Use of cyclophosphamide in the treatment of thrombotic thrombocytopenic purpura complicating systemic lupus erythematosus: report of two cases

Abstract

Thrombotic thrombocytopenic purpura (TTP) is an unusual complication of systemic lupus erythematosus (SLE). There are no randomized, prospective studies of its treatment. The association of plasma infusions and (or) plasmapheresis with steroids improves survival when compared with steroid treatment alone, but these patients still have a higher mortality than those with "classic" TTP. The role of immunosuppressive drugs in the management of this disorder remains uncertain. We report two cases of TTP in SLE which presumably benefited from the addition of cyclophosphamide to the treatment with plasmapheresis and steroids.

Systemic lupus erythematosus and thrombotic thrombocytopenic purpura: a case and review

“…Nephrotic syndrome has been reported in three cases [6,13,14], whereas acute renal failure has occurred in six cases [4,7,9,11,12,13]. Our patient presented with nephrotic syndrome and ultimately acute renal failure.…”

“…Caramaschi et al [2] reviewed most of the 31 cases in 1998 [2,3,4,15,16,17,18,19,20,21,22,23, 24] To our knowledge, only 17 pediatric patients with TTP and SLE have been reported [2,3,4,5,6,7,8,9,10,11,12,13]; 16 patients, including the presented case, were female and 2 were male. The mean age was 15.5±2 years (range 10-19 years).…”

“…Systemic lupus erythematosus (SLE) is a chronic connective tissue disorder that can involve joints, kidneys, serous surfaces, and vessel walls, and can present with many of the features of TTP. However, these two conditions are rare in both children and adults [2,3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25]. We present the 18th known case of TTP and SLE in a pediatric patient, and the first reported case of associated membranous nephropathy.…”

“…In contrast, the pediatric cases have a more-diverse presentation. Unlike the 94% of adult cases, who developed TTP after SLE, only 6 of 17 pediatric patients had such a presentation [2,3,4,5,9,13,14,16,17,18,19,20,21,22,23]. SLE was inactive in all these patients for a period between 1 and 8 years before the occurrence of TTP [1,2,3,4,8,9,11,12,13,14,15,16,17,18].…”

“…Other cases had findings such as proteinuria and hematuria. A kidney biopsy was performed in 6 of 17 reported pediatric cases, whereas 2 had necropsy [2,3,4,5,6,7,8,9,10,11,12,13,14]. The autopsy revealed type IV diffuse proliferative glomerulonephritis in both cases.…”