Good outcome of interstitial lung disease in patients with scleroderma associated to anti-PM/Scl antibody

Castillo, Alfredo; Simeón-Aznar, Carmen Pilar; Fonollosa-Plà, Vicent; Alonso-Vila, Serafín; Reverte-Vinaixa, M M; Muñoz, Xavier; Pallisa, Esther; Selva-O’Callaghan, Albert; Fernández‐Codina, Andreu; Vilardell‐Tarrés, Miquel

doi:10.1016/j.semarthrit.2014.07.002

Cited by 45 publications

(21 citation statements)

References 39 publications

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“…Our finding that significant ANA titers and/or detection of specific autoantibodies are found in most patients who develop ARDS raises the question if there is a comparable mechanism of lung damage between SARS-CoV-2 infection and exacerbating autoimmune disease. In 4/6 COVID-19 patients with specific ENAs who developed ARDS, detected autoantibodies were anti-PM-Scl or anti-Scl-70; if the borderline positivity for RP155 in patient #9 was included, 5/6 specific ENAs in our cohort would be associated with a form of sclerosing CTD, as these autoantibodies (as well as similar HR-CT) patterns have previously been described in dermatomyositis, (progressive) systemic sclerosis and CTD-overlap syndromes ( 26 , 27 ). Of note, a significant proportion of anti-PM-Scl-/anti-Scl-70-positive patients develop pulmonary fibrosis, raising the question of long-term effects of severe COVID-19 in these patients ( 28 ).…”

Section: Discussionmentioning

confidence: 81%

Clinical, Serological, and Histopathological Similarities Between Severe COVID-19 and Acute Exacerbation of Connective Tissue Disease-Associated Interstitial Lung Disease (CTD-ILD)

et al. 2020

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Background and Objectives Understanding the pathophysiology of respiratory failure in coronavirus disease 2019 (COVID-19) is indispensable for development of therapeutic strategies. Since we observed similarities between COVID-19 and interstitial lung disease in connective tissue disease (CTD-ILD), we investigated features of autoimmunity in SARS-CoV-2-associated respiratory failure. Methods We prospectively enrolled 22 patients with RT-PCR-confirmed SARS-CoV-2 infection and 10 patients with non-COVID-19-associated pneumonia. Full laboratory testing was performed including autoantibody (AAB; ANA/ENA) screening using indirect immunofluorescence and immunoblot. Fifteen COVID-19 patients underwent high-resolution computed tomography. Transbronchial biopsies/autopsy tissue samples for histopathology and ultrastructural analyses were obtained from 4/3 cases, respectively. Results Thirteen (59.1%) patients developed acute respiratory distress syndrome (ARDS), and five patients (22.7%) died from the disease. ANA titers ≥1:320 and/or positive ENA immunoblots were detected in 11/13 (84.6%) COVID-19 patients with ARDS, in 1/9 (11.1%) COVID-19 patients without ARDS (p = 0.002) and in 4/10 (40%) patients with non-COVID-19-associated pneumonias (p = 0.039). Detection of AABs was significantly associated with a need for intensive care treatment (83.3 vs. 10%; p = 0.002) and occurrence of severe complications (75 vs. 20%, p = 0.03). Radiological and histopathological findings were highly heterogeneous including patterns reminiscent of exacerbating CTD-ILD, while ultrastructural analyses revealed interstitial thickening, fibroblast activation, and deposition of collagen fibrils. Conclusions We are the first to report overlapping clinical, serological, and imaging features between severe COVID-19 and acute exacerbation of CTD-ILD. Our findings indicate that autoimmune mechanisms determine both clinical course and long-term sequelae after SARS-CoV-2 infection, and the presence of autoantibodies might predict adverse clinical course in COVID-19 patients.

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Section: Discussionmentioning

confidence: 81%

Clinical, Serological, and Histopathological Similarities Between Severe COVID-19 and Acute Exacerbation of Connective Tissue Disease-Associated Interstitial Lung Disease (CTD-ILD)

et al. 2020

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“…Among the MAAs, Pm/scl75 kD and Pm/scl100 kD (based on the protein recognized) are found in IIMs, overlap syndromes, and SSc, generally seronegative for Scl70 or ACA [102] and are associated with mild myositis and ILD with better outcome compared to Scl70 + SSc [103]. These autoantibodies were not found in healthy subjects [104].…”

Section: Second-line Autoimmunity Examsmentioning

confidence: 99%

Patients with Interstitial Lung Disease Secondary to Autoimmune Diseases: How to Recognize Them?

Sambataro

Pignataro³

et al. 2020

Diagnostics

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The diagnostic assessment of patients with Interstitial Lung Disease (ILD) can be challenging due to the large number of possible causes. Moreover, the diagnostic approach can be limited by the severity of the disease, which may not allow invasive exams. To overcome this issue, the referral centers for ILD organized Multidisciplinary Teams (MDTs), including physicians and experts in complementary discipline, to discuss the management of doubtful cases of ILD. MDT is currently considered the gold standard for ILD diagnosis, but it is not often simple to organize and, furthermore, rheumatologists are still not always included. In fact, even if rheumatologic conditions represent a common cause of ILD, they are sometimes difficult to recognize, considering the variegated clinical features and their association with all possible radiographic patterns of ILD. The first objective of this review is to describe the clinical, laboratory, and instrumental tests that can drive a diagnosis toward a possible rheumatic disease. The secondary objective is to propose a set of first-line tests to perform in all patients in order to recognize any possible rheumatic conditions underlying ILD.

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“…They are more likely to have myopathy and pulmonary disease [24]. Patients with scleroderma and anti-PmScl antibodies had fewer digital ulcers and cutaneous thickening or diffuse form while they had better pulmonary prognosis [25]. In the EULAR register [6], muscle weakness and creatinine phosphokinase increase were significantly associated to diffuse cutaneous form (37.1% vs. 11.3%) and anti-Scl70 antibodies (32.2% vs. 8.7%).…”

Section: Muscular Involvementmentioning

confidence: 99%

Systemic sclerosis: An update in 2016

Desbois

Cacoub

2016

Autoimmunity Reviews

133

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Systemic sclerosis (SSc) is a chronic immune disorder of unknown origin, dominated by excessive fibrosis responsible for cutaneous and pulmonary fibrosis, and by vascular endothelial dysfunction at the origin of skin ischemia, renal and pulmonary artery lesions. Renal and pulmonary complications are mainly responsible for the severity of the disease. Recent advances led to a better understanding of pathological mechanisms and a more accurate classification of patients according to clinical and biological (auto-antibodies) phenotype. Recent trials provided interesting data on different therapeutic strategies, depending on organ involvement. These data are of particular importance in such disease, still characterized by increased mortality and morbidity rates. In this review, we aim to synthetize recent advances in diagnosis and prognosis leading to better classification of SSc patients, and in therapeutic management.

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Good outcome of interstitial lung disease in patients with scleroderma associated to anti-PM/Scl antibody

Cited by 45 publications

References 39 publications

Clinical, Serological, and Histopathological Similarities Between Severe COVID-19 and Acute Exacerbation of Connective Tissue Disease-Associated Interstitial Lung Disease (CTD-ILD)

Clinical, Serological, and Histopathological Similarities Between Severe COVID-19 and Acute Exacerbation of Connective Tissue Disease-Associated Interstitial Lung Disease (CTD-ILD)

Patients with Interstitial Lung Disease Secondary to Autoimmune Diseases: How to Recognize Them?

Systemic sclerosis: An update in 2016

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