A literature survey reveals that both lipid lowering drugs - statins and fibrates--and hypothyroidism are documented causes of muscle disorders including rhabdomyolysis leading to acute renal failure. We describe a case of fenofibrate monotherapy (Lipicard) induced dialysis dependent acute renal failure in an undiagnosed hypothyroid patient which is the first case to be reported from Sri Lanka. We strongly recommend that all patients who are receiving statins and/or fibrates should be screened for occult hypothyroidism which seems to aggravate the muscle damage due to the above drugs, with or without other risk factors.
Results: There were 342 (24%) upper GI bleeders. The mean age of presentation was 55.5 years +/-14.4 SD. A high proportion was seen between 50-70 years of age. Sex distribution was, male:female = 229:114 (2:1). 45% have presented with haematemesis, 31% with malaena, and 24% with both. Endoscopy showed the presence of severe antral gastritis and duodenitis, oesophageal varices, oesaphagitis, erosive gastropathy, portal hypertensive gastropathy, pangastritis and peptic ulcer disease in 38%, 35%, 28%, 28%, 20%, 17% and 15% of the instances respectively. Hiatus herniae were seen in 43% although its role was unclear. Often combined pathologies were seen. Only 52% of the varices showed endoscopic evidence of bleeding. Excess alcohol consumption was seen in 36% while 5% were on NSAIDs. 5% had endoscopic negatives.Conclusions: UGIB is a major problem to the endoscopist, constituting about 25% of the work load, in this part of the continent. Although approximately 50% of the varices found had bled, portal hypertension related pathologies are a cause for concern. Alcohol was a contributory factor in 1/3 of the endoscoped population. Bleeding from PUD was not a dominant feature compared to the west.
Dapsone-induced agranulocytosis is a rare but potentially fatal adverse effect of treatment for tuberculoid leprosy. Publications distributed by the WHO Leprosy Elimination Campaign for patient information on leprosy do not contain any advice or guidelines for post-dapsone therapy follow-up. As a result of this major deficiency, the local anti-leprosy campaign in Sri Lanka has no such guidelines on dapsone therapy for leprosy patients. We report two patients with total agranulocytosis caused by dapsone therapy for tuberculoid leprosy including one fatality. As leprosy is more prevalent in developing countries such as Sri Lanka, we recommend that WHO publications on patient information should include post-dapsone therapy follow-up guidelines to avoid such catastrophes which are undetected until the patients are critically ill.
BackgroundHemophagocytic lymphohistiocytosis is an aggressive life-threatening syndrome of excessive immune activation. Hemophagocytic lymphohistiocytosis due to systemic lupus erythematosus is described as acute lupus hemophagocytic syndrome. Acute lupus hemophagocytic syndrome presenting with negative antinuclear antibody is uncommon.Case presentationA 57-year-old Sri Lankan woman presented with intermittent fever, weight loss, episodic confusion, and alopecia for 3 months. Investigations showed pancytopenia. Her erythrocyte sedimentation rate was 76 mm/hour and C-reactive protein was 2 mg/l. Septic screen was negative except for a positive sputum culture for Gram-negative coliforms. Chest X-ray was normal. Direct antiglobulin test was positive. Fever persisted with clinical worsening despite treatment with intravenous antibiotics. Antinuclear antibodies and double-stranded-deoxyribonucleic acid antibodies were negative. Bone marrow aspiration revealed features compatible with hemophagocytosis. Her serum ferritin and triglycerides were elevated. Diagnosis of hemophagocytic lymphohistiocytosis due to an evolving autoimmune disorder was made and she was treated with steroids. She showed a dramatic improvement and was discharged on oral steroids.After 6 months, while the steroids were being tapered she experienced oral ulcers, frothy urine, and ankle swelling and she was rehospitalized. Urine analysis revealed proteinuria with active sediment. Antinuclear antibodies and double-stranded-deoxyribonucleic acid antibodies were positive. Complement C3 and C4 were reduced. A renal biopsy revealed class IV-G lupus nephritis with immunofluorescence pattern consistent with systemic lupus erythematosus. Steroid dose was increased and mycophenolate mofetil was commenced. She improved.ConclusionsThis case showcases an uncommon presentation of acute lupus hemophagocytic syndrome with initial negative antinuclear antibody probably due to its cytokine-mediated pathogenesis. This is the first such reported case in South Asia to the best of our knowledge. According to the American College of Rheumatology criteria, our patient did not fulfill the criteria for systemic lupus erythematosus diagnosis for the initial hospitalization. But, according to the 2012 Systemic Lupus International Collaborating Clinics criteria, she did fulfill the criteria for systemic lupus erythematosus even in the first hospitalization which was subsequently proven with renal biopsy findings. This case confirms the increased sensitivity of Systemic Lupus International Collaborating Clinics criteria over American College of Rheumatology criteria in diagnosis of systemic lupus erythematosus.
BackgroundLymphadenopathy is not an uncommon presentation of adult onset Still’s disease: it is present in up to two thirds of patients with adult onset Still’s disease. The characteristic appearance of lymphadenopathy is described as intense, paracortical immunoblastic hyperplasia. Changes in light microscopy may resemble lymphoma, but immunohistochemistry reveals a benign polyclonal B cell hyperplasia.Case presentationWe describe a 67-year-old Sri Lankan woman who manifested relapsing prolonged fever, raised inflammatory markers, arthralgia, myalgia, transient skin rash, and cervical lymphadenopathy histologically characterized by noncaseating granulomatous adenitis with central suppuration. Due to the fact of high prevalence of tuberculosis in the region, an extensive diagnostic evaluation was done to exclude the possibility of extrapulmonary tuberculosis; unsuccessful therapeutic trials of complete antituberculosis regime reliably excluded the possibility of tuberculosis and strengthened the diagnostic validity. Disease flares were characterized by systemic inflammatory response syndrome with immediate clinical and laboratory response to corticosteroids. After systematic diagnostic workup which ruled out possible malignant, rheumatic, or autoimmune diseases and infections previously described as causes of granulomatous adenitis, our patient was diagnosed as having adult onset Still’s disease based on Yamaguchi criteria. She required a trial of indomethacin followed by methylprednisolone pulse therapy and long-term maintenance steroid therapy without steroid-sparing immunosuppressive agents or biological disease-modifying antirheumatic drugs. She achieved full disease remission in 3 months. Reevaluation after 6 months and 1 year did not reveal residual disease activity.ConclusionsTo the best of our knowledge this is the first report of suppurative noncaseating granulomatous lymphadenitis attributed to adult onset Still’s disease among Asian or South Asian ethnicities and it is also rarely reported among Europeans and North Americans. It is an extremely challenging situation to diagnose Still’s disease with granulomatous lymphadenitis where tuberculosis is highly prevalent. This case highlights the importance of consideration of adult onset Still’s disease as a potential diagnosis in a compatible clinical context in the presence of noncaseating granulomatous adenitis and indicates that one should not be misled into a diagnosis of tuberculosis by the fact of the high prevalence of tuberculosis, however, the exclusion of other diagnoses is a prerequisite.
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