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Sehgal - Scleroderma Renal Crisis

Updated: Jul 8, 2019


CC: Middle aged male presenting with scleroderma with shortness of breath and chest pain, ongoing x 2-3 months who presented with pericardial effusion and scleroderma renal crisis.

 

TEACHING TOPICS:


1. Presentation of scleroderma renal crisis:

  • Risk factors: DcSSc, use of moderate- to high-dose glucocorticoids, presence of anti-RNA polymerase III antibodies

  • Pathophys: abnormalities in R-A-A system and endotherlin-1 --> treat with ACEi (captopril). May temporarily need dialysis

  • Various manifestations of hypertensive emergency - headache, encephalopathy, seizure, hypertensive retinopathy

  • Rarely, normotensive form of scleroderma renal crisis can occur

  • Lab tests: microangiopathic hemolytic anemia w/ schistocytes, thrombocytopenia, proteinuria

  • Serum creatinine may remain elevated for some time after controlling blood pressure

2. Cardiac involvement of scleroderma:

  • Pericarditis in 10% of patients, pericardial involvement noted in 70-80% at biopsy. Effusions can be small or develop rapidly into tamponade

  • Indicates poor prognosis

  • Myocardial fibrosis can occur by vascular vasospasm --> ischemia and fibrosis

  • OR cardiac complications secondary to systemic or pulmonary hypertension




3. Differential diagnosis of pericardial effusion:

  • Malignancy, infection (TB if endemic), autoimmune disease, hypothyroidism

  • Iatrogenic: medications, anticoagulation

  • Idiopathic

  • If concern for cancer or bacterial etiology --> pericardiocentesis should be considered for diagnostic purposes.

  • If unknown cause and elevated inflammatory markers, can trial empiric treatment

  • Drainage should be considered if large, idiopathic effusions are present for more than 3 months due to risk of progression to tamponade

4. Signs/symptoms of cardiac tamponade:

  • Sinus tachycardia

  • Elevated JVP

  • Pulsus paradoxus (>10 mmHg)

  • Pericardial rub

5. Differential diagnosis of raynaud phenomena: idiopathic vs. secondary to underlying vascular disease

-Systemic sclerosis: unique changes to microvascular system develop with intimal fibrosis and endothelial dysfunction, increased platelet adhesion, decreased storage of von Willebrand factor, decreased adenosine uptake

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