Polymyositis is all 4 above
Definite Dermatomyositis is 3/4 above + Skin/Rash Findings c/w disease

                               So what are the skin/rash findings... There are many. For boards, you'll need to know:

                                                    Gottren's Papules - purple/scaly eruption over the MCPs

Heliotrope Rash - purple discoloration of upper eyelids

Shawl Sign - photosensitive erythema on anterior chest

So, like stated Dermatomyositis is clinically polymyositis with the skin changes. 

Workup/Labs:

Your workup for these patients will be what is necessary to help diagnose (i.e EMG).

Muscle biopsy is done, but usually after MRI - so they know where to target the biopsy

CXR should be checked at the time of diagnosis to look for Interstitial Lung Disease

ANA is Positive in >90 of Patients

Anti-Mi2 can be present in Dermatomyositis (<25% of the time)

Anti-Jo1 can be present in Polymyositis - and when this is present patients is known to "antisynthetase antibody syndrome) - characterized by polymyositis, arthritis, interstitial lung disease, fever, Raynaud's and

"mechanics hands" (a dry scaly rash on the fingers)

Other antibodies may be positive if there is an overlap syndrome present (more on this later)

Complications:

- Like stated before, pharyngeal involvement can lead to aspiration

- Patients can get cardiomyopathy or arrythmias from cardiac involvement

- Interstitial lung disease is a complication as well

- Malignancy:  not so much a complication, but a disease association.  Especially in patients with Dermatomyositis, there is evidence that a workup for malignancy should be done.  The most common malignancies found in these patients are ovarian, lung and head/neck. So, a good screening/workup should be done in patients with Dermatomyositis.  CT scanning of the Chest, Abdomen and Pelvis are usually done

Treatment:

Initially with High Dose Prednisone (1-2 mg/kg/day) which is continued until elevated enzymes decrease

After these values begin to decrease, the dose is tapered to maintenance of 5-10mg daily.  Steroid sparing agents (i.e. MTX or AZA) can be used.  Hydroxychloroquine can be used for the skin lesions. 

Inclusion Body Myositis:

All you need to know is that this occurs in patients that are older than 50 years, and patients have DISTAL muscle weakness> Proximal weakness.  Dyphagia is usually present as well (greater than 50% of patients).  Concominant cardiovascular disease is usually present in these patients, but may be due to their age on diagnosis.  Muscle Biopsy is diagnostic.  Sometimes it is precipitated by drugs such as alcohol and cocaine. To treat, stop offending agent and can give steroids, but they don't help too much.

For Boards, here is a quick Antibody Review For You:
ANA - Positive is so many things....  SLE, Scleroderma, Sjogrens, Mixed Connective Tissue, Myopathies

Anti-Histone - Drug Induced Lupus... Remember these patients have the same clinical findings as in regular Lupus, but the CNS are Renal findings are rare

Anti-CCP - Diagnose/Send in patient with early RA

RF - Rheumatoid Arthritis

Anti-Jo1 - Antisynthesase Syndrome

Anti-Mi2 - Polymyositis

Anti-Smith - Specific Test for SLE. Good to Send for Flare.

Anti-dsDNA - SLE. Good to Send for Flare. Predicts Renal Involvement

Anti-U1 RNP - Mixed Connective Tissue Disease - Patients get SLE + Polymyositis + Scleroderma

SSA (Anti-Ro)  and SSB (Anti-LA) - Sjogren's and SLE. SSA with Neonatal Lupus too. 

Anti-scl70 - Diffuse Scleroderma

Anti-Centromere - CREST Syndrome (i.e. Limited Scleroderma)

c-ANCA - Wegeners

p-ANCA - Churg-Strauss and microscopic Polyangiitis.  These two are also MPO +: know that there are diseases which are p-ANCA positive and MPO Negative (such as ulcerative colitis and hepatitis)

Anti-Mitochondrial - Primary Biliary Cirrhosis

Anti-Smooth Muscle - Autoimmune Hepatitis (patients are usually ANA positive as well)

Reference:
Images Courtesy of
Picasaweb: picasaweb.google.com/.../6sgfojHcfP1D90x2j_2ANw
Emedicine: emedicine.medscape.com/article/332783-overview
mediwire.skyscape.com

January 8th - MHH - Glomerulonephritis

Today at Hermann, a case of possible GN was presented in a patient with Hepatitis C.  Here is a general overview of Glomerulonephritis:






Patients can present with HTN, Oligemia, Hematuria and peripheral edema.  If you see dysmorphic RBCs or RBC Casts, think of a glomerular disorder with a nephritic syndrome component

There are multiple ways to memorize the GNs.  I like to think of them as those causing low complement and those with normal complement. The ones with low complement you can think of having a "systemic disease"  present versus a more renal-based pathology...

Low Complement - Systemic Diseases
SLE - Think when patient has some of the 11 current diagnostic criteria
Endocarditis - In setting of fever and murmur
Cryoglobulinmenia - Patient with underlying Liver Disease, arthralgias and skin rash
Shunt Nephritis - after insertion of vascular shunt
Cholesterol Emboli Syndrome - Post cath/vascular intervention with toe rash/ischemia and eosinophilia

Low Complement - Renal Causes
1) Post-Infectious - Occurs on average 10-14 days after strept infection (including pharyngitis or impetigo) but other infections can cause PIGN as well.  If you want to tell for sure that there is prior infection, you can send ASO titers or anti-DNAse B titers.  Complements are low, but for no longer than 2 months.  Most patients self-recover, but some go on to need dialysis.  Treat the underlying infection. 

2) Membranoproliferative - Remember this disease can present with a nephrotic overlap in addition to a GN. There are many underlying "triggers" - but Hepatitis C is the most common.  Complements stay low for LIFE - unlike with PIGN where they can return to normal after 2 months.  Children are usually given steroids, adults have the underlying condition treated (especially IFN for Hepatitis C) and some are give Aspirin as well. 

Normal Complement (i.e. the Pauci-Immune diseases)
1) ANCA-associated and other vasculitis
Wegeners: c-ANCA Positive. Granulomas in the Upper Airway, Lung and Kidneys. Consider in a patient with history of sinusitis or recurrent episodes of otitis media.  CXR with Lung nodules, cavities or infiltrates.
Arthritis and purpura are common as well. 
Churg-Strauss: - p-ANCA positive.  History of allergic rhinits or asthma that is difficult to control. High serum eosinophils. Many patients with have arthralgias and neuropathy. 
HSP - Arthralgias, palpable purpura, abdominal pain and renal failure.  The debate is out whether or not to give these patients steroids, as most patients recover without treatment. 
Microscopic Polyangiitis - p-ANCA Positive.  Can present with a multitude of symptoms, and often involves the lung.  Many patients have fever, palpable purpura, mononeuritis multiplex and arthralgias. 

2) GBM Diseases - Essentially those that attack the basement membrabe of both the kidney and lung together. Goodpastures Syndrome is the one to know.  Pulmonary hemorrhage with various degrees of hematuria are key. Send anti-GBM antibodies.  Treat with repeated plasmapheresis combined with glucocorticosteroids and cyclophosphamide. 

3) IgA Nephropathy - More common in males and Asians.  Unlike with PIGN, these patients will have GN immediately after the antecedent viral infection.  They can have microscopic or gross hematuria, and even extreme exercise can "trigger" the disease.  Patients may need ACEI and Fish Oils. 

4) Alports - X-Linked with cochlear disease and lens involvement.

Also remember that any of these nephritic syndromes can become RPGN (i.e crescenteric GN) if the creatinine rapidly rises within days to weeks.  Those diseases with ANCA positivity and GBM Syndromes are notorious for causing this to occur.  Treat the ANCA disorders (and SLE) with steroids + cyclophosphamide.  Treat the Anti-GBM diseases with plasmapheresis, followed by steroids + cyclophospamide.

January 7th - LBJ - Delirium

How do you Diagnose Delirium? With the CAM:
A - Acute Onset and Fluctuating Course
B - Inattention
C - Disorganized thought (rambling speech, changing subjects)
D - Altered Consciousness (from stupor to hyperalert)
(To diagnose Delirium you need A and B plus C or D) 






How to differentiate from dementia - Essentially more insidious onset, and patients with dementia usually have normal consciousness. 

Risk Factors:  Increasing Age, previous history of delirium or cognitive impairment, underlying pain, high dose of pain meds, underlying chronic diseases (i.e. malignancy) 

Causes:  You name it and it can cause delirium...
I WATCH DEATH is a good mnemonic, but there are many out there:


Infection (UTI, PNA are common, but any cause possible)
Withdrawal (of EtOH or other drugs)
Acute metabolic (renal failure, liver failure, glucose)
Trauma
CNS pathology (you name it, it's here)
Hypoxia (any cause - Embolism, infection, anemia)
Deficiencies (electrolytes)
Endocrinopathies (esp Glucose, Thyroid)
Acute vascular diseases (MI, CVA)
Toxins or drugs (This list is endless - offenders include benzos, quinolones, narcotics, steroids)
Heavy metals

Other Issues That can cause this - HTN, post-op state, seizures, fecal impation and many others....
 Treament:
1) Prevention - Frequent reorientation and stimulation (i.e. Lights on during the day/sitter).  Maintain the sleep-wake cycle in patients, make sure they can hear well and see well, make sure they get good hydration and nutrition, and don't restrain them ig you can (this includes foleys & SCD/TEDS)
2) Treat the underlying condition (i.e. infection, offending medication, pain)
3) Haloperidol and other antipsychotics may be needed, especially in the post-op state

January 7th - LBJ - TTP

So, remember that mnemonic in First Aid - "FATRN" - Fever, Anemia, Thrombocytopenia, Renal Failure, Neurologic Changes - Well it's good for med school memorization, but not applicable to TTP in the real world.  Remember - before the treatment for TTP was perfected, those patients did have Renal Failure and Altered Mental Status - but we see that not too often now because we treat patients beforehand - if you do see these in a patient it is late stage disease!!

So when do you think of TTP on Boards?  Whenever you see the combination of a Microangiopathic Hemolytic Anemia (MAHA) And Thrombocytopenia.  What Makes a MAHA? - fibrin deposition that shears the RBC cells causing fragments, ie schistocytes.  These patients will have a low haptoglobin and a high LDH with a Normal Coombs Test.  However, do not wait for these lab tests to diagnose.  As stated before patients can get Neurologic Disease - anywhere from forgetfullness, delerium to seizures.  Don't wait for this to diagnose.  Renal Failure is late stage.  Don't have to have this to diagnose. So the take home:

TTP + MAHA will be TTP (or even HUS) or some other causes listed below...

There are two forms of TTP - idiopathic and acquired. Acquired is usually due to medications - ticlopidine, clopidogrel, cyclosporine, mitomycin C.  Almost all patients with TTP regardless of the cause have a deficiency of ADAMTS13 - which usually cleaves vWF.  If you can't cleave vWF - you get large aggregates that cause a thrombotic state.  ADAMTS13 is not involved in HUS. 

So, If you suspect TTP, look for schistocytes on the smear. If there are at least "occasional" on the smear (i.e. more than 2 per hpf) then they are significant. If you see this with thrombocytopenia, consider treatment immediately...

Before treatment was perfected, mortality was >90%. If TTP/HUS is suspected (and you are sure it's not another condition listed below) then treat with plasma exhange.  If you can't get the Line or there is no availability to do plasma exchange, then transfuse FFP in the meantime.  Some will even give steroids in the interim.  No matter what the platelets are, do not transfuse, as you will cause more clotting.  Monitor treatment with LDH Levels. 

What else can cause Schistocytes (i.e a MAHA) that you need to know for Boards, and how to tell Apart:
TTP: As per this article
HUS: Can present like TTP, more likely in young patients, almost all will have some renal insufficiency
DIC: Will have MAHA and TCP, But PT and PTT are elevated, unlike with TTP/HUS where they are       normal        
HELLP - MAHA + TTP But Patient will be pregant and have elevated liver enzymes
Malfunctioning Valve Prosthesis: - Usually does NOT cause Thrombocytopenia but will have MAHA.        PT and PTT are normal unless there is an underlying disorder or patient is on warfarin for the valve!
Malignant HTN - Can tell by the BP Reading.  Also, Thrombocytopenia is uncommon with this

Just a bit more on HUS: There are two forms - in children this is usually triggered by antecedent bacterial enteritis, especially E. Coli. Within a week of the infection, patients can get renal failure, MAHA and TCP.  In adults there are forms of HUS after bone marrow transplant and chemotherapy.  Sometimes it is hard to distinguish TTP/HUS - so even when HUS is considered, plasma exhange may be used "just in case" TTP is going on.

January 5th - LBJ - Testicular Cancer

Morning Report Today was a patient with Testicular Lymphoma.  Here is some brief board relevant material for testicular cancer.  There is a lot in the reading material out there, but for the boards you will not need to know the basics (not the nitty gritty) about staging and treatment, but you must know workup and tumor markers....




Testicular cancer is the most common solid malignancy in adult males under the age of 35.  The case presented was testicular lymphoma - essentially for boards, suspect testicular lymphoma with a testicular mass in any man above the age of 45!!! PEARL: If you think a patient has epididymitis, and they fail a course of antibiotics - U/S!!!! - it's cancer!!!!

The vast majority of testicular cancers overall (>95%) are germ cell tumors (i.e. Seminomas and Non-Seminomas, more on this later). Others, such as the aforementioned lymphoma as well as sertoli/leydig are rare. You will not likely be tested on these "other" cancers on boards. 

Risk Factors for testicular cancer include cryptoorchism, family history, Klinefelters, and HIV/AIDS.  Remember that men with cryptoorchism have increased risk of cancer in BOTH testicles, and the longer you wait to perform orchiopexy, the higher the risk of developing cancer. 

Workup:
You should know about workup as well as tumor markers and classification for the boards. If your ultrasound is suggestive of cancer, NEVER NEVER NEVER biopsy. Always do an inguinal orchiectomy with pathology.  If you are suspecting testicular cancer on U/S you should order LDH, AFP and hCG. These makers are never used to diagnose (with one exception - mentioned below) and are used for staging and monitoring therapy/relapse.

The Markers: 
Seminomas:  ALWAYS AFP Negative, can produce hCG, but rarely do so (about 10% secrete hCG)
Non-Seminomas:
    - Teratoma  - ALWAYS Negative for BOTH AFP and hCG)
    - Choriocarcinoma  - ALWAYS Positive for ONLY hCG)
    - Yolk Sac  - ALWAYS Positive for ONLY AFP)
    - Embryonal  - ALWAYS Negative for AFP, about half secrete hCG)

The exception - So, the take away from these markers for boards (besides knowing them) from a diagnostic purpose is the classic question - testicular pathology reveals that the cancer is a seminoma, but the AFP is high - well, the pathology is wrong, and there is some non-seminoma component!!!!! Therefore, the patient is treated along the non-seminoma pathway per below...

Staging for both Seminomas and Non-Seminomas: There are pretty involved details, but you'll just need to know the basics for knowing the treatment approach. Know that markers and levels are also used, but you do not need to know the specifics, only this:
Stage I: Testicle Along
Stage II: Retroperitoneal LN involvement
Stage III: Mets

Treatment: When Combo Chemo is Given it's usually either BEP (Bleomycin, Etoposide and Cisplatin) or EP alone.

Seminoma:
Stage I: Orchiectomy alone or combined with surveillence or single agent chemo or radiation. [NOTE: Surveillance is generally only used for patients that have a risk of side effects from radiation...  Chemotherapy is sometimes slightly favored over radiation due to the side effect profile of single agent chemo being better than radiation.... so the boards will not ask you which is better, chemo or rads....)]
Stage II:  Orchiectomy + Chemo or Orchiectomy + Radiation
Stage III:  Orchiectomy + Chemo

Non-Seminoma:  Treat based on stage
Stage I: Orchiectomy + Chemo (favored) or Orchiectomy with surgical removal of the LN (I.e. RPLND)
Stage II: Orchiectomy + Chemo or Orchiectomy + RPLND, although most will do Orchiectomy + both Chemo and RPLND
Stage III: Orchiectomy + Chemo

Some info on relapse: If tumor markers rise after Surgery or Radiation - More chemo is given. Be aware that alternative regimens to BEP and EP are used, but you don't need to know them (...so I won't list them!) If patient has a non-seminoma and have masses found on re-imaging - consider teratoma with surgical removal.

Complications: Infertility, so recommend sperm banking prior to Tx
                         Secondary Malignancies, esp AML, from Chemo

Overall, there is a lot of info on testicular tumors that technically can be asked on boards, but like I stated - just know basic workup, markers and some info about treatment options...

January 5th - MHH - Aortic Stenosis

Today at Morning Report, a case was presented by the cardiology service regarding a consult for a patient with aortic stenosis.  Here is a brief overview of the disease for board purposes...





Predisposing conditions: Most common cause is calcific disease of the normal trileaflet valve, but patients with congenital bicuspid valves also get AS.  Patients with this congenital condition usually present earlier, usually between ages 40-60, as opposed to those with calcific disease who presents after 60.    Rheumatic fever is a cause as well, but rarely without involvement of mitral valves. Also, keep in mind that patients with AS commonly have concurrent coronary artery disease. Patients with Heyde's syndrome, associated with acquired von Willebrand syndrome, can have AV Malformations and GI Bleeds in association with Aortic Stenosis.

Clinical presentation: In the earlier stage of the disease, patients can present with exercise intolerance and fatigue.  Remember the triad of Angina, Syncope and LV Failure.  These symptoms also portend prognosis, with medical treatment leading to survival rates of about 5 years with angina, 3 years with syncope, and 2 years with LVF.   

Physical Exam:  The murmur is a harsh mid-late systolic cresendo-decresendo (i.e. "diamond shaped" murmur) at the RUSB or suprasternal notch.  It can radiate into the carotids.  The carotids develop a slow upstroke. The aortic component of the 2nd heart sound is usually diminished, and as the condition gets worse, A2 can disappear. Also, due to radiation of the murmur, some elderly patients may have the highest intensity at the apex.  Overall, the murmur intensity decreases with Valsava and intensifies with squatting.  An S4 is usually present, and a click may be present in those with bicuspid valves. 

Diagnosis:  Doppler is needed, and used to classify based on severity (the valve area is probably all you need to know for boards!). Patients also need repeat TTE's yearly, sometimes more frequently if severe dz present. 

Severity   Mean gradient (mm Hg)     Aortic valve area (cm2)
Mild                < 25                                      >1.5
Moderate          25-50                                 1-1.5
Severe              >50                                    <1
Critical            >80                                     <0.5



Treatment:
Medications: Judicious use of diuretics if patient has symptoms of volume overload, but this can cause problems in patients with severe disease.  Same with afterload reduction - use carefully.  Treat HTN with usual agents. Some studies have shown that "statins" reduce the progression of the disease. 

Surgery  (i.e. aortic valve replacement) is recommened for:
      -Severe AS with Depressed EF
      -Patients that have symptoms (angina, syncope, NYHA II or greater)
      -Asymptomatic and needs other surgery (i.e. CAGB)

SO... what is aortic sclerosis?  Patients with this condition have a thickened valve but without outflow obstruction. Therefore, they are usually asymptomatic - although the murmur findings are very similar to aortic stenosis, so it is hard to distinguish on physical exam.