Clinically amyopathic dermatomyositis with interstitial lung disease double-positive for anti-MDA5 and anti-PL12 antibodies

Hiramatsu, Toshiya; Murano, Moeko; Nakai, Shogo; Murakami, Yurina; Nishimoto, Koji; Matsushima, Sayomi; Harada, Masanori; Uto, Tomohiro; Sato, Jun; Imokawa, Shiro; Suda, Takafumi

doi:10.1016/j.rmcr.2022.101606

Cited by 3 publications

(3 citation statements)

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“…Most responded to these treatments and showed good prognosis, except for two who died of respiratory failure despite intensive immunosuppressive therapy. Hiramatsu T ( 11 ) reported on a female case with anti-MDA5+/ARS+ DM, who was initially refractory to glucocorticoids combined with tacrolimus; her condition was subsequently stabilized by IVCY. Therefore, glucocorticoids combined with one or more immunosuppressants may offer a basic treatment approach in cases of anti-MDA5+/ARS+ DM.…”

Section: Discussionmentioning

confidence: 99%

“…We analyzed five published case reports (7)(8)(9)(10)(11). Their characteristics are summarized in Table 3.…”

Section: Literature Reviewmentioning

confidence: 99%

“…The coexistence of anti-MDA5 and anti-ARS antibodies is very rare; in fact, they are believed to be mutually exclusive ( 1 ). Very few such cases have been reported ( 7 – 11 ). In this study, we attempted to identify cases that are positive for anti-MDA5 and anti-ARS antibodies and explore the clinical features and prognosis of dermatomyositis in these individuals.…”

Section: Introductionmentioning

confidence: 99%

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The clinical features and prognoses of anti-MDA5 and anti-aminoacyl-tRNA synthetase antibody double-positive dermatomyositis patients

et al. 2022

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ObjectiveTo explore the clinical features and prognoses of dermatomyositis (DM) associated with a double-positive anti-MDA5 and anti-aminoacyl-tRNA synthetase (anti-ARS) antibody presentation.MethodsWe retrospectively analyzed 1280 consecutive patients with idiopathic inflammatory myopathy (IIM). Individuals with anti-MDA5 and anti-ARS antibodies (anti-MDA5+/ARS+) were compared to anti-MDA5-/ARS+ and anti-MDA5+/ARS- control individuals based on clinical, pulmonary radiological characteristics, treatment, and follow-up information.ResultsSix individuals (0.47%) presented with anti-MDA5+/ARS+; of these, 2 (33.3%) were anti-PL-12+, 2 (33.3%) were anti-Jo-1+, 1 (16.7%) was anti-EJ+, and 1 (16.7%) was anti-PL-7+. Hallmark cutaneous manifestations, including Gottron’s sign (100%), heliotrope rash (50%), mechanic’s hand (66.7%), and skin ulcers (16.7%) were common. Anti-MDA5+/ARS+ patients tended to have higher ferritin levels (p = 0.038) than anti-MDA5-/ARS+ group, and higher CD4+ T-cell counts (p = 0.032) compared to the anti-MDA5+/ARS- group. Radiologically, NSIP with OP overlap was predominant (60%). Consolidation (60%), ground-glass attenuation (GGA) (80%), traction bronchiectasis (80%), and intralobular reticulation (100%) were common in anti-MDA5+/ARS+ individuals. All were diagnosed with ILD and 50% were categorized as RPILD. All patients received glucocorticoids combined with one or more immunosuppressants. Most (83.3%) had a good prognosis following treatment, but there was no difference in the survival rate between the three subgroups.ConclusionPresentation with anti-MDA5+/ARS+ DM was rare. The clinical and radiological characteristics of anti-MDA5+/ARS+ DM combined the features of anti-MDA5+ and anti-ARS+ individuals. Individuals with anti-MDA5+/ARS+ antibodies may respond well to glucocorticoid therapy; glucocorticoids combined with one or more immunosuppressants may be considered a basic treatment approach.

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Section: Discussionmentioning

confidence: 99%

“…We analyzed five published case reports (7)(8)(9)(10)(11). Their characteristics are summarized in Table 3.…”

Section: Literature Reviewmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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The clinical features and prognoses of anti-MDA5 and anti-aminoacyl-tRNA synthetase antibody double-positive dermatomyositis patients

et al. 2022

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Prednisolone

2022

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Hidden in Plain Sight: Pitfalls of a Case of Amyopathic Anti-Synthetase Syndrome Associated with Grawitz Tumor

Dinu,

Prie,

Evsei

et al. 2024

Internal Medicine

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Anti-synthetase syndrome (ASS) is a rare autoimmune disease whose hallmark consists of the presence of autoantibodies directed against aminoacyl transfer RNA (tRNA) synthetases thus making it a distinctive pathology of the broad spectrum of idiopathic inflammatory myopathies (IIM). Clinically, lung involvement in the form of interstitial lung disease (ILD) and myositis are typical findings. Inflammatory myopathies should always raise the suspicion of an adjancent tumoral process and therefore prompt the clinician to perform a thorough screening. We present the case of a 55 year old caucasian male who was diagnosed with amyopathic ASS and a clear cell renal carcinoma (Grawitz tumor), along with the consequent therapeutic challenges.

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Clinically amyopathic dermatomyositis with interstitial lung disease double-positive for anti-MDA5 and anti-PL12 antibodies

Cited by 3 publications

References 23 publications

The clinical features and prognoses of anti-MDA5 and anti-aminoacyl-tRNA synthetase antibody double-positive dermatomyositis patients

The clinical features and prognoses of anti-MDA5 and anti-aminoacyl-tRNA synthetase antibody double-positive dermatomyositis patients

Prednisolone

Hidden in Plain Sight: Pitfalls of a Case of Amyopathic Anti-Synthetase Syndrome Associated with Grawitz Tumor

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