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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