The clinical phenotype of systemic sclerosis patients with anti-PM/Scl antibodies: results from the EUSTAR cohort

Lazzaroni, M; Marasco, Emiliano; Campochiaro, Corrado; DeVries-Bouwstra, Jeska; Gonzalez-Perez, Montserrat-Ixchel; Rojas-Serrano, Jorge; Hachulla, É.; Zanatta, Elisabetta; Barsotti, Simone; Furini, Federica; Triantafyllias, Konstantinos; Abignano, Giuseppina; Truchetet, Marie-Élise; Luca, Giacomo De; Langhe, Ellen De; Hesselstrand, Roger; Ingegnoli, Francesca; Bertoldo, Eugenia; Smith, Vanessa; Bellando-Randone, Silvia; Poormoghim, Hadi; Colombo, Enrico; Ceribelli, Angela; Furloni, A.; Zingarelli, Stefania; Cavazzana, Ilaria; Franceschini, Franco; Galdo, Francesco Del; Denton, Christopher P.; Cavagna, Lorenzo; Distler, Oliver; Allanore, Yannick; Airò, Paolo

doi:10.1093/rheumatology/keab152

Cited by 43 publications

(36 citation statements)

References 34 publications

(46 reference statements)

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“…Anti-PM/Scl (anti-PM-100 and anti-PM-75) autoantibodies are frequently reported in patients with overlap syndromes of myositis and SSc. A consistent association has been found between calcinosis and anti-PM/Scl autoantibody in SSc 3,26–29 and DM patients. 30 The association between calcinosis and scleroderma-specific autoantibodies has not been consistent.…”

Section: Epidemiologysupporting

confidence: 74%

Subcutaneous calcinosis: Is it different between systemic sclerosis and dermatomyositis?

Valenzuela

Chung

2021

Journal of Scleroderma and Related Disorders

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Calcinosis cutis is the deposition of insoluble calcium in the skin and subcutaneous tissues. It is a manifestation of several autoimmune connective tissue diseases, most frequently with systemic sclerosis and juvenile dermatomyositis, followed by adult dermatomyositis. Autoimmune connective tissue disease–associated calcinosis is of the dystrophic subtype, which occurs at sites of damaged tissue in the setting of normal serum calcium and phosphate levels. In juvenile dermatomyositis, calcinosis is considered a marker of ongoing disease activity and possibly inadequate treatment, while in adult dermatomyositis, it is a hallmark of skin damage due to chronic rather than active disease. Calcinosis is associated with long disease duration in systemic sclerosis and dermatomyositis, anti-polymyositis/sclerosis autoantibodies in systemic sclerosis and NXP-2 and melanoma differentiation-associated gene 5 in dermatomyositis. Calcinosis in systemic sclerosis occurs most frequently in the hands, particularly the fingers, whereas in dermatomyositis, it affects mainly the trunk and extremities. The primary mineral component of calcinosis is hydroxyapatite in systemic sclerosis and carbonate apatite in dermatomyositis. Calcinosis in dermatomyositis and systemic sclerosis share some pathogenic mechanisms, but vascular hypoxia seems to play a more important role in systemic sclerosis, whereas the release of calcium from mitochondria in muscle cells damaged by myopathy may be a primary mechanism contributing to dermatomyositis-related calcinosis. Multiple treatment strategies for dermatomyositis and systemic sclerosis-related calcinosis have been used with variable results. Early aggressive treatment of underlying myositis in patients with dermatomyositis may improve long-term outcomes of calcinosis. A better understanding of the pathogenesis of calcinosis is needed to improve treatment options.

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Section: Epidemiologysupporting

confidence: 74%

Subcutaneous calcinosis: Is it different between systemic sclerosis and dermatomyositis?

Valenzuela

Chung

2021

Journal of Scleroderma and Related Disorders

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“…However, it is unclear whether the malignancies in these patients are related to anti-PM/Scl syndrome because they occurred separated in time from each other, and in one patient even 6 years before the anti-PM/Scl syndrome, and one case as late as 1 year after the onset of the disease. Lazzaroni et al reported the frequency of malignancies was not significantly higher in anti-PM/Scl positive SSc patients than in anti-PM/Scl negative controls from the EULAR Scleroderma Trials and Research group (EUSTAR) database ( 19 ). More cases are needed to confirm any associations between the malignancy and the disease.…”

Section: Discussionmentioning

confidence: 99%

Interstitial Lung Disease Is a Major Characteristic of Patients Who Test Positive for Anti-PM/Scl Antibody

Shu

et al. 2022

Front. Med.

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ObjectiveThis study aimed to analyze the clinical features of anti-PM/Scl antibodies in Chinese patients.MethodWe reviewed the clinical data of anti-PM/Scl antibody-positive patients, including their long-term follow-up.ResultsA total of 30 patients carried anti-PM/Scl antibodies, 21 (70%) were females, and the mean age was 55.4 years, 15 (50%) and 10 (33.3%) patients were positive for anti-PM/Scl-75 and anti-PM/Scl-100, respectively. Fifteen cases (50%) were diagnosed as inflammatory myopathy, namely, 11 dermatomyositis (DM) and 4 polymyositis (PM). Five (16.7%) patients were diagnosed with overlap syndrome, and only one (3.3%) was diagnosed as systemic sclerosis. The other 9 (30%) patients were classified as undifferentiated connective tissue disease. Twenty-six (86.7%) had interstitial lung disease (ILD) and was the sole manifestation in 8 (26.7%) patients, 15 (58.0%) showed non-specific interstitial pneumonia based on high-resolution CT or lung biopsy. The majority of patients (95%) with mild and moderate groups on basis of pulmonary function tests. Compared to the anti-PM/Scl-100 group, the occurrence of clinical characteristics was not significantly different from the anti-PM/Scl-75 group, except the levels of C-reactive protein and erythrocyte sedimentation rate in the anti-PM/Scl-75 antibody-positive group were higher (p < 0.05). All patients with positive Ro-52 antibodies had ILD and were more likely to develop skin rash in the group with Ro-52 (p = 0.024). With a follow-up of the present cohort, 70.8% improved with treatment, but 16.7% of patients are easy to relapse.ConclusionThe anti-PM/Scl antibody occurred frequently in DM/PM patients, ILD was the major clinical feature, especially in patients combined with Ro-52. Some patients may complicate with ILD alone without extrapulmonary manifestations. Anti-PM/Scl antibodies positive patients were responsive to treatment.

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“…In the large cohort of 124 SSc/PM OS patients, 33.1% of patients presented anti-PM/Scl antibodies. 12 Myositis, calcinosis, mild interstitial pneumonia, and DM cutaneous signs are reported to be associated with anti-PM/Scl antibody positivity in SSc, 13 while 2-4% of patients with DM or SSc alone also present this antibody. 13 In addition, Mahler et al 14 reported that four of 100 SLE patients presented both anti-dsDNA and anti-PM/Scl antibodies.…”

Section: Discussionmentioning

confidence: 99%

Anti‐polymyositis/Scl antibody‐positive overlap syndrome of diffuse cutaneous systemic sclerosis, dermatomyositis, systemic lupus erythematosus, and antiphospholipid syndrome

Saito

Endo

Nishio

et al. 2021

The Journal of Dermatology

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A 37‐year‐old Japanese man with a 3‐year history of diffuse cutaneous systemic sclerosis was admitted to our hospital with high fever, arthralgia, myalgia, and muscle weakness. A physical examination revealed facial erythema, Gottron’s sign, and mechanic’s hands in addition to skin sclerosis. Laboratory data revealed significantly elevated levels of creatine kinase and decreased complement. Anti‐RNP, anti‐Smith, anti‐DNA, anti‐β2‐glycoprotein 1, anti‐polymyositis (PM)/Scl75, and anti‐PM/Scl100 antibodies were detected. He also had urinary protein, interstitial lung disease, pericarditis, multifocal cerebral infarctions, and leukoencephalopathy. Thus, a diagnosis of overlap syndrome of diffuse cutaneous systemic sclerosis, dermatomyositis, and systemic lupus erythematosus with antiphospholipid syndrome was made. Because of the intractable course, he was treated with multiple immunosuppressive and immunomodulatory drugs, including three rounds of 1000 mg methylprednisolone pulse therapy. This is the first case report of anti‐PM/Scl antibody‐positive overlap syndrome of three major connective tissue diseases.

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The clinical phenotype of systemic sclerosis patients with anti-PM/Scl antibodies: results from the EUSTAR cohort

Cited by 43 publications

References 34 publications

Subcutaneous calcinosis: Is it different between systemic sclerosis and dermatomyositis?

Subcutaneous calcinosis: Is it different between systemic sclerosis and dermatomyositis?

Interstitial Lung Disease Is a Major Characteristic of Patients Who Test Positive for Anti-PM/Scl Antibody

Anti‐polymyositis/Scl antibody‐positive overlap syndrome of diffuse cutaneous systemic sclerosis, dermatomyositis, systemic lupus erythematosus, and antiphospholipid syndrome

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