renal disease in rheumatoid arthritis ( ra ) is usually asymptomatic and is detected only on biochemical investigations . thrombotic microangiopathy ( tma ) results in thrombocytopenia , microangiopathic hemolytic anemia ( maha ) , and thrombotic occlusion of the microvasculature . primary tma includes hemolytic - uremic syndrome ( hus ) and thrombotic thrombocytopenic purpura ( ttp ) . secondary tma is associated with neoplasia , autoimmune diseases ( antiphospholipid antibody syndrome , systemic lupus erythematosus ( sle ) , and others ) , infections , and drugs . we report here a female patient with ra who developed tma and improved subsequently with plasmapheresis . she was known to have ra and was taking oral steroids and diclofenac tablets on and off as self - medication . there was no history of diarrhea , fever , native medication , or any other drug intake . on examination , she had edema legs , pulse rate of 80/min , and blood pressure of 130/80 mm / hg . urinalysis showed 3 + protein , 56 red cells / hpf ; urine protein creatinine ratio 2.8 . hemoglobin was 9.2 g / dl ; total count 5600/cu.mm ; platelet count 80,000/cu.mm ; peripheral smear numerous schistocytes ; blood urea 200 mg / dl ; serum creatinine 12 mg / dl ; prothrombin time 12.3 s ; inr 0.9 ; activated partial thromboplastin time 38.5 s ; serum lactate dehydrogenase ( ldh ) 931 iu / l ; total bilirubin1.2 mg / dl ; indirect bilirubin 0.8 mg / dl ; and direct coomb 's test negative . rheumatoid factor and anti - cyclic citrullinated peptide antibodies were positive ; antinuclear antibody ( ana ) and anti dsdna antibody were negative . anti sjogren 's syndrome related antigen a ( ssa ) , anti - sjogren 's syndrome related antigen b ( ssb ) , anti - sm , anti - u1rnp anti - jo antibodies and serum antineutrophil cytoplasmic antibodies were negative . renal biopsy revealed patchy cortical necrosis , fibrin thrombi , and fragmented red blood cells ( rbcs ) [ figure 1 ] occluding capillary loops in 4/7 glomeruli . she received totally 6 sessions of plasmapheresis ( two plasma volume exchanges each time ) after which her platelet count and ldh levels were normalized . she was started on 1 mg / kg / day prednisolone tapered over next 6 months . her renal failure improved and was discharged with serum creatinine of 2.2 mg / dl . she was started on methotrexate and at 2 years after presentation , her creatinine was 1.5 mg / dl without further recurrence of tma . renal biopsy showing fibrin thrombi and fragmented red blood cells in glomerulus ( h and e ) ra is a chronic systemic disease characterized by persistent inflammatory synovitis , involving peripheral joints in a symmetric distribution . tma was defined as pathological lesions characterized by endothelial injury and platelet - rich thrombi formation in microvasculature , leading to maha , thrombocytopenia , and end - organ ischemic damages . amorosi and ultmann defined the classic pentad of thrombocytopenia , maha , acute renal impairment , neurologic abnormalities , and fever . however , the dyad of otherwise unexplained thrombocytopenia and maha are sufficient to diagnose of tma . although tma occurs in association with connective tissue disorders ( ctds ) , tma in ra is rarely reported . to our knowledge , only two case reports and a case study with ra and tma have been described so far . published a case report of a 35-year - old female patient with ra presenting with tma and antiphospholipid syndrome . kfoury baz et al . reported another female patient presenting with ttp at 18 months after onset of ra . she was tested negative for ana and antiphospholipid antibodies and was treated with plasma exchanges and glucocorticoids . the pathogenetic mechanisms behind the development of tma in ra are linked to severe deficiency of adamts13 ( a disintegrin and metalloprotease with thrombospondin-1-like domains ) due to autoantibodies that alter its function . adamts13 is a plasma enzyme involved in cleavage of unusually large ( ul ) von willebrand factor ( vwf ) multimers into smaller and less adhesive vwf forms . failure to degrade these ul - vwf multimers leads to excessive platelet aggregation and microvascular occlusion . in a study of 127 patients with ctd and tma including 10 patients with ra , severe deficiency of adamts13 activity was seen and was closely associated with the presence of anti - adamts13 igg antibodies . therapeutic plasma exchange resulted in high remission rate in patients with sle than in patients with ra . our patient , who had ra for 5 years , presented with features suggestive of tma . renal biopsy showed platelet - fibrin thrombi and fragmented rbcs in glomeruli and patchy cortical necrosis . the possibility of sle , mixed connective tissue disorder , antiphospholipid syndrome , and drug - induced tma was ruled out . however , atypical hus , which may have been unmasked by the inflammatory process in this patient , could not be ruled out . testing for adamts13 deficiency , antibodies against adamts13 , factor h and anti - factor h autoantibodies was not done due to logistic reasons . she was treated with high - dose steroids and plasmapheresis following which her renal function improved . she was started on methotrexate due to high disease activity and currently on follow - up . to conclude , the possibility of renal tma should be considered in ra patients who present with renal failure as timely diagnosis and treatment with plasmapheresis improve the long - term renal outcome .
rheumatoid arthritis ( ra ) is one of the commonest rheumatological diseases . 
 renal involvement is not common but can occur as a result of chronic inflammation as part of disease process or drug toxicity . 
 thrombotic microangiopathy ( tma ) is characterized by microangiopathic hemolytic anemia , thrombocytopenia , and organ failure of variable severity . 
 only a few cases of tma in patients with ra were reported to date . 
 we describe a 45-year - old female patient with ra who presented with oliguria and edema . 
 renal biopsy showed tma with patchy cortical necrosis . 
 she improved with hemodialysis and plasmapheresis .