Day #59 - Sclerdoderma Renal Crisis

Today we discussed a case of a patient with diffuse scleroderma who presented with a hypertensive crisis with acute renal failure and microangiopathic hemolytic anemia. The key in this case is recognizing the clinical presentation and aggressive management of the hypertension with supportive care for the hemolytic anemia.

We discussed the differential diagnosis of Raynaud's:

• Primary (younger age of onset, symmetrical, tend to be female, no ulcers/pitting, predictable and frequent attacks)
• Secondary
  
  • Trauma (i.e. jack hammer operators)
  • Collagen Vascular Associated - Scleroderma, SLE, MTCD (Scleroderma + SLE + myositis overlap), less likely in Sjogren's and RA
  • Secondary to vascultitis (i.e. Berger's disease)
  • Paraneoplastic (ovarian cancer, angioblastic lymphoma)
    
  • Vascular - peripheral vascular disease
  • Drugs (b-blockers, ergotamine, interferons, PVCs)
  • Hyperviscosity (Waldenstroms, cryoglobulinemia (HepC), cryofibrinogenemia, cold agglutinins, polycythemia)
  • Endocrine (carcinoid, pheo, hypothyroidism)

Treatment of Primary:

• Avoid precipitants (keep warm, pre-warm hands before going outside, avoid drugs that worsen)
• Calcium Channel Blockers (i.e. verapamil, amlodipine) or ARBs (losartan)
  
• Topical nitrates

Treatment of Ischemic Digit from Raynaud's:

• As above
• Analgesia
  
• Topical/Intravenous nitrates
• Limited evidence for prostaglandin, NO, sildenafil, epoprostinol

We also discussed limited scleroderma and distinguised it from diffuse disease by the absence of skin thickening above the elbow, or on the trunk and legs. We talked about the CREST syndrome (now referred to as limited scleroderma)

C = calcinosis of digits from infarcts
R = raynaud's
E = esophogeal reflux
S = sclerodactaly
T = telangectasia

10-15% of people with limited scleroderma will unfortunately develop pulmonary hypertension, which is the complication that is likely to cause significant morbidity and mortality. These patients should be screened yearly with PFT (with DLCO) and 2D echo and pulmonary hypertension should be treated.

Patients with diffuse disease tend to get other end organ complications including pulmonary fibrosis (and secondary pulmonary hypertension) which, once established cannot really be treated. We quickly touched upon the treatment of early pulmonary fibrosis with cyclophosphamide.