Anti‐nuclear matrix protein 2 antibody‐positive dermatomyositis with gastrointestinal ulcers: A case report

Abstract

Gastrointestinal manifestations are a very rare complication of dermatomyositis (DM) and are much less frequent in adult cases than in juvenile cases. Only a few previous papers have reported adult patients who had DM with anti‐nuclear matrix protein 2 (anti‐NXP2) antibodies and who developed gastrointestinal ulcers. Herein, we report a similar case of a 50‐year‐old man who had DM with anti‐NXP2 antibodies followed by relapsing multiple gastrointestinal ulcers. Even after the administration of prednisolone, his muscle weakness and myalgia deteriorated and gastrointestinal ulcers relapsed. In contrast, intravenous immunoglobulin and azathioprine improved his muscle weakness and gastrointestinal ulcers. Based on the parallel disease activity of the muscular and gastrointestinal symptoms, we considered that his gastrointestinal ulcers were a complication of DM with anti‐NXP2 antibodies. We also propose that early intensive immunosuppressive therapy would be required for the muscular and gastrointestinal symptoms in DM with anti‐NXP2 antibodies.

Juvenile dermatomyositis with central nervous system involvement: two case reports from a retrospective single-center cohort, with literature review

“…In our center, both patients were anti-NXP2 antibody-positive. Previous studies on anti-NXP2 antibody-positive JDM have primarily focused on clinical features like calcinosis, severe muscle involvement, gastrointestinal vasculitis, and swallowing difficulties, with poor prognosis (22,(32)(33)(34). The CNS involvement observed in our two patients who were anti-NXP2 antibody-positive represents a relatively new phenotype.…”