Clinical and Serologic Correlates of Anti‐PM/Scl Antibodies in Systemic Sclerosis: A Multicenter Study of 763 Patients

D’Aoust, Julie; Hudson, Marie; Tatibouet, Solène; Wick, James; Mähler, Michael; Baron, Murray; Fritzler, Marvin J.

doi:10.1002/art.38428

Cited by 103 publications

(75 citation statements)

References 19 publications

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“…Anti-PM/Scl autoantibodies have been found in 17% of patients with IIM-limited SSc overlap syndrome, compared with 7% of patients with isolated SSc [31], 9% of dermatomyositis patients and 6% of polymyositis patients [8]. Thus, anti-PM/Scl autoantibodies are usually classified as MAA (table 2).…”

Section: Classification Based On Autoantibodiesmentioning

confidence: 99%

Idiopathic inflammatory myopathies and the lung

et al. 2015

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Idiopathic inflammatory myositis (IIM) is a group of rare connective tissue diseases (CTDs) characterised by muscular and extramuscular signs, in which lung involvement is a challenging issue. Interstitial lung disease (ILD) is the hallmark of pulmonary involvement in IIM, and causes morbidity and mortality, resulting in an estimated excess mortality of 50% in some series. Except for inclusion body myositis, these extrapulmonary disorders are associated with the general and visceral involvement frequently found in other CTDs including fever, Raynaud's phenomenon, arthralgia, nonspecific cutaneous modifications and ILD, for which the prevalence is estimated to be up to 65%. Substantial heterogeneity exists within the spectrum of IIMs, and each condition is associated with various frequencies and subtypes of pulmonary involvement. This heterogeneity is partly related to the presence of various autoantibodies encompassing anti-synthetase, anti-MDA5 and anti-PM/Scl. ILD is present in all subsets of IIM including juvenile myositis, but is more frequent in dermatomyositis and overlap myositis. IIM can also be associated with other presentations of respiratory involvement, namely pulmonary arterial hypertension, pleural disease, infections, drug-induced toxicity, malignancy and respiratory muscle weakness. Here, we critically review the current knowledge about adult and juvenile myositis-associated lung disease with a detailed description of therapeutics for chronic and rapidly progressive ILD. @ERSpublications Interstitial lung disease is a leading cause of morbidity in dermatomyositis/polymyositis

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Section: Classification Based On Autoantibodiesmentioning

confidence: 99%

Idiopathic inflammatory myopathies and the lung

et al. 2015

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“…Antibodies directed against the PM-Scl complex in the human exosome identify a distinct subset of patients with SSc and/or myositis overlap [21,[46][47][48]. Studies highlight how anti-PMScl patients tend to be younger at disease onset and have milder cutaneous SSc features with limited skin disease and in some inflammatory arthropathy [46][47][48].…”

Section: Pm-scl Overlap Syndromementioning

confidence: 99%

“…Studies highlight how anti-PMScl patients tend to be younger at disease onset and have milder cutaneous SSc features with limited skin disease and in some inflammatory arthropathy [46][47][48]. Although Raynaud's is a common feature, peripheral vasculopathy with digital ulceration appears less common.…”

Section: Pm-scl Overlap Syndromementioning

confidence: 99%

“…Characterisation of AnuAb can guide the clinician and help define specific clinical manifestations and in some cases prognosis, for example anti-RNAPol3 is a strong predictor of SSc renal crisis. In the context of CTD-myositis overlap, autoAb against PM-Scl protein complex and U3RNP is the most relevant, with a higher frequency of parenchymal or vascular lung involvement [21,[46][47][48].…”

Section: Systemic Sclerosis-myositis Overlap and Anti-nucleolar/anti-mentioning

confidence: 99%

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The Clinical Features of Myositis-Associated Autoantibodies: a Review

Gunawardena

2015

Clinic Rev Allerg Immunol

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The idiopathic inflammatory myopathies (IIM) are a group of autoimmune diseases traditionally defined by clinical manifestations including skeletal muscle weakness, skin rashes, elevated skeletal muscle enzymes, and neurophysiological and/or histological evidence of muscle inflammation. Patients with myositis overlap can develop other features including parenchymal lung disease, inflammatory arthritis, gastrointestinal manifestations and marked constitutional symptoms. Although patients may be diagnosed as having polymyositis (PM) or dermatomyositis (DM) under the IIM spectrum, it is quite clear that disease course between subgroups of patients is different. For example, interstitial lung disease may predominate in some, whereas cutaneous complications, cancer risk, or severe refractory myopathy may be a significant feature in others. Therefore, tools that facilitate diagnosis and indicate which patients require more detailed investigation for disease complications are invaluable in clinical practice. The expanding field of autoantibodies (autoAbs) associated with connective tissue disease (CTD)-myositis overlap has generated considerable interest over the last few years. Using an immunological diagnostic approach, this group of heterogeneous conditions can be separated into a number of distinct clinical phenotypes. Rather than diagnose a patient as simply having PM, DM or overlap CTD, we can define syndromes to differentiate disease subsets that emphasise clinical outcomes and guide management. There are now over 15 CTD-myositis overlap autoAbs found in patients with a range of clinical manifestations including interstitial pneumonia, cutaneous disease, cancer-associated myositis and autoimmune-mediated necrotising myopathy. This review describes their diagnostic utility, potential role in disease monitoring and response to treatment. In the future, routine use of these autoAb will allow a stratified approach to managing this complex set of conditions.

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“…Indeed, these cases illustrate the extended period of time often needed to diagnose these patients and the key role of anti-PM/Scl antibodies in confirming the diagnosis. This antibody correlates strongly with the syndrome 8,9 and results in a nucleolar antinuclear antibody pattern 8,10 . Although it can be observed in isolated DM/JDM or SSc, 80% of patients with anti-PM/Scl antibodies will have overlap SSc/myositis syndrome 4 .…”

Section: Four Cases Of Anti-pm/scl Antibody-positive Juvenile Overlapmentioning

confidence: 87%