Background: Acquired idiopathic generalized anhidrosis (AIGA) is a rare disorder primarily observed in Asian populations, particularly in Japan. Although pulse methylprednisolone therapy is an effective treatment for AIGA, predictors of therapeutic response remain poorly defined.
Objectives: To identify factors that predict the efficacy of pulse methylprednisolone therapy in patients with AIGA.
Methods: Data obtained from 32 patients with AIGA were assessed based on clinical, histopathological and serological examinations. Statistical analyses were conducted to explore predictors of response to pulse methylprednisolone therapy.
Results: The average age of participants was 32.1 years (SD 12.3); 66% were male and 34% female. The response to pulse methylprednisolone therapy was closely associated with the time from the onset to start of therapy (Wilcoxson's rank sum test, P = 0.02, n = 27), with earlier intervention resulting in a better outcome. Notably, male patients and patients presenting with severe symptoms at diagnosis responded better to treatment. High serum carcinoembryonic antigen (CEA) levels and histological evidence of inflammation around sweat glands also correlated with a positive therapeutic response.
Conclusions: Earlier intervention, elevated serum CEA levels and inflammation around sweat glands are potential indicators of a successful response to pulse methylprednisolone therapy in patients with AIGA.
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