scholarly journals The Case of a Patient with Limited Systemic Sclerosis and Interstitial Lung Disease Overlapping with Systemic Lupus Erythematosus

Dermato ◽  
2021 ◽  
Vol 1 (2) ◽  
pp. 59-70
Author(s):  
Karolina Krawczyk ◽  
Ewelina Mazur ◽  
Jaromir Kargol ◽  
Robert Kijowski ◽  
Adam Reich
Keyword(s):  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Lupus Erythematosus ◽  
Diffuse Alveolar Damage ◽  
Overlap Syndrome ◽  
The World ◽  
Organ Manifestations ◽  
Limited Systemic Sclerosis ◽  
Substantial Morbidity

About 20% of patients with systemic sclerosis have symptoms of another connective tissue disease (CTD). Interstitial lung disease (ILD) is one of the most common organ manifestations in systemic sclerosis (SSc) as well as viral illnesses, such as COVID-19, and can lead not only to diffuse alveolar damage, but also trigger an exacerbation of fibrosis among patients with preexisting ILD. It is also associated with substantial morbidity and mortality. According to the World Scleroderma Foundation, SSc-ILD can mask or mimic early COVID-19 lesions and there are no available computed tomography guidelines on how to discern those two conditions. We present a case of systemic sclerosis exacerbation after COVID-19 in a patient with SSc-Lupus Overlap Syndrome.

scholarly journals Interstitial lung disease pathology in systemic sclerosis

2021 ◽  
Vol 13 ◽  
pp. 1759720X2110324
Author(s):  
Kristine E. Konopka ◽  
Jeffrey L. Myers
Keyword(s):  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Diffuse Alveolar Damage ◽  
Usual Interstitial Pneumonia ◽  
High Resolution Computed Tomography ◽  
Restrictive Lung Disease ◽  
Diffuse Parenchymal Lung Disease ◽  
Lung Biopsies

Interstitial lung disease is a relatively frequent manifestation of systemic sclerosis with approximately one-third of patients developing clinical restrictive lung disease. Fibrotic nonspecific interstitial pneumonia is the most common cause of diffuse parenchymal lung disease in patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD), followed by usual interstitial pneumonia (UIP). Radiographic pleuroparenchymal fibroelastosis-like changes may accompany other forms of interstitial lung disease, most commonly UIP. In an appropriate clinical setting with supportive high-resolution computed tomography findings, lung biopsy is not needed to confirm the presence of interstitial lung disease and surgical lung biopsies are often reserved for atypical presentations. In this review, we discuss the histological findings that define the most common patterns of SSc-ILD and outline other findings sometimes encountered in lung biopsies obtained from systemic sclerosis patients, including pulmonary vascular changes, aspiration, chronic pleuritis, and diffuse alveolar damage.

scholarly journals New Centromere Autoantigens Identified in Systemic Sclerosis Using Centromere Protein Microarrays

2013 ◽  
Vol 40 (4) ◽  
pp. 461-468 ◽  
Author(s):  
Guang Song ◽  
Chaojun Hu ◽  
Heng Zhu ◽  
Li Wang ◽  
Fengchun Zhang ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Renal Disease ◽  
Lung Disease ◽  
Western Blotting ◽  
Lupus Erythematosus ◽  
Clinical Manifestations ◽  
Protein Microarrays ◽  
Biliary Cirrhosis ◽  
Centromere Protein

Objective.To identify novel centromere protein (CENP) targets of anticentromere antibodies (ACA), and to investigate their association with clinical manifestations of systemic sclerosis (SSc).Methods.A CENP-focused protein microarray was fabricated by spotting 14 purified CENP. These microarrays were individually incubated with 35 ACA-positive SSc sera and 20 ACA-negative healthy control samples. Newly identified CENP autoantigens with high sensitivities were selected for validation and characterization.Results.Statistical analysis revealed 11 CENP are potential target antigens of ACA in patients with SSc. Of them, 5 [CENP-P, CENP-Q, CENP-M (isoform I), CENP-J, and CENP-T] are novel, among which CENP-P and CENP-Q showed high sensitivities in ACA-positive SSc sera of 34.3% and 28.6%, respectively. Subsequently, 186 SSc sera (35 ACA-positives and 151 negatives), 69 ACA-positive sera from other various autoimmune diseases (primary Sjögren syndrome, systemic lupus erythematosus, rheumatoid arthritis, and primary biliary cirrhosis), and 31 healthy sera were assayed for the presence of anti-CENP-P and -Q autoantibodies by ELISA followed by Western blotting analysis. CENP-P and -Q autoantibodies were detected in ACA-positive sera of various disease groups; among them, SSc showed the highest detection rate. Anti-CENP-P was also found in 9 of the 151 ACA-negative sera. Analyses of the correlation with clinical information showed anti-CENP-P-positive patients had higher levels of IgG, IgA, and erythrocyte sedimentation rate among the ACA-positive cohort and were more vulnerable to renal disease in the ACA-negative patients with SSc. Regardless of ACA status, anti-CENP-P or Q-negative patients seem to be predominantly affected by interstitial lung disease.Conclusion.CENP-P and CENP-Q were identified as novel ACA autoantigens by CENP microarray assays followed by validation of ELISA and Western blotting. Both of them have prognostic utility for interstitial lung disease. CENP-P was associated with renal disease in an ACA-negative cohort.

scholarly journals AB0591 ANALYSIS OF A COHORT OF PATIENTS WITH SYSTEMIC SCLEROSIS AND INTERSTITIAL LUNG DISEASE

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1592.1-1592
Author(s):  
L. Mendez Diaz ◽  
R. J. Gil Velez ◽  
I. Madroñal García
Keyword(s):  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Usual Interstitial Pneumonia ◽  
Pulmonary Involvement ◽  
Overlap Syndrome ◽  
Lung Involvement ◽  
Positive Antibody ◽  
Radiological Pattern

Background:Interstitial lung disease (ILD) is a common manifestation of systemic sclerosis that is associated with early mortality. The development or progression of ILD can occur at any time, so patients should be monitored regularly, particularly in the first years after diagnosis.Treatment should be considered when the disease is clinically significant, particularly when there is evidence of progression based on a decrease in lung function, progression of fibrosis on the HRCT or worsening of respiratory symptoms.Objectives:-To relate the type of systemic sclerosis (SS) with pulmonary involvement with the radiological pattern.-To study if there is a relationship between the antibodies and the aforementioned affectation.Methods:Retrospective descriptive study of patients treated in our Hospital (2009-2019) by the Rheumatology and Internal Medicine department diagnosed with systemic sclerosis and interstitial lung disease.The data were obtained through the review of medical records.We have included data from patients who have a diagnosis of limited or diffuse systemic sclerosis or overlap with interstitial lung involvement.Results:Of the 213 patients with systemic sclerosis in our database 43 had interstitial lung involvement (20.2%). 79% of the patients with ILD (34) had a non-specific interstitial pneumonia type (NSIP) radiological pattern and 21% of the patients (9) had a pattern of usual interstitial pneumonia (UIP)Among the patients with ILD with a NSIP -type radiological pattern, 19 patients were diagnosed with diffuse SS, 9 patients with overlap syndrome and 6 with limited SS.Of the patients with ILD with radiological pattern type UIP, 5 patients were diagnosed with diffuse SS, 3 patients with overlap syndrome and 1 patient was diagnosed with limited SS.TABLE 1.RELATIONSHIP BETWEEN SS TYPE AND RADIOLOGICAL PATTERNNSIPUIPLIMITED SS6 (17.6%)1 (11.1%)DIFFUSE SS19 (55.8%)5 (55.5%)OVERLAP9 (26.4%)3 (33.3%)Among the patients with the NSIP pattern, 17 had positive SCL70 antibody, 3 positive ANA patients and 1 patient had positive anti-centromere antibody.Of the patients with UIP type interstitial pneumopathy, 8 patients had anti-SCL70 antibody, 3 patients ANA positive antibody and 2 patients anti-centromere positive antibody.TABLE 2.RELATIONSHIP BETWEEN TYPE OF AB AND RADIOLOGICAL PATTERNNSIPUIPAnti SCL70178Anti centromere12ANA33Regarding treatment, 21 patients were taking Mycophenolate, 16 patients required cyclophosphamide and 6 patients rituximab.No patient in our cohort died due to interstitial lung disease.Conclusion:The data obtained are consistent with what is collected in the medical literature.The subtype of scleroderma most related to ILD was diffuse SS. The most frequent antibody was anti-SCL 70.Regarding the treatment,the most used in ILD in our center was the mycophenolate.From our sample analyzed when applying the likelihood ratio (RV) a value of 47,186 is obtained, which has an associated probability of 0, which is less than 0.05, leads to reject the null hypothesis (there is no dependence between antibodies and type of radiological pattern of ILD in SS), concluding that there is dependence between the analyzed variables.After this analysis, we can conclude that in our sample there is a relationship between the type of interstitial pneumopathy pattern and the antibody present in patients with SS.Disclosure of Interests:None declared

Successful experience of rituximab therapy for systemic sclerosis-associated interstitial lung disease with concomitant systemic lupus erythematosus

2014 ◽  
Vol 41 (5) ◽  
pp. 418-420 ◽  
Author(s):  
Hayakazu Sumida ◽  
Yoshihide Asano ◽  
Zenshiro Tamaki ◽  
Naohiko Aozasa ◽  
Takashi Taniguchi ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Lupus Erythematosus ◽  
Systemic Lupus

scholarly journals Role of autoantibodies in the diagnosis and prognosis of interstitial lung disease in autoimmune rheumatic disorders

2021 ◽  
Vol 13 ◽  
pp. 1759720X2110324
Author(s):  
Masataka Kuwana ◽  
Albert Gil-Vila ◽  
Albert Selva-O’Callaghan
Keyword(s):  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Topoisomerase I ◽  
Initiation Factor ◽  
Severe Condition ◽  
Diagnosis And Prognosis ◽  
Antisynthetase Antibodies ◽  
Substantial Morbidity

Interstitial lung disease (ILD) has been recognized as a frequent manifestation associated with a substantial morbidity and mortality burden in patients with autoimmune rheumatic disorders. Serum autoantibodies are considered good biomarkers for identifying several subsets or specific phenotypes of ILD involvement in these patients. This review features the role of several autoantibodies as a diagnostic and prognostic biomarker linked to the presence ILD and specific ILD phenotypes in autoimmune rheumatic disorders. The case of the diverse antisynthetase antibodies in the antisynthease syndrome or the anti-melanoma differentiation-associated 5 protein (MDA5) antibodies as a marker of a severe condition such as rapidly progressive ILD in patients with clinically amyopathic dermatomyositis are some of the associations herein reported in the group of myositis spectrum disorders. Specific autoantibodies such as the well-known anti-topoisomerase I (anti-Scl70) or the anti-Th/To, anti-U11/U12 ribonucleoprotein, and anti-eukaryotic initiation factor 2B (eIF2B) antibodies seems to be specifically linked to ILD in patients with systemic sclerosis. Overlap syndromes between systemic sclerosis and myositis, also have good ILD biomarkers, which are the anti-PM/Scl and anti-Ku autoantibodies. Lastly, other not so often reported disorders as being associated with ILD but recently most recognized as is the case of rheumatoid arthritis associated ILD or entities herein included in the miscellaneous disorders section, which include anti-neutrophil cytoplasmic antibody-associated interstitial lung disease, Sjögren’s syndrome or the mixed connective tissue disease, are also discussed.

scholarly journals Therapeutic molecular targets of SSc-ILD

2020 ◽  
Vol 5 (2_suppl) ◽  
pp. 17-30 ◽  
Author(s):  
Yun Zhang ◽  
Jörg HW Distler
Keyword(s):  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Treatment Options ◽  
High Mobility ◽  
Molecular Targets ◽  
Unknown Etiology ◽  
Target Tissue ◽  
Medical Need ◽  
Organ Manifestations

Systemic sclerosis is a fibrosing chronic connective tissue disease of unknown etiology. A major hallmark of systemic sclerosis is the uncontrolled and persistent activation of fibroblasts, which release excessive amounts of extracellular matrix, lead to organ dysfunction, and cause high mobility and motility of patients. Systemic sclerosis–associated interstitial lung disease is one of the most common fibrotic organ manifestations in systemic sclerosis and a major cause of death. Treatment options for systemic sclerosis–associated interstitial lung disease and other fibrotic manifestations, however, remain very limited. Thus, there is a huge medical need for effective therapies that target tissue fibrosis, vascular alterations, inflammation, and autoimmune disease in systemic sclerosis–associated interstitial lung disease. In this review, we discuss data suggesting therapeutic ways to target different genes in distinct tissues/organs that contribute to the development of SSc.

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