Internal medicine
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Case Reports
A Case of Anti-synthetase Syndrome that Relapsed with Pulmonary Arterial Hypertension and Malignancy.
A 69-year-old man with a history of anti-synthetase antibody-positive polymyositis and interstitial lung disease (ILD) stable for more than 20 years suddenly developed pulmonary artery hypertension (PAH) with a mean PA pressure of 46 mmHg. At this stage, ILD was mild, but it became acutely exacerbated later, and high-dose corticosteroid and intravenous cyclophosphamide ameliorated both PAH and ILD. ⋯ During a systemic examination, bone metastatic cancer of unknown origin was found. We herein report the relationship between anti-synthetase syndrome (ASS) and PAH as well as ASS and malignancy.
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Case Reports
Focal Segmental Sclerosis Associated with the Novel Multi-tyrosine Kinase Inhibitor Ponatinib: A Case Report.
Ponatinib is a novel multi-tyrosine kinase inhibitor (TKI) with potent inhibitory activity against refractory chronic myeloid leukemia (CML). Despite its high clinical efficacy, ponatinib induces various adverse events due to its multi-target characteristic. ⋯ Our patient developed proteinuria and renal function deterioration during treatment with ponatinib but not with imatinib or nilotinib. We herein report the first case of a patient with secondary focal segmental glomerulosclerosis (FSGS) with partial glomerular collapse induced by ponatinib treatment.
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Case Reports
Advanced Appendiceal Cancer with Systematic Metastasis without Gastrointestinal Symptoms Found by Subcutaneous Tumor.
An 86-year-old woman with a subcutaneous nodule in her left axilla visited our hospital. She had no gastrointestinal symptoms, but contrast-enhanced computed tomography revealed a cecal mass and systemic metastasis, including cutaneous, bone, peritoneal dissemination and ascites. Colonoscopy revealed a circumferential, elevated cecal lesion. ⋯ The pathological diagnosis was poorly differentiated adenocarcinoma (por1>tub2>muc) arising from the appendix with a BRAFV600E mutation and microsatellite instability-high. Chemotherapy was administered, and she is currently still alive and undergoing chemotherapy. We describe a rare case of advanced appendiceal cancer without gastrointestinal symptoms diagnosed due to cutaneous metastasis.
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We performed 3 kidney biopsies in a 71-year-old man. At the first biopsy, we made the diagnosis of immunoglobulin G4 (IgG4)-related interstitial nephritis characterized by the simultaneous presence of IgG4-positive plasma cells and characteristic fibrosis with a bird's-eye pattern. ⋯ The third biopsy showed the recurrence of the disease with inflammatory cells accompanied by fibrosis. These findings suggest that IgG4-positive plasma cells and fibrosis occur simultaneously.
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Case Reports
A Case of Antiphospholipid Syndrome Nephropathy with Acute Thrombotic Microangiopathy After Renal Transplantation.
We experienced a 36-year-old man with lupus nephritis and antiphospholipid syndrome (APS) who received a donor kidney from his father. Twenty-two months after transplantation, at a time of poor adherence to immunosuppressants and warfarin, the patient developed sudden graft loss due to hemolytic uremic syndrome with rapid deterioration of renal function, thrombocytopenia, and hemolytic anemia. A kidney biopsy showed thrombotic microangiopathy (TMA) related to platelet thrombus formation; however, there was no recurrence of lupus and no findings suggestive of post-transplant rejection, so acute TMA associated with APS was thought to be the cause of the graft loss. This case highlights the importance of instructing patients with lupus nephritis to adhere to treatment with warfarin, a therapeutic drug for APS.