RESUMEN

Eosinophilic myocarditis is a type of inflammatory cardiomyopathy characterized by eosinophilic infiltration into myocardial tissue. The accurate myocarditis incidence rate is difficult to determine because of the clinical limitations of an endomyocardial biopsy. The primary pathogenesis of eosinophilic myocarditis is the release of related substances by eosinophils, leading to cell membrane damage and cell destruction. However, evidence suggests that specific genes play a role in myocarditis development.As CMR imaging availability increases, the diagnosis rate of eosinophilic myocarditis will increase. The diagnosis of myocarditis mainly depends on an endocardial biopsy. Glucocorticoids can relieve patients' symptoms, but the early use of steroids may prevent intermediate disease stage development (i.e., thrombonecrosis and fibrosis with wall thrombosis). Anticoagulant therapy may also affect disease development. In addition to routine follow-up, a regular myocardial biopsy should be considered for discharged patients, if possible.

RESUMEN

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) constitutes a group of blood vessel inflammation diseases of autoimmune origin. Myeloperoxidase (MPO) ANCA is closely related to ANCA associated AAV. The MPO-ANCA positive AAV patients have lung involvement at high rates; however, there are only a few reported cases with organizing pneumonia (OP). A 78-year-old man was presented to our hospital due to a fever of 38 °C despite a whole month of antibiotics treatment. Chest computed tomography image revealed restricted consolidations visible in the middle lobe of the right lung and the upper lobe of the left lung, which suggested an OP pattern. MPO-ANCA and urine occult blood tests were positive. Histopathological examination of the transbronchial biopsy revealed OP and mucus plug. Histological findings on renal biopsy showed necrotizing glomerulonephritis related to AAV. The patient was diagnosed with MPO-ANCA positive AAV and was treated with systemic corticosteroid therapy, from which he recovered rapidly. Thus, when diagnosing OP, the possibility of AAV should be considered by ordering patients' serum ANCA and occult hematuria tests.

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We present the case of a patient with granulomatous endocarditis of the mitral valve leading to severe valve stenosis caused by granulomatosis with polyangiitis. Endocarditis is a rare complication of granulomatosis with polyangiitis that may be misdiagnosed as infectious endocarditis or, as in our case, thrombotic lesions. (Level of Difficulty: Intermediate.).

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Anti-Neutrophil Cytoplasmic Antibody (ANCA)-negative Rapidly Progressive Glomerulonephritis (RPGN) is a severe form of autoimmune renal injury with a bleak prognosis. A 60-year-old Indian woman was treated with classical homeopathy for ANCA-negative RPGN, and after one year of treatment, serum creatinine and other parameters indicating renal injury dropped steadily despite the withdrawal of immunosuppressive drugs; renal dialysis, which was conducted twice a week initially, was made rarer and stopped after one year. Classical homeopathy may be considered a potential therapeutic modality in severe pathologies. Controlled studies are required to establish further the extent to which classical homeopathy may relieve patients from procedures such as dialysis that cause considerable physical and economic discomfort.

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RESUMEN

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is rare in children and is characterised as necrotising vasculitis predominantly affecting small and medium-sized vessels. Propylthiouracil (PTU), an antithyroid drug, has been implicated in drug-induced AAV. In contrast, Kawasaki disease (KD) is a common systemic vasculitis, typically observed in children, which affects the medium-sized vessels, including the coronary arteries. An 11-year-old girl who developed AAV while receiving PTU therapy for Graves' disease is described. She was admitted to hospital following a 2-day history of fever, cervical adenopathy, cheilitis and papular rash, 3 weeks after an increase in the PTU dose. Despite discontinuation of PTU and the administration of intravenous antibiotic therapy, her clinical condition deteriorated and over the next 2 days she developed severe diarrhoea, conjunctival injection and swelling and redness of the right index finger. Additional findings included liver dysfunction, hydrops of the gallbladder, coagulopathy and urine abnormalities, suggesting glomerulonephritis. She met the diagnostic criteria for KD and received intravenous immunoglobulin (IVIG) combined with prednisolone, with rapid resolution of clinical and laboratory parameters. Peeling of the right index fingertip became evident on Day 12 of admission. Serial ultrasound cardiography demonstrated no evidence of cardiac involvement. A high titre of myeloperoxidase ANCA was detected in the patient's serum on admission, and the titre decreased during the convalescent stage. This case demonstrates that children with PTU-associated AAV may present with clinical features mimicking KD, and that IVIG along with corticosteroid therapy may be effective in treating patients with drug-induced severe systemic AAV.

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BACKGROUND: Acute exacerbation of Autoimmune Hepatitis (AIH) poses a significant challenge for diagnosis as it can mimic acute viral hepatitis especially in absence of autoantibodies and hypergammaglobulinemia. AIM: To determine the clinical, laboratory, histopathological characteristics and response to treatment in AIH patients with acute exacerbation. METHODS: A retrospective analysis of 16 patients with acute exacerbation of AIH diagnosed over a period of eight years (2008-2016). RESULTS: Out of the 111 patients diagnosed with AIH, acute exacerbation of AIH was diagnosed in 16 (14.4%) patients. All patients were females with median age of 35 years. Nine patients (56%) had Type 1 AIH and seven (44%) patients were diagnosed with seronegative AIH. All 16 (100%) patients had acute viral hepatitis like illness at presentation. The median bilirubin was 4.2 mg/dl (range, 2.2-20), aspartate transaminase was 568 IU/L (range, 390-908), alanine transaminase was 430 IU/L (range, 257-1026) and serum alkaline phosphatase was 395 IU/L (range, 112-890) during symptomatic period. The histopathological examination showed underlying chronic hepatitis in 10 (71.4%) patients, only fibrosis in 2 (14.2) patients and cirrhosis with activity in 2 (14.2%). All 16 (100%) patients were treated with a combination of steroids and azathioprine. Thirteen (81%) patients achieved complete biochemical remission and three (19%) patients achieved partial remission out of which one (6%) patient succumbed to illness because of the complications of cirrhosis. CONCLUSION: A suspicion of acute exacerbation of AIH should be considered in patients with unexplained acute hepatitis mimicking acute viral hepatitis in the absence of positive viral markers. Through evaluation with immunoserological markers and liver biopsy can clinch the diagnosis of acute exacerbation of AIH in such cases.

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Histoplasma capsulatum is the most common endemic mycosis worldwide. Although most of the globe's largest urban hubs fall outside this organism's regions of endemicity, clinicians practicing in a metropolis like New York City or Los Angeles must nevertheless remain vigilant for histoplasmosis because of the large immigrant population that is served by its hospitals. H. capsulatum infection ranges from asymptomatic pulmonary infection to life-threatening diffuse pneumonia with dissemination. The early years of the AIDS epidemic first introduced U.S. clinicians working in areas previously unfamiliar with histoplasmosis to newly immunocompromised patients from endemic regions presenting with disseminated H. capsulatum originally acquired in their home countries. Improvement in HIV prevention and therapeutics has reduced the frequency of such cases. Herein we report three cases of histoplasmosis encountered in our New York City institution over the last three years to emphasize that awareness of this infection remains mandatory for the frontline urban clinician.

RESUMEN

Progressive narrowing of the central airways due to diffuse inflammation is a potential life-threatening condition. A number of diseases have been described as possible causes. We present two siblings with severe central airway obstruction. Despite considerable efforts we have not been able to match the clinical appearance of our patients with the diagnostic criteria of any of the disease entities known to cause this condition.