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In the literature thrombotic microangiopathy (TMA) associated with ANCA-associated vasculitis (AAV) has only been reported in isolated case reports. Here, we report a patient with granulomatosis with polyangiitis (GPA), who presented with TMA after initiation of remission induction therapy. A 36-yearold male patient presented with dyspnea and decreased urine output. Laboratory results demonstrated elevated creatinine, low albumin, low hemoglobin, normal leukocyte and platelet count, normal LDH, and elevated acute phase reactants. Urinalysis revealed proteinuria (1275 mg/day) and an active urine sediment. Serum complement levels were normal and proteinase 3 ANCA titer was> 200 IU/ml. Urinary ultrasound revealed normal kidney sizes and normal parenchymal thicknesses with increased renal parenchymal echogenicity. A kidney biopsy revealed pauciimmune crescentic glomerulonephritis. The diagnosis was GPA and an induction treatment of pulse steroid, intravenous cyclophosphamide, and plasma exchange was initiated. After two doses of cyclophosphamide, rituximab treatment was initiated. Fifteen days after the second dose of rituximab, thrombotic microangiopathy (TMA) was considered in the patient who had no increase in hemoglobin value (despite initiation of erythropoetin) and decreased platelet count. Peripheral blood smear revealed 5-9 schistocytes in each area. A corrected reticulocyte count was elevated, and haptoglobin was low. ADAMTS13 activity was normal. Plasma exchange was not reinitiated. The kidney biopsy was re-evaluated, but no histopathological changes consistent with TMA were found. The patient was under follow-up for TMA by checking his hematological parameters once a week. Two months later, at the third month of rituximab treatment, an increase in hemoglobin and platelet values was observed. Reticulocyte percent and haptoglobin were within normal limits. His follow-up as an outpatient is continuing. In most of the reported cases of TMA associated with ANCA-associated vasculitis, TMA appeared in the course of active vasculitis. Our case is noteworthy due to the fact that TMA developed after the active phase of GPA, even after the initiation of potent remission induction therapy. [ABSTRACT FROM AUTHOR]

A 20 year old woman presented with right arm pain. Pulses of right upper extremity were weak, acute phase reactants were elevated and MR angiography demonstrated total occlusion of subclavian artery and right axillary artery with collaterals. The diagnosis was Takayasu arteritis and she was treated with prednisolone, azathioprine and acetylsalicylic acid. During follow up, azathioprine was switched to methotrexate. Three years later, patient presented with elevated blood pressure. CT angiography demonstrated reduced calibration of the aorta and almost total occlusion of the lumen of proximal parts of left and right renal arteries. C-reactive protein was elevated. Steroid dose was increased, methotrexate was discontinued and IV tocilizumab and antihypertensive medications were initiated. One month later, she presented to emergency department with elevated blood pressure and blurred vision in the left eye. Fundoscopic examination revealed bilateral grade 3 hypertensive retinopathy and serous detachment of retina in the left eye. Laboratory results revealed normal CRP, elevated creatinine, elevated lactate dehydrogenase, thrombocytopenia, low hemoglobin and low haptoglobin. Peripheral blood smear revealed 2– 3 schistocytes in every field. She was admitted to rheumatology department with the diagnosis of thrombotic microangiopathy secondary to malignant hypertension. IV tocilizumab was administered, and methylprednisolone was maintained at a dose of 20 mg/day. Despite treatment with maximum dose of six antihypertensive medications, her blood pressure was not controlled adequately and she became hypervolemic. After undergoing ultrafiltration, balloon dilation was performed in the left renal artery, and a stent was placed there. After stent placement, creatinine and platelet count normalized, hemoglobin increased and hypertension was controlled. In this case, malignant hypertension which was triggered by bilateral renal artery stenosis due to Takayasu arteritis had caused acute kidney injury and advanced stage hypertensive retinopathy. In addition, unlike other Takayasu arteritis cases with malignant hypertension, thrombotic microangiopathy was also detected. [ABSTRACT FROM AUTHOR]