Internal medicine
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Case Reports
A Case of Demyelinating Neuropathy with Markedly Elevated Serum IgG4 Levels and Anti-Contactin 1 IgG4 Antibody.
We herein report a 77-year-old man with a 4-month history of progressive gait and sensory disturbances of the extremities. A nerve conduction study indicated demyelinating polyneuropathy. ⋯ Treatment with steroids resulted in an amelioration of functional status, improvement of nerve conduction parameters, decreased serum IgG4 levels, and negative conversion of anti-contactin 1 antibody. Further studies are needed to clarify the significance of IgG4-positive plasma cell infiltration in anti-contactin 1 antibody-positive neuropathies.
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Case Reports
Schnitzler Syndrome Presenting as a Fever of Unknown Origin with Elevated Alkaline Phosphatase Levels.
Schnitzler syndrome (SchS) is a rare, acquired, autoinflammatory disease that is sometimes associated with a fever of unknown origin (FUO). Elevated alkaline phosphatase (ALP) stemming from abnormal bone remodeling is a characteristic laboratory finding of SchS and is included in the diagnostic criteria. However, its utility as a clue to the diagnosis of SchS has been under-emphasized. We herein report a case of SchS presenting with a FUO and highly elevated ALP concentration, which led to repeated, unnecessary liver biopsies.
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Review Case Reports
Severe Enteritis after Cyclophosphamide Administration in a Patient with Microscopic Polyangiitis: A Case Report and Literature Review.
Severe enteritis is a rare side effect of cyclophosphamide (CPA) therapy, and only two cases have been reported to date. We herein report a 60-year-old man who developed severe enteritis after intravenous CPA administration for microscopic polyangiitis. ⋯ A diagnosis of CPA-associated enteritis was made based on the clinical course and imaging and pathological findings. This review of three cases of CPA-related enteritis, including our case, suggests that prompt CPA discontinuation and intensive systemic management are necessary when patients have gastrointestinal symptoms after CPA administration.
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A 59-year-old man with a high level of antinuclear antibody received nivolumab and ipilimumab plus chemotherapy for lung cancer. Two weeks after the second course, he was admitted with a fever and severe fatigue. ⋯ However, his cardiac function rapidly worsened, and he died on the fifth day after admission. There is no established treatment strategy for fulminant myocarditis as an irAE, and the further exploration of viable treatment strategies is required.