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Copyright belongs to The Arab Society of Nephrology and Renal Transplantation (ASNRT)
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Scleroderma Renal Crisis Precipitated by Steroid Treatment in Systemic Lupus Erythematosus and Scleroderma Overlap Syndrome
Abstract
Introduction: Connective tissue disorders can overlap in various ways. Patients may present with features of more than one specific disease without satisfying the diagnostic criteria and thereafter evolve into a specific disease entity. Occasionally, patients may fulfil simultaneously
the diagnostic crateria of two or more diseases. Several cases of systemic sclerosis (SSc) and systemic lupus erythematosus (SLE) overlap syndrome have been reported. SLE patients often develop lupus nephritis, the treatment of which is based on immunosuppression with corticosteroids (CS) and cytotoxic drugs. However, the use of high dose of CS has been associated with scleroderma renal crisis (SRC) in patient with SSc.
Case report: a 43-year-old woman presented to the nephrology department of the Military hospital in Rabat, Morocco, in August 2011 with progressive dyspnea and oliguria. She was diagnosed as SLE and scleroderma overlap syndrome based on clinical and serological
markers. Renal biopsy showed lupus nephritis. Immunosuppression consisting of high-dose steroid and cyclophosphamide pulses was given. There was response to treatment but 15 days later the course of the disease was complicated by scleroderma renal crisis evidenced
by elevated blood pressure, deteriorating kidney function, hemolysis and thrombocytopenia. The patient was treated with perindopril and rapid reduction of steroid doses. This was followed by correction of hemolysis and thrombocytopenia. Two months later, the patient was off dialysis, but had chronic renal insufficiency with an estimated GFR of 25 ml/minute.
Conclusion: This report describes the occurrence of SRC in a patient with lupus nephritis and SSc/SLE overlap syndrome who was treated by CS and
cyclophosphamide.
the diagnostic crateria of two or more diseases. Several cases of systemic sclerosis (SSc) and systemic lupus erythematosus (SLE) overlap syndrome have been reported. SLE patients often develop lupus nephritis, the treatment of which is based on immunosuppression with corticosteroids (CS) and cytotoxic drugs. However, the use of high dose of CS has been associated with scleroderma renal crisis (SRC) in patient with SSc.
Case report: a 43-year-old woman presented to the nephrology department of the Military hospital in Rabat, Morocco, in August 2011 with progressive dyspnea and oliguria. She was diagnosed as SLE and scleroderma overlap syndrome based on clinical and serological
markers. Renal biopsy showed lupus nephritis. Immunosuppression consisting of high-dose steroid and cyclophosphamide pulses was given. There was response to treatment but 15 days later the course of the disease was complicated by scleroderma renal crisis evidenced
by elevated blood pressure, deteriorating kidney function, hemolysis and thrombocytopenia. The patient was treated with perindopril and rapid reduction of steroid doses. This was followed by correction of hemolysis and thrombocytopenia. Two months later, the patient was off dialysis, but had chronic renal insufficiency with an estimated GFR of 25 ml/minute.
Conclusion: This report describes the occurrence of SRC in a patient with lupus nephritis and SSc/SLE overlap syndrome who was treated by CS and
cyclophosphamide.
