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Inflammatory myopathies are characterized by the skeletal muscle inflammation leading to symptoms of myopathy along with varying involvement of other organs such as lung, skin and joints. The strong association between inflammatory myopathies and malignancy has been well recognized. Recently, âËcancer-associated myositis (CAM)â™, has been proposed to be a paraneoplastic syndrome due to the anti-tumor immunity secondary to similar tumor and regenerating muscle antigens. As the prognosis of myositis depends on the prognosis and treatment of the underlying malignancy, physicians must determine the degree of testing necessary to detect cancer both at myositis onset and thereafter. However, there are no clear guidelines regarding the best approach. Emerging medical evidence shows that identification of certain risk factors and serology patterns can be utilized to predict cancer risk in patients with myositis.
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Gastrointestinal bleeding from angiodysplasias in patients with aortic stenosis is termed as 'Heyde's syndrome'. We report a case of Heyde's syndrome successfully treated with trans-catheter aortic valve replacement.
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Light chain amyloidosis has very rarely been reported in association with chronic lymphocytic leukemia (CLL). We reported on a 76-years-old female who presented with simultaneous kappa-restricted chronic lymphocytic leukemia (CLL) and a lambda-restricted multiple myeloma with plasma cells causing AL amyloidosis involving the heart. While monoclonal immunoglobulins occasionallyproduced by CLL have previously been implicated in AL amyloidosis, there only a few cases reported of AL amyloidosis resulting from a distinct plasma cell dyscrasia that is not clonally related to the concurrent CLL.
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INTRODUCTION: Propylthiouracil (PTU)-induced antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis presenting with renal failure, acute hepatic failure, and cerebral angiitis is a rare yet fatal disease. Early diagnosis and management may help in reducing mortality and morbidity. Plasmapheresis and induction with either cyclophosphamide or rituximab is indicated. Understanding the pathophysiology and complex management of this disease poses challenges to clinicians. CASE REPORT: A 42-year-old woman presented with acute renal and hepatic failure. She had been on PTU for 11 months for Graves' disease. Initial urine microscopy showed red blood cell casts. Anti PR-3 antibodies were positive. Kidney biopsy revealed pauci-immune glomerulonephritis with crescent formation. Renal and hepatic failures were attributed to PTU-induced c-ANCA production as other serological workup was negative. Pulse steroids and plasmapheresis were initiated. Later she developed pneumonia. She was also given rituximab. After the first dose of rituximab, plasmapheresis was held for 3 days. The second dose of rituximab was given in 5 days owing to removal by plasmapheresis. She got 8 sessions of plasmapheresis. She also developed seizures and MRA of her head revealed cerebral infarct, with findings suggestive of cerebral angiitis. She did not recover and expired 20 days after presentation. CONCLUSION: PTU can cause ANCAassociated vasculitis resulting in multiorgan failure. Plasmapheresis should be held for 3 days after rituximab infusion in order to allow maximum exposure. The second dose of rituximab may be given before the recommended 7-day interval in cases in which plasmapheresis is being performed to maximize therapeutic benefit.
