Endocrine Abstracts

Introduction

Vasculitis with antineutrophilic cytoplasmic antibodies (ANCA) has been reported in patients treated with anti-thyroid drugs, especially Propylthiouracil and exceptionally with benzylthiouracil (BTU). We present here a case of ANCA-anti-MPO-associated vasculitis related to BTU.

Case presentation

A 46 old women was treated with BTU during 18 months for Basedow’s disease. She showed abruptly an alteration of the general state with fever, hepatosplenomegaly, cytopenia with microcytic anemia at 6.8 g/dl and leucolymphopenia at 2500, proteinuria between 0.4 and 0.8 g/24 h with normal kidney function and biological inflammatory syndrome. A chest-abdominal-pelvic CT showed hepatosplenomegaly with splenic infarction. The p- ANCA assay was positive with anti-myeloperoxidase specificity. The benzylthiouracil was discontinued. The patient was treated with bolus of solumedrol relayed by corticosteroids at a dose of 1 mg/kg per day with disappearance of bicytopenia and regression of hepatosplenomegaly. She reconsults few weeks later for respiratory failure, with crackling rattles in pulmonary auscultation, anemia at 5.4 g/dl and images of alveolar condensation to chest radiography. The diagnosis of intra-alveolar hemorrhage was retained. She was treated with solumedrol bolus. One month later, she developed a high-abundance hemoptysis with hemodynamic failure and death.

Conclusion

Because of the gravity of this complication, clinical monitoring is recommended in patients taking benzylthiouracil. If vasculitis develops, the anti-thyroid drug should be discontinued and corticosteroid treatment, with immunosuppressors in some cases, is initiated. Prognosis is less severe than primary ANCA vasculitis, and death due to anti-thyroid therapy-induced AAV as our case is exceptional, related generally to severe alveolar hemorrhage.