Anti-melanoma differentiation–associated gene 5 (MDA5) dermatomyositis: A concise review with an emphasis on distinctive clinical features

Kurtzman, Drew J.B.; Vleugels, Ruth Ann

doi:10.1016/j.jaad.2017.12.010

Cited by 159 publications

(241 citation statements)

References 59 publications

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“…Arthritis and arthralgia occur frequently in anti-MDA 5 positive CADM [10]. The arthritis is typically symmetric and affects the small joints of the hands, resembling rheumatoid arthritis (RA).…”

Section: Discussionmentioning

confidence: 99%

A case of anti-melanoma differentiation-associated gene 5 antibody-positive interstitial lung disease complicated with tracheobronchial ulcers

Tsumiyama

Yamakawa

Sato

et al. 2018

Respiratory Medicine Case Reports

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We herein report the first case, to our knowledge, of tracheobronchial ulcer with anti-melanoma differentiation-associated gene 5 (anti-MDA 5) antibody-positive interstitial lung disease (ILD). A 53-year-old man complained of shoulder and wrist pain and was suspected of having polymyalgia rheumatica at another hospital. Thereafter, treatment with prednisolone was started. Although his arthralgia improved, he suffered from progressive dyspnea on exertion and an abnormal shadow was noted on chest X-ray, so he was transferred to our hospital. Chest computed tomography scan revealed ground-glass opacities and intralobular septal thickening. We diagnosed him as having clinically amyopathic dermatomyositis associated with ILD based on the specific skin findings and elevated anti-MDA 5 antibody titer. Fiberoptic bronchoscopy showed ulcerations of the trachea and bronchus. Treatment with dose increments of prednisolone combined with other immunosuppressive drugs resulted in improvement of his respiratory condition and tracheobronchial lesions. Clinicians should be aware that tracheobronchial ulcers can be associated with anti-MDA 5 antibody-positive interstitial lung disease.

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Section: Discussionmentioning

confidence: 99%

A case of anti-melanoma differentiation-associated gene 5 antibody-positive interstitial lung disease complicated with tracheobronchial ulcers

Tsumiyama

Yamakawa

Sato

et al. 2018

Respiratory Medicine Case Reports

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Section: Discussionmentioning

confidence: 99%

“…Anti‐MDA5 DM is a subtype of DM with a characteristic mucocutaneous phenotype, exhibiting punched‐out cutaneous ulcers in up to 82% of patients, with a predilection for digital pulp, periungual region, MCP and interphalangeal joints, elbows, and knees . Other features include non‐scarring alopecia, lateral digit hyperkeratosis or scaling, palmar papules, oral ulcers, panniculitis, hand swelling, fever/fatigue, and arthritis/arthralgia . As muscle involvement in this phenotype is minimal, the histology of the muscle also follows the same pattern with the absence of perifascicular fiber atrophy and less intense focal perivascular inflammation and signs of capillary loss …”

Section: Discussionmentioning

confidence: 99%

“…Anti‐MDA5 antibodies, when positive, also have a potential value as a biomarker of ILD, with a high diagnostic accuracy (sensitivity > 0.80, specificity > 0.80) for screening rapidly progressive ILD in these patients . Serum ferritin levels are also commonly increased in anti‐MDA5 DM, with increasing levels correlated with greater disease activity, development of ILD, and poor survival . In addition, the finding of deep dermal necrosis on histology also poses an increased risk for ILD in DM patients .…”

Section: Discussionmentioning

confidence: 99%

“…The recognition of the histologic findings, increased ferritin levels (326.9 ng/mL), and clinical features of anti‐MDA5 subtype allowed early and aggressive surveillance in our patient. Alternatively, most studies have shown a decreased risk for malignancy in anti‐MDA5 subtype …”

Section: Discussionmentioning

confidence: 99%

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Seronegative dermatomyositis presenting with features of anti‐MDA5 subtype

et al. 2018

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Dermatomyositis is a chronic inflammatory disorder of muscles and skin, presenting with muscle weakness, characteristic rash, and classic serology findings. Histologically, cutaneous lesions show interface dermatitis with mild perivascular lymphocytic infiltrate and increased intradermal mucin. These findings are non-specific, and the diagnosis is based on clinicopathologic correlation and serology results. However, variation in clinical presentation, serology, and histology can make the diagnosis difficult and requires cognizance of uncommon findings that may serve as a clue to the diagnosis. Herein, we report such a case with unusual histologic features of vascular damage and deep dermal necrosis in a seronegative patient who presented with clinical features of anti-MDA5 variant of dermatomyositis.

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