Gastrointestinal Perforation in a Patient With Antinuclear Matrix Protein 2 Antibody–Positive Dermatomyositis

Valenzuela, Antonia; Rieger, Kerri E.; Blish, Catherine A.; Chung, Lorinda; Fiorentino, David

doi:10.1002/acr.24879

Cited by 1 publication

(7 citation statements)

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“…Concerning therapy, the patients of 2 previous cases, who developed severe gastrointestinal ulcers with perforation, required intensive therapy such as IVIG and immunosuppressants and showed a therapeutic response (Table 1, cases 1 and 2). 3,4 Our patient also needed IVIG and azathioprine, which were effective for improvement of both muscular and gastrointestinal symptoms. Therefore, intensive immunosuppressive therapy seems to be useful for patients with gastrointestinal ulcers in DM with anti-NXP2 antibodies.…”

Section: Discussionmentioning

confidence: 93%

“…2 Myositis-specific autoantibodies have not been described in most cases, but, recently, 2 cases of DM with anti-NXP2 antibodies were reported (Table 1, cases 1 and 2). 3,4 Similarly, 5 cases of juvenile DM with anti-NXP2 antibodies were documented in case reports (Table 1, cases 3-7). 6 Moreover, in a case series of 430 juvenile DM patients, the patients with anti-NXP2 antibodies had the highest incidence of gastrointestinal ulcers at 7% (6/86 patients) compared with the patients with other myositis-specific autoantibodies.…”

Section: Discussionmentioning

confidence: 96%

“…A previous study proposed that the gastrointestinal ulcers of DM might be induced by vasculitis 2 . Indeed, the previous 2 cases of DM with anti‐NXP2 antibodies showed vasculitis in the pathology of gastrointestinal ulcers (Table 1, cases 1 and 2) 3,4 . However, vasculitis was not proven in 3 cases of DM with severe gastrointestinal ulcers, in which the patients' myositis‐specific autoantibodies were unknown 8 .…”

Section: Discussionmentioning

confidence: 98%

“…Among gastrointestinal manifestations of DM, including juvenile cases, ulcers have been most frequently reported, followed by intestinal pneumatosis, intestinal wall thickening, and macroscopic inflammation 2 . Myositis‐specific autoantibodies have not been described in most cases, but, recently, 2 cases of DM with anti‐NXP2 antibodies were reported (Table 1, cases 1 and 2) 3,4 . Similarly, 5 cases of juvenile DM with anti‐NXP2 antibodies were documented in case reports (Table 1, cases 3–7) 6 .…”

Section: Discussionmentioning

confidence: 99%

“…2 Indeed, the previous 2 cases of DM with anti-NXP2 antibodies showed vasculitis in the pathology of gastrointestinal ulcers (Table 1, cases 1 and 2). 3,4 However, vasculitis was not proven in 3 cases of DM with severe gastrointestinal ulcers, in which the patients' myositis-specific autoantibodies were unknown. 8 According to previous cases and our case, gastrointestinal ulcers might not always be induced by vasculitis.…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2023

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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.

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Section: Discussionmentioning

confidence: 93%