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Pulmonary-renal syndrome in systemic sclerosis
J Bar1, M Ehrenfeld, J Rozenman
1Departments of Medicine C and Imaging and Pathology, The Chaim Sheba Medical Center, Tel Hashomer, and the Sackler Faculty of Medicine, Tel Aviv University, Israel.
Seminars in Arthritis and Rheumatism
|June 19, 2001
Summary
Scleroderma-pulmonary-renal syndrome (SPRS) is a rare, fatal complication of systemic sclerosis (SSc). This study analyzed 11 cases, highlighting its poor prognosis and suggesting aggressive treatment strategies for this severe pulmonary and renal manifestation.
Area of Science:
- Rheumatology
- Nephrology
- Pulmonology
Background:
- Systemic sclerosis (SSc) commonly causes renal failure, pulmonary hypertension, and interstitial lung disease.
- Concomitant pulmonary hemorrhage and acute renal failure in SSc, termed scleroderma-pulmonary-renal syndrome (SPRS), is rarely documented.
- This study presents the first analysis of SPRS in SSc patients.
Observation:
- A 44-year-old woman with SSc experienced a fatal course of acute renal failure with diffuse alveolar hemorrhage.
- Literature review identified 10 additional cases, forming a cohort of 11 patients with SPRS.
- The average age of affected patients was 46, with 80% being female and most having diffuse SSc.
Findings:
- SPRS occurred an average of 6.4 years after SSc onset, often associated with prior fibrosing alveolitis or D-penicillamine treatment.
- Normotensive renal failure characterized most SPRS cases (82%).
- SPRS demonstrated a poor prognosis, with all 11 patients dying within 12 months; only 60% received corticosteroids.
Implications:
- Pulmonary-renal syndrome is a rare but fatal SSc complication.
- Given the scarcity of treatment data and poor prognosis, aggressive therapeutic strategies are suggested.
- Recommended treatments include pulse corticosteroids, cyclophosphamide, and potentially plasmapheresis.