Internal medicine
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We herein report three cases of microscopic polyangiitis (MPA). Two patients were administered avacopan in combination with glucocorticoid (GC), whereas one patient was treated with avacopan monotherapy; none of the patients were co-administered either rituximab or cyclophosphamide. The doses of GC were successfully reduced after the introduction of avacopan in the two patients, and the serum C-reactive protein levels decreased in the patient treated with avacopan monotherapy. Avacopan may therefore be effective either in combination with GC or as monotherapy, even for patients at a high risk of developing adverse effects when administered rituximab or cyclophosphamide.
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A 71-year-old woman developed nephrotic syndrome during 10-year follow-up for chronic lymphocytic leukemia. A renal biopsy sample analysis revealed IgG1-lambda-positive monoclonal immunotactoid glomerulopathy (mITG). ⋯ In particular, mITG, which presents immune deposits that exhibit light-chain restriction, is often associated with hematologic disorders. Most patients with mITG receive immunosuppressive therapy and/or chemotherapy; however, to our knowledge, there have been no reports of treatment with ibrutinib.
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A delayed diagnosis of polyarteritis nodosa may lead to critical limb-threatening ischemia (CLTI). A 74-year-old woman presented with left-foot pain and was treated with oral vasodilators and antiplatelet agents. ⋯ Based on the progressive distal gangrene, mononeuropathy multiplex, and pathological findings of necrotic vasculitis, polyarteritis nodosa was diagnosed, and the patient's condition improved. A biopsy and neurological examination are essential for the appropriate diagnosis of PAN and immediate treatment.
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A 73-year-old man who presented with nonspecific general symptoms and cognitive impairment was initially diagnosed with mild cognitive impairment due to dementia with Lewy bodies (DLB) based on a reduced blood flow in the parietal and occipital lobes on single-photon emission computed tomography (SPECT) imaging. However, the patient later presented with hyponatremia and hypoglycemia, leading to impaired consciousness, and was diagnosed with isolated adrenocorticotropic hormone deficiency (IAD). Hydrocortisone treatment improved the blood test scores and general symptoms, including cognitive impairment. IAD may show a DLB-like presentation on cerebral blood flow SPECT; therefore, caution is required for the correct diagnosis of IAD.
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A 54-year-old woman with persistent fatigue and a high fever presented with central pontine myelinolysis (CPM) on brain imaging, despite the absence of neurological symptoms. The patient had a slightly low serum sodium concentration. ⋯ CPM was successfully resolved after splenectomy and immunochemotherapy. This is the first reported case of CPM in a patient with PS-DLBCL confirmed by a pathological diagnosis following splenectomy and BAEP-detected abnormalities that improved with immunochemotherapy.