Infective endocarditis (IE) is more common in patients with predisposing cardiac lesions and has many potential complications, including stroke and arterial thromboembolisms. Renal manifestations have an estimated prevalence of ∼20%. Rapidly progressive glomerulonephritis (RPGN) is a nephrological emergency manifested by autoimmune-mediated progressive loss of renal function over a relatively short period of time. Here, we report the case of a 60-year-old Caucasian male, who presented with speech impairment and was found to have multiple embolic strokes caused by aortic valve IE. His renal function declined rapidly, and his urine sediment featured hematuria and proteinuria. ANCA titer was negative by immunofluorescence (IF); however, the PR3 antibody was elevated. The renal biopsy revealed pauci-immune focally necrotizing glomerulonephritis with the presence of ∼25% cellular crescents. He was initially treated with plasmapheresis and pulse dose steroids. Hemodialysis was initiated for uremic symptoms. After four weeks of antibiotic therapy and with blood cultures remaining negative, he was treated with rituximab. Two months after discharge, his renal function showed improvement, and hemodialysis was discontinued. This case highlights several complications associated with lactobacillus endocarditis including RPGN.
Sclerosing thymoma is an extremely rare neoplasm with only 16 reported [documented] cases worldwide in literature till date. The patient in our case presented with Sclerosing thymoma WHO Type A as well as clinical findings suggestive of Isaac's-Merten's syndrome (Neuromyotonia). CASE PRESENTATION: The Patient is a 53-year-old Caucasian male former smoker who presented with worsening dyspnea on exertion, and productive cough onset two weeks prior to presentation. He also complained of unintentional weight loss, generalized weakness, and progressively worsening bilateral hand contractures over the last year. On physical examination, the patient was noted to have contracted fingers on both hands with impaired flexion and extension. CXR and CT chest findings were consistent with pneumonia and an incidental large anterior 11.1 cm and 8.4 cm necrotic mediastinal mass that enveloped the main pulmonary artery. CT guided biopsy was performed and the mass was found to be of thymic origin. Neoadjuvant chemotherapy and radiation therapy were administered outpatient for tumor shrinkage prior to resection. The patient later underwent complete thymectomy and wedge resection of the left lung upper lobe. Pathology and immunohistochemistry results confirmed definitive diagnosis of sclerosing thymoma WHO Type A pattern. DISCUSSION: Sclerosing thymoma is an extremely rare type of thymoma accounting for <1% of all thymomas with preoperative clinical presentations that are widely varied. Kuo reported the first two cases of sclerosing thymoma in 1994. The WHO histologic classification of thymomas in 2004 entails 5 subtypes A, A1, B1, B2 and B3. These main subtypes are distinguished from the other rare variants of thymoma (sclerosing, micronodular, metaplastic, microscopic and lipofibroadenoma). Till date, only 16 cases of this sclerosing variant have been reported in literature worldwide. Although previously considered benign, even low-grade thymomas with type A pattern are considered malignant per International Thymic Malignancy Interest Group (ITMIG) guidelines. Certain paraneoplastic syndromes including the Isaac's-Merten's syndrome (Neuromyotonia) have been reported in association with thymomas on literature review. Neuromyotonia also known as peripheral nerve hyperexcitability is characterized by muscle stiffness as evidenced by bilateral hand contractures in our patient. CONCLUSIONS: Thymomas are rare mediastinal tumors. Sclerosing thymoma WHO type A is a very rare variant with few cases reported. Careful follow up of patients with thymic tumors is recommended to detect any local recurrence or distant metastasis.
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