Digestive vasculitis: a rare but severe complication of NXP2 dermatomyositis

Tréfond, Ludovic; Smets, P.; Beltzung, Fanny; Olagne, L.; Bardy, A.; Aumaître, O.; André, M.

doi:10.1093/rheumatology/keaa740

Cited by 3 publications

(10 citation statements)

References 7 publications

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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: 89%

“…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%

“…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%

“…Gastrointestinal manifestations are a very rare complication of dermatomyositis (DM) 1,2 . DM with gastrointestinal involvement has been reported mostly in juvenile patients and less frequently in adult patients 2–4 . Severe gastrointestinal ulcers in adult DM might exhibit poor prognosis 2 .…”

Section: Introductionmentioning

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: 89%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

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

et al. 2023

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“…GI involvement may be present in 5% to 37% of all subjects with juvenile DM, but intestinal vasculopathy is seen in only 1% of adult-onset anti-NXP-2 antibody-associated DM (22,25). Individuals with adult-onset anti-NXP-2 antibody-associated DM often experience weakness of the distal upper (35%) and distal lower (25%) limbs in addition to classic proximal muscle involvement (26), which is a higher prevalence of distal muscle involvement observed compared to other DM subsets.…”

Section: Discussionmentioning

confidence: 99%

A Rare Manifestation of a Rare Disease: The Importance of Thinking Outside the Box in a Patient With Complex Dermatomyositis

Milne

Sims

Anderson

et al. 2022

Arthritis Care & Research

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

Yang,

Guan,

Liu

et al. 2024

Front. Pediatr.

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BackgroundJuvenile dermatomyositis (JDM) is a systemic autoimmune disease primarily involving the muscles and skin; it can also affect the central nervous system (CNS). The relevant literature provides limited information regarding the characteristics of JDM with CNS involvement.MethodWe reviewed patients with JDM who were hospitalized at our center between January 2016 and August 2023, with a focus on those with CNS involvement. The aim was to provide detailed case reports on these patients, and to summarize the relevant literature about the characteristics of similar cases.ResultsAmong 193 hospitalized patients with JDM, two (1.03%) had CNS involvement. Two patients, a 5.5-year-old girl and an 11-year-old boy, were admitted with severe proximal muscle weakness and seizures, and presented with active cutaneous vasculitis. Both were ultimately diagnosed with JDM, with CNS involvement. Both patients had confirmed presence of anti-NXP2 antibody through myositis-specific antibody analysis. Additionally, they all exhibited hyperferritinemia and thrombocytopenia. Salvage therapies like intravenous methylprednisolone (IVMP) pulse therapy and/or plasma exchange were administered successfully. At final follow-up, both patients had achieved complete clinical response and full neurological recovery. Our literature review identified nine similar case studies. CNS involvement usually occurred within the first 10 months of the disease course, and most of these patients had fatal outcomes, with a mortality rate of 66.6% (6/9). Including the two patients described herein, the median age for disease onset is 10.5 years (range 4–17 years), and the male: female ratio is 6:5. Seizures are the most common neurological symptom, accompanied by active cutaneous vasculitis. The brain biopsies showed two distinct pathological presentations: one was central nervous system vasculitis, and the other was cerebral macrophage activation syndrome.ConclusionsCNS involvement is a rare but life-threatening JDM complication. Herein, our cases and the literature indicate that it typically occurs within the first 10 months of the disease course and manifests as seizures, often accompanied by active cutaneous vasculitis, with fatal outcomes. Timely implementation of salvage therapies, like IVMP pulse therapy and plasma exchange, may significantly impact patient outcomes.

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Digestive vasculitis: a rare but severe complication of NXP2 dermatomyositis

Cited by 3 publications

References 7 publications

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

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

A Rare Manifestation of a Rare Disease: The Importance of Thinking Outside the Box in a Patient With Complex Dermatomyositis

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

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