Wednesday, January 27, 2010

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
-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%)

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.

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