A Case of Anti-SAE1 Dermatomyositis

Vries, Max de; Schreurs, Marco W. J.; Ahsmann, Els J.M.; Spee-Dropková, Marcela; Karim, Faiz

doi:10.1155/2022/9000608

Cited by 3 publications

(3 citation statements)

References 10 publications

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“…Pulmonary involvement is variable in DM according to different MSA and is mainly associated with the anti-MDA5 subset, where it often presents as nonspecific interstitial pneumonia and car-ries a high risk of developing into rapidly progressive ILD (21). It is important to emphasise that the primary type of ILD reported in anti-SAE DM is organising pneumonia (22), typically characterised by a chronic course and a favourable prognosis (23,24). However, we observed a case of rapidly progressive ILD that led to death.…”

Section: Discussionmentioning

confidence: 99%

Anti-SAE dermatomyositis: clinical and histologic characteristics from a monocentric Italian cohort

Fornaro,

Coladonato,

Giannini

et al. 2024

Clinical and Experimental Rheumatology

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ObjectiveMultiple myositis-specific antibodies have been identified, each associated with different clinical subsets of dermatomyositis (DM). Anti-SAE associated DM is considered the least studied subset. Our study aimed to evaluate the clinical and histological characteristics of DM patients with anti-SAE antibodies. As reference, patients with anti-Mi2 antibodies associated DM, representing a well-characterised subset, were analysed. Methods We recorded data from our DM cohort in the INflammatory MYositis REgistry (INMYRE).Patients were divided into two groups: those positive for anti-SAE and those positive for anti-Mi2 antibodies. Clinical characteristics, including skin, muscle, and extra-muscular involvements, were recorded. Available muscle biopsies were compared between the two groups. ResultsOf 92 DM patients, 10 (10.9%) were positive for anti-SAE and 17 (18.5%) for anti-Mi2. Anti-SAE positive DM patients showed classic DM findings but were characterised by a higher prevalence of skin itching (60% vs. 11.8%, p<0.01), shawl sign (40% vs. 5.9%, p<0.05) and lung involvement (30% vs. 0%, p<0.05) compared to anti-Mi2 positive patients. Furthermore, anti-SAE positive DM patients showed lower creatine kinase levels than those with anti-Mi2 (median [IQR]: 101 [58-647] vs. 1984 [974-3717], p<0.05) and a lower percentage of muscle fibre degeneration and necrosis (1.5%±1.7 vs. 5.9%±3.2, p<0.05) in muscle biopsies. No other differences were observed. ConclusionAnti-SAE DM represents a disease subset characterised by classic cutaneous involvement often associated with itching, less severe muscle involvement, but potential pulmonary involvement that should always be investigated in these patients.

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Section: Discussionmentioning

confidence: 99%

Anti-SAE dermatomyositis: clinical and histologic characteristics from a monocentric Italian cohort

Fornaro,

Coladonato,

Giannini

et al. 2024

Clinical and Experimental Rheumatology

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“…5 ILD associated with anti-SAE antibodies is characterized by an insidious clinical course with few respiratory complaints, a preserved pulmonary function, and a limited extent of pulmonary lesions with subpleural peripheral-dominant small ground glass opacities/consolidations on chest CT. 6 Most studies on anti-SAE1 antibodies and cancer show a positive association. A study by De Vries et al 1 indicated that most patients suffered from adenocarcinoma of cervical, ovarian, pulmonary, esophageal, or rectal origin. Therefore, all patients diagnosed with anti-SAE DM should undergo an age and sex-appropriate malignancy workup.…”

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Case report: A case of resistant anti‐SAE1 dermatomyositis with severe periorbital edema after hydroxychloroquine that responded to adalimumab

Farhat,

Soutou

2024

Int J of Rheum Dis

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The frequency of anti-SAE1 (small ubiquitin-like modifier activating enzyme) in dermatomyositis (DM) patients is very low, ranging from 1.5% to 8.0%. 1 The typical phenotype is usually that of widespread skin involvement with amyopathic or mild muscle involvement. The association with interstitial lung disease (ILD), dysphagia, other extramuscular involvement, and cancer varies per cohort. 2 Standard first-line treatments are corticosteroids with disease-modifying agents such as methotrexate or azathioprine. In severe cases, intravenous immunoglobulin (IVIG), cyclophosphamide, and/or rituximab can be used. Anti-TNF-a (Tumor necrosis factor alpha) antibodies have been used successfully to treat juvenile DM but are not routinely used in adult DM patients. 3 As few patients with anti-SAE1 dermatomyositis have been described, it is important to describe more cases of this rare subtype.We report the case of a 57-year-old Caucasian woman with a medical history of hypertension, diabetes, hyperlipidemia, and hyperthyroidism, respectively treated with amlodipine, propranolol, metformin, rosuvastatin, thiamazole, along with omeprazole, who presented to our clinic for a 6-month history of pruritic diffuse erythema and scaling over the scalp, face, trunk, and limbs. A short empirical treatment with medium-dose oral prednisone led to partial and temporary improvement a month earlier. On physical examination, she had a heliotrope rash, periorbital violaceous edema (Figure 1A), a "V-neck" sign, Gottron's papules on the fingers (Figure 1B), and diffuse erythema of the back with an "angel wings" sign (Figure 2A). She was complaining of fatigue, weight loss, myalgias without muscular weakness, and swallowing difficulties.No arthralgias nor pulmonary or digestive symptoms were noted.

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Patients with anti-small ubiquitin-like modifier activating enzyme-positive dermatomyositis resembling antisynthetase syndrome with poor prognosis: a bicentric international retrospective study and literature review

De Carvalho,

Bayeh,

De Souza

et al. 2024

Reumatismo

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Objective. This study aimed to describe adult Brazilian and Japanese patients with anti-small ubiquitin-like modifier activating enzyme (SEA)-positive dermatomyositis (DM), as there are few studies in the literature. A literature review was also conducted. Methods. This bicentric international retrospective study, conducted between 2012 and 2023, included patients with anti-SAE-positive DM (2017 European League Against Rheumatism/American College of Rheumatology classification criteria). All demographic features and clinical, laboratory, therapeutic, and follow-up data were collected from Brazilian and Japanese centers using pre-standardized and parameterized information. Results. We included 17 adult patients with a median age of 65 (56-76) and a predominance of females (82.4%). Constitutional symptoms at baseline were present in 58.8% of the patients. In addition to classical cutaneous DM lesions, one-third of the patients had myalgia and significant muscle weakness, whereas half presented with dysphagia, interstitial lung disease, and joint manifestations. The first-line treatment consisted of intravenous methylprednisolone and immunoglobulin pulse therapy in 41.2% and 28.6% of the patients, respectively. The median follow-up duration was 20 (13-74) months; at the last medical evaluation, half had active disease and were still using oral glucocorticoids (median dosage, 10.0 mg/day). Approximately one-fifth to one-third of the patients were diagnosed with different types of cancer, had severe infections, or died. Conclusions. Patients with anti-SAE-positive DM not only resemble the phenotype of antisynthetase syndrome but are also associated with a poor prognosis.

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A Case of Anti-SAE1 Dermatomyositis

Cited by 3 publications

References 10 publications

Anti-SAE dermatomyositis: clinical and histologic characteristics from a monocentric Italian cohort

Anti-SAE dermatomyositis: clinical and histologic characteristics from a monocentric Italian cohort

Case report: A case of resistant anti‐SAE1 dermatomyositis with severe periorbital edema after hydroxychloroquine that responded to adalimumab

Patients with anti-small ubiquitin-like modifier activating enzyme-positive dermatomyositis resembling antisynthetase syndrome with poor prognosis: a bicentric international retrospective study and literature review

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