Interstitial Lung Disease In Systemic Sclerosis Patients Research Articles

Association of serum interferon alpha-2a levels with disease severity and prognosis in systemic sclerosis.

To determine serum type I interferon IFN-α2a concentrations in systemic sclerosis (SSc) patients, explore its association with cytokine/chemokine expressions, and evaluate correlation with the phenotype including the predictive value for interstitial lung disease (ILD) progression. Serum samples were obtained from 200 SSc patients and 29 healthy controls. IFN-α2a levels were measured by ultrasensitive electrochemiluminescence assay. Pro-inflammatory and chemokine panels were determined by Luminex® Discovery Assay multiplex kit. Baseline SSc disease characteristics were recorded together with longitudinal data for determining ILD progression after 2 years. IFN-α2a concentrations were higher in SSc patients compared with controls, although not reaching significance (means ± SD of 49.20 ± 156.8 pg/ml vs 9.606 ± 4.399 pg/ml, respectively; (p = 0.158)). Using the cut-off of 15.9 pg/ml, we identified 62 patients as having a type 1 (T1) IFN signature in their circulation. Patients with an IFN signature had significantly higher levels of chemokines (CCL8, CCL19, CXCL10, CXCL11), and the cytokine IL-1α compared with those without an IFN signature. IFN-α2a concentrations strongly correlated with a T1 IFN related chemokine score supporting activation of this pathway. Phenotyping association queries revealed association between IFN values and both skin and ILD involvements at baseline. Longitudinal data did not identify IFN as a predictive marker for ILD progression. Using serum determinations, the activation of the T1 IFN pathway showed strong correlations with inflammatory mediators and associations with clinical manifestations, especially skin fibrosis and ILD in SSc patients. However, activated IFN pathway was not predictive of ILD progression.

Real-World Clinical Profile and Safety of Nintedanib in Systemic Sclerosis-Associated Interstitial Lung Disease: A Subgroup Analysis of Interstitial Lung Disease Data From an Interstitial Lung Disease (ILD) Specialty Clinic in India.

Systemic sclerosis (SSc) is a multisystem autoimmune disorder characterized by dysregulated innate and adaptive immunity. Interstitial lung disease (ILD) is a common and serious complication of SSc, often leading to significant morbidity and mortality. Consistent demographic characteristics that aid in the early diagnosis of ILD in SSc are lacking. This study aims to identify clinical and demographic parameters associated with ILD in SSc patients and assess the safety and tolerability of nintedanibwith other immunosuppressants. This study is a subgroup analysis of data from the ILD clinic at All India Institute of Medical Sciences Raipur, collected between January 2022 and January 2024. We assessed the clinical and demographic profiles, high-resolution computed tomography thorax patterns, autoantibody profiles, lung function, and treatments used in the patients. We enrolled 57 patients with SSc-associated ILD. The mean age of the participants was 39.0 ± 11.1 years, with 53 (92.9%) being women. The mean body mass index was 20.4 ± 4.32 kg/m². Dyspnea was the most common symptom, followed by skin tightening and cough. Antinuclear antibody tests were positive in 92.9% of patients, and anti-Scl-70 antibodies were positive in 57.9%. Rheumatoid arthritis-SSc overlap was observed in 15.8% of patients. The mean predicted forced vital capacity was 46.5 ± 19.9%, the mean predicted total lung capacity was 64.5 ± 20.4%, and the mean predicted diffusing capacity for carbon monoxide was 46.2 ± 15.7%. The mean six-minute walk distance was 360.3 ± 81.2 meters, and the mean King's Brief Interstitial Lung Disease score was 63.9 ± 10.7. Common radiological abnormalities included ground-glass opacities in 57.8%, traction bronchiectasis in 43.8%, and honeycombing in 28.07%. The predominant ILD pattern was nonspecific interstitial pneumonia. Patients received a combination of prednisolone (5 mg/day) with mycophenolate mofetil (63.2%), hydroxychloroquine (17.5%), cyclophosphamide (12.3%), and methotrexate (7.02%). Nintedanib, the only antifibrotic used, was administered to 17 (29.8%) patients. ILD is relatively common in SSc, particularly in patients with diffuse cutaneous SSc and those with anti-topoisomerase antibodies. Female patients comprised the predominant population in this study. Patients tolerated mycophenolate mofetil and cyclophosphamide well. Nintedanib was the only antifibrotic used, and all patients tolerated the combination of antifibrotics and immunosuppressants well. Early diagnosis is crucial to slow disease progression and preserve lung function. Our results highlight the need for vigilant screening in high-risk groups and suggest that MMF, cyclophosphamide, and nintedanib can be safely incorporated into treatment regimens, offering a potential strategy to improve patient outcomes.

Risk Factors and Biomarkers for Interstitial Lung Disease and Pulmonary Arterial Hypertension in Systemic Sclerosis: Experience of Two Tertiary Centers in Turkey

Purpose: To define the clinical and laboratory characteristics of patients with systemic sclerosis (SSc), and to investigate the risk factors affecting the prevalence of interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH), which are important causes of morbidity and mortality. Patients and methods: 88 patients with SSc were compared according to the presence of ILD and PAH. ILD was confirmed by chest computed tomography, and PAH was suspected and considered probable PAH when right ventricular systolic pressure was >40 mmHg according to echocardiography. Results: Of the 88 patients, 44.3% had diffuse-type and 55.7% had limited-type SSc. Diffuse type, percentages of positive anti-scleroderma-70 (anti-Scl70) antibody and anti-centromere antibody, white blood cell (WBC), platelet, erythrocyte sedimentation rate (ESR), smoking, and presence of the sclerodactyly and telangiectasia differed significantly in SSc with ILD group. The positive titer of anti-Scl70 antibody (odds ratio (OR)=6.124, p=0.004), platelet count (OR=0.138, p=0.002), ESR (OR=1.042, p=0.035) and presence of telangiectasia (OR=10.571, p=0.001) were associated with ILD in patients with SSc. Also, while diffuse-type (OR=0.223, p=0.010), the presence of sclerodactyly (OR=11.112, p=0.028) and telangiectasia (OR=3.861, p=0.020) were risk factors for the development of ILD in nonspecific interstitial pneumonia pattern, anti-Scl-70 antibody positivity (OR=12.921, p=0.019) and high ESR (OR=1.034, p=0.030) were found to be risk factors for the development of usual interstitial pneumonia pattern. Conclusion: Positive titer of the anti-Scl70 antibody, diffuse type, presence of telangiectasia, and high ESR were independently associated with ILD in SSc patients.

The Value of Ultrasound for Detecting and Following Subclinical Interstitial Lung Disease in Systemic Sclerosis.

Interstitial lung disease (ILD) is a complication in patients with systemic sclerosis (SSc). Accurate strategies to identify its presence in early phases are essential. We conducted the study aiming to determine the validity of ultrasound (US) in detecting subclinical ILD in SSc, and to ascertain its potential in determining the disease progression. 133 patients without respiratory symptoms and 133 healthy controls were included. Borg scale, Rodnan skin score (RSS), auscultation, chest radiographs, and respiratory function tests (RFT) were performed. A rheumatologist performed the lung US. High-resolution CT (HRCT) was also performed. The patients were followed every 12 weeks for 48 weeks. A total of 79 of 133 patients (59.4%) showed US signs of ILD in contrast to healthy controls (4.8%) (p = 0.0001). Anti-centromere antibodies (p = 0.005) and RSS (p = 0.004) showed an association with ILD. A positive correlation was demonstrated between the US and HRCT findings (p = 0.001). The sensitivity and specificity of US in detecting ILD were 91.2% and 88.6%, respectively. In the follow-up, a total of 30 patients out of 79 (37.9%) who demonstrated US signs of ILD at baseline, showed changes in the ILD score by US. US showed a high prevalence of subclinical ILD in SSc patients. It proved to be a valid, reliable, and feasible tool to detect ILD in SSc and to monitor disease progression.

Interstitial Lung Disease in Systemic Sclerosis: A Single-center Retrospective Analysis.

Systemic sclerosis (SSc) is a systemic autoimmune disease characterized by microangiopathy, inflammation, fibrosis. Interstitial lung disease (ILD) is common among SSc patients. This study aims to define the clinical, laboratory, and serologic characteristics of SSc patients with ILD and to present the frequency of chest computed tomography features. Two hundred twenty-six SSc patients who applied to the Rheumatology Department between January 2007 and August 2019 were retrospectively examined. A total of 100 SSc patients with ILD (44.2%) were determined. Clinical, laboratory, and serological features of SSc patients with and without ILD were compared. Both groups had similar characteristics in terms of age and sex. The duration of disease (p=0.001) and follow-up time (p=0.001) were longer in SSc patients with ILD. Multivariable logistic regression analysis indicated that the duration of disease (OR: 1.06 (1.01-1.13), p=0.029), presence of gastrointestinal system involvement (OR: 3.29 (1.28-8.46), p=0.013) and anti-SCL70-positivity (OR: 6.04 (2.35-15.49), p <0.001) were associated with ILD. There was an inverse relationship between Anti-CENP-B positivity and the presence of ILD (p=0.001). The assessment regarding the chest computed tomography characteristics of interstitial pneumonia patterns were as follows: 82.5% non-specific interstitial pneumonia, 14.4% usual interstitial pneumonia, and 2.1% desquamative interstitial pneumonia. The most frequent abnormal findings included ground-glass opacification (88.7%), reticulation (64.9%), traction bronchiectasis (57.7%), septal thickening (52.6%) and honeycombing (28.9%). We have shown a relationship between anti-SCL70, disease duration, gastrointestinal system involvement, and ILD in SSc patients.

Laboratory strategy for autoantibodies testing as a diagnostic marker of pulmonary fibrosis in systemic sclerosis: A preliminary study prior to cohort registry of systemic sclerosis in West Java Indonesia

Background: Interstitial lung disease (ILD) is a complication often found in patients with Systemic Sclerosis (SSc). This usually manifests as pulmonary fibrosis and is the leading cause of death in SSc patients. Antinuclear antibodies indirect immunofluorescence (ANA-IIF) test and specific autoantibodies testing could be employed as a diagnostic marker to detect pulmonary fibrosis, but the high cost of these tests often burdens patients in developing countries. This study aims to compare ANA-IIF and an alternative, possibly inexpensive method to detect pulmonary fibrosis in SSc. Methods: This is a preliminary study prior to the cohort systemic sclerosis registry conducted in Rheumatology Outpatient Clinic and Clinical Pathology Department at Dr. Hasan Sadikin General Hospital, Bandung, from January 1, 2018 to December 31, 2020. Two contrastive laboratory testing strategies were followed to confirm pulmonary fibrosis in 61 SSc patients. The first strategy was to perform antinuclear antibodies indirect immunofluorescence (ANA-IIF) test followed by specific autoantibodies detection [anti-topoisomerase-I (ATA), Anti-Th/To, SSc Profile, Immunoblot]. The alternative strategy included conducting the tests for SSc autoantibodies in the reverse order. Results: A total of 42 out of 61 (68.8%) subjects were diagnosed with pulmonary fibrosis by high-resolution computed tomography (HRCT) scan. The first strategy detected dominant specific pulmonary fibrosis autoantibodies in subjects with pulmonary fibrosis: homogeneous nucleolar pattern was dominant (n = 25, 59.52%), followed by nucleolar pattern (n = 17, 40.48%). Of all the subjects with a homogeneous nucleolar pattern, ATA was detected in 15 subjects (60%), the combination of ATA and Th/To in 9 subjects (36%), and Ro52 in one subject (4%). The second strategy yielded positive autoantibodies results in 29 out of 42 (69.05%) subjects, ATA in 20 subjects (47.61%), and Th/To in 9 patients (21.44%), but failed to detect any autoantibodies specific to SSc-ILD in 13 subjects (30.95%). Conclusion: The first strategy [ANA-IIF followed specific autoantibodies testing (ATA, Th/To, SSc profile and immunoblot)] is more likely to detect specific autoantibodies toward ILD in SSc patients.

Endothelial Progenitor Cells: Relevant Players in the Vasculopathy and Lung Fibrosis Associated with the Presence of Interstitial Lung Disease in Systemic Sclerosis Patients.

Endothelial progenitor cells (EPC), which are key effectors in the physiologic vascular network, have been described as relevant players in autoimmune diseases. We previously showed that EPC frequency may help to identify the presence of interstitial lung disease (ILD) in rheumatoid arthritis patients. Given that ILD constitutes the main cause of mortality in systemic sclerosis (SSc) patients, we aimed to determine the EPC contribution to the pathogenic processes of vasculopathy and lung fibrosis in SSc-ILD+. EPC quantification was performed by flow cytometry on blood from 83 individuals: 21 SSc-ILD+ patients and subjects from comparative groups (20 SSc-ILD− and 21 idiopathic pulmonary fibrosis (IPF) patients and 21 healthy controls (HC)). EPC were considered as CD34+, CD45low, CD309+, and CD133+. A significant increase in EPC frequency was found in SSc-ILD+ patients when compared to HC (p < 0.001). SSc-ILD+ patients exhibited a higher EPC frequency than SSc-ILD− patients (p = 0.012), whereas it was markedly reduced compared to IPF patients (p < 0.001). EPC frequency was higher in males (p = 0.04) and negatively correlated to SSc duration (p = 0.04) in SSc-ILD+ patients. Our results indicate a role of EPC in the processes of vasculopathy and lung fibrosis in SSc-ILD+. EPC frequency may be considered as a biomarker of ILD in SSc patients.

Subclinical Interstitial Lung Disease in Patients with Systemic Sclerosis. A Pilot Study on the Role of Ultrasound.

Interstitial lung disease (ILD) is a common comorbidity present in patients with systemic sclerosis (SSc). Employment of high-resolution computed tomography (HRCT) is very limited and lung ultrasound (LUS) can be an alternative tool for the early evaluation of ILD. To determine the validity of LUS in the early detection of ILD in patients with SSc. Sixty-eight patients with SSc ≥18 years without respiratory symptoms were included. A rheumatologist rated the subclinical respiratory condition, another rheumatologist blinded to the clinical assessment performed the LUS. To determine validity HRCT was performed as well. Prevalence of ILD in SSc patients was 41.2% in contrast to the 4.8% healthy controls (P=.0001). Variables associated with LUS and HRCT findings were anti-centromere antibodies (P=.005) and the Rodnan skin score (P=.004). A positive correlation was present between the findings of HRCT and LUS (P=.001). Sensitivity and specificity were 91.2% and 88.6% respectively. Good reliability in the LUS findings was found between observers (k=.72). By proving to be a valid, trustworthy and feasible alternative tool, we consider that LUS can be implemented for the early detection of ILD in SSc.

Validating Alinity immuno- and clinical chemistry assays for routine use

The autoantibody profile, which varies between races, has rarely been reported in Chinese Han systemic sclerosis (SSc) patients. The present study aims to investigate the autoantibody profile of Chinese Han SSc patients using a commercial line immunoassay.The prevalence of autoantibodies to Ro-52, PDGFR, Ku, PM75, PM100, Th/To, NOR90, Fib, RP155, RP11, CENP-B, CENP-A and Scl-70 were analyzed in serum samples obtained from healthy controls (n = 30), SSc patients (n = 320) and non-SSc connective-tissue disease patients (n = 100) using an Euroline Systemic Sclerosis Profile kit.SSc patients had increased prevalence of anti-RP11 (P = 0.032), anti-CENPB (P < 0.001), anti-CENPA (P = 0.001) and anti-Scl-70 (P < 0.001), but had decreased prevalence of anti-Ro-52 (P = 0.004), when compared to non-SSc CTDs. Furthermore, SSc patients had increased prevalence of anti-Ro-52 (P = 0.003), anti-CENPB (P = 0.034), anti-CENPA (P = 0.034) and anti-Scl-70 (P < 0.001), when compared to HCs. In addition, SSc patients with interstitial lung disease (ILD) had increased prevalence of anti-Ro-52 (P = 0.046) and anti-Scl-70 (P = 0.035), but had decreased prevalence of anti-CENPB (P < 0.001) and anti-CENPA (P < 0.001). Moreover, SSc patients with pulmonary arterial hypertension (PAH) had increased prevalence of anti-Ro-52 (P = 0.013) and decreased prevalence of anti-Scl-70 (P = 0.001). Anti-Ro-52 and anti-Scl-70 had increased values for predicting ILD in SSc patients, while anti-Ro-52 had increased values for predicting PAH in SSc patients.A commercial line immunoassay was used to evaluate the diagnostic value of autoantibodies in Chinese Han SSc patients. The results clearly indicate that the immunoblot assay has good diagnostic utility for SSc. Anti-Ro-52, anti-RP11, anti-CENPB, anti-CENPA and anti-Scl-70 are useful for diagnosing SSc in the Chinese Han population. Anti-Ro-52, anti-CENPB, anti-CENPA and anti-Scl-70 have a potential value for diagnosing ILD in SSc patients. Furthermore, anti-Ro-52 and anti-Scl-70 may be used to diagnose PAH in SSc patients.

Harmonization protocols for Lp-PLA2 activity reagents: An initial attempt in China

The autoantibody profile, which varies between races, has rarely been reported in Chinese Han systemic sclerosis (SSc) patients. The present study aims to investigate the autoantibody profile of Chinese Han SSc patients using a commercial line immunoassay.The prevalence of autoantibodies to Ro-52, PDGFR, Ku, PM75, PM100, Th/To, NOR90, Fib, RP155, RP11, CENP-B, CENP-A and Scl-70 were analyzed in serum samples obtained from healthy controls (n = 30), SSc patients (n = 320) and non-SSc connective-tissue disease patients (n = 100) using an Euroline Systemic Sclerosis Profile kit.SSc patients had increased prevalence of anti-RP11 (P = 0.032), anti-CENPB (P < 0.001), anti-CENPA (P = 0.001) and anti-Scl-70 (P < 0.001), but had decreased prevalence of anti-Ro-52 (P = 0.004), when compared to non-SSc CTDs. Furthermore, SSc patients had increased prevalence of anti-Ro-52 (P = 0.003), anti-CENPB (P = 0.034), anti-CENPA (P = 0.034) and anti-Scl-70 (P < 0.001), when compared to HCs. In addition, SSc patients with interstitial lung disease (ILD) had increased prevalence of anti-Ro-52 (P = 0.046) and anti-Scl-70 (P = 0.035), but had decreased prevalence of anti-CENPB (P < 0.001) and anti-CENPA (P < 0.001). Moreover, SSc patients with pulmonary arterial hypertension (PAH) had increased prevalence of anti-Ro-52 (P = 0.013) and decreased prevalence of anti-Scl-70 (P = 0.001). Anti-Ro-52 and anti-Scl-70 had increased values for predicting ILD in SSc patients, while anti-Ro-52 had increased values for predicting PAH in SSc patients.A commercial line immunoassay was used to evaluate the diagnostic value of autoantibodies in Chinese Han SSc patients. The results clearly indicate that the immunoblot assay has good diagnostic utility for SSc. Anti-Ro-52, anti-RP11, anti-CENPB, anti-CENPA and anti-Scl-70 are useful for diagnosing SSc in the Chinese Han population. Anti-Ro-52, anti-CENPB, anti-CENPA and anti-Scl-70 have a potential value for diagnosing ILD in SSc patients. Furthermore, anti-Ro-52 and anti-Scl-70 may be used to diagnose PAH in SSc patients.

Evaluation of a commercial immunoassay for autoantibodies in Chinese Han systemic sclerosis population

ObjectiveThe autoantibody profile, which varies between races, has rarely been reported in Chinese Han systemic sclerosis (SSc) patients. The present study aims to investigate the autoantibody profile of Chinese Han SSc patients using a commercial line immunoassay. MethodsThe prevalence of autoantibodies to Ro-52, PDGFR, Ku, PM75, PM100, Th/To, NOR90, Fib, RP155, RP11, CENP-B, CENP-A and Scl-70 were analyzed in serum samples obtained from healthy controls (n = 30), SSc patients (n = 320) and non-SSc connective-tissue disease patients (n = 100) using an Euroline Systemic Sclerosis Profile kit. ResultsSSc patients had increased prevalence of anti-RP11 (P = 0.032), anti-CENPB (P < 0.001), anti-CENPA (P = 0.001) and anti-Scl-70 (P < 0.001), but had decreased prevalence of anti-Ro-52 (P = 0.004), when compared to non-SSc CTDs. Furthermore, SSc patients had increased prevalence of anti-Ro-52 (P = 0.003), anti-CENPB (P = 0.034), anti-CENPA (P = 0.034) and anti-Scl-70 (P < 0.001), when compared to HCs. In addition, SSc patients with interstitial lung disease (ILD) had increased prevalence of anti-Ro-52 (P = 0.046) and anti-Scl-70 (P = 0.035), but had decreased prevalence of anti-CENPB (P < 0.001) and anti-CENPA (P < 0.001). Moreover, SSc patients with pulmonary arterial hypertension (PAH) had increased prevalence of anti-Ro-52 (P = 0.013) and decreased prevalence of anti-Scl-70 (P = 0.001). Anti-Ro-52 and anti-Scl-70 had increased values for predicting ILD in SSc patients, while anti-Ro-52 had increased values for predicting PAH in SSc patients. ConclusionA commercial line immunoassay was used to evaluate the diagnostic value of autoantibodies in Chinese Han SSc patients. The results clearly indicate that the immunoblot assay has good diagnostic utility for SSc. Anti-Ro-52, anti-RP11, anti-CENPB, anti-CENPA and anti-Scl-70 are useful for diagnosing SSc in the Chinese Han population. Anti-Ro-52, anti-CENPB, anti-CENPA and anti-Scl-70 have a potential value for diagnosing ILD in SSc patients. Furthermore, anti-Ro-52 and anti-Scl-70 may be used to diagnose PAH in SSc patients.

Clinical Characteristics of Systemic Sclerosis With Interstitial Lung Disease.

This study aims to compare the clinical characteristics of systemic sclerosis (SSc) patients with or without interstitial lung disease (ILD), and figure out whether the differences can be useful to suspect ILD in SSc. We retrospectively collected the clinical data of 108 patients with SSc (13 males, 95 females; mean age 50.1±13.5 years; range 14 to 78 years) and compared them according to the presence of ILD. ILD was confirmed by chest computed tomography, and pulmonary arterial hypertension was suspected when right ventricular systolic pressure was ≥40 mmHg based on echocardiography. Of the 108 patients, 49 (45.4) had diffuse type and 59 (54.6) had limited type SSc. Disease duration, percentages of positive anti-scleroderma 70 (anti-Scl70) antibody and anti-centromere antibody, white blood cell, platelet, erythrocyte sedimentation rate (ESR), and presence of pulmonary hypertension differed significantly. On multivariate logistic analysis, positive titer of anti-Scl70 antibody (odds ratio [OR]=15.65, p<0.001), platelet (OR=1.01, p=0.026), ESR (OR=1.02, p=0.037) and pulmonary hypertension (OR=21.97, p=0.003) were associated with ILD in patients with SSc. In SSc patients with ILD, disease duration was longer and positive titer of anti-Scl70 antibody was more frequent, positive titer of anti- centromere antibody was less frequent, and white blood cell and platelet counts, ESR levels, and incidence of possible pulmonary hypertension were significantly higher than in those without ILD. Positive titer of anti-Scl70 antibody, platelet, ESR, and combination of pulmonary hypertension were independently associated with the presence of ILD in SSc patients.

Serum interleukin-34 levels in patients with systemic sclerosis: Clinical association with interstitial lung disease.

Interleukin (IL)-34 is a hematopoietic cytokine promoting proliferation and differentiation of macrophages. Because abnormal activation of macrophages is involved in the development of systemic sclerosis (SSc), we investigated serum IL-34 levels in patients with SSc. Serum IL-34 levels were significantly increased in diffuse cutaneous SSc compared with limited cutaneous SSc and healthy controls, while there were no significant differences between limited cutaneous SSc and healthy controls. In addition, SSc patients with increased serum IL-34 levels more often had interstitial lung disease (ILD) than those with normal levels. Moreover, in SSc patients, serum IL-34 levels negatively correlated with the percentage of predicted vital capacity, while they positively correlated with ground-glass opacity score and fibrotic score on chest computed tomography. Collectively, increased serum IL-34 levels were associated with greater frequency and severity of ILD in SSc patients. Serum IL-34 levels may be a useful serological marker for SSc-associated ILD.

Isolated decrease of the lung diffusing capacity in patients with systemic sclerosis without pulmonary arterial hypertension: a long-term prospective study

The objective of this 5year prospective study was to investigate a clinical role of isolated decrease of DLCO in patients with systemic sclerosis (SS) without pulmonary arterial hypertension (PAH).Methods. We selected 48 out of 142 patients with SS: 35 (73%) with limited SS and 13 (27%) with diffuse SS. The average length of the disease was 12.9 ± 7.9 years. Inclusion criteria were DLCO &lt; 80% pred., forced vital capacity (FVC) ≥ 80% pred. and systolic pulmonary artery pressure (PAP) ≤ 35 mm Hg according to echocardiographic examination (echoCG). High resolution computed tomography (HRCT), spirometry, DLCO measurement, and echoCG were obtained at baseline and after 4.7 ± 1 year of follow up. All patients were treated with standard therapy.Results. CT signs of interstitial lung disease (ILD) were found in 43 (89.6%) patients at baseline and newly developed in 3 other patients during the followup. During the followup, lung CT improved in 5 (10.4%) patients and progressed in 15 (31.3%) patients. Over 5 years, FVC did not change significantly (97.6 ± 10.7% and 100.8 ± 18.9%; р = 0.15), while DLCO significantly decreased both in limited and diffuse SS groups (59.8 ± 13.5% and 56.3 ± 12%; р = 0.006). Mean PAP values remained within normal range in majority of patients. Clinically significant FVC reduction (≥ 10%) was found in 5 patients; of them, CT signs of ILD at baseline were seen in 3 patients and newly developed during the followup in 2 others. Clinically significant DLCO reduction (≥ 10%) was documented in 11 (23%) patients, all had CT signs of ILD at baseline, although CT progression during the followup was noted only in six of them. Contemporary deterioration in FVC, DLCO and CT was found in 3 patients.Conclusion. Clinical course of ILD in SS patients with isolated DLCO reduction and without PAH was relatively benign, with respiratory volumes being preserved within normal range for long time. Comparison of radiological and functional changes in a prospective study has suggested that DLCO is a more sensitive tool to determine ILD progression compared to HRCT. Regular DLCO measurements could be used as a reliable tool for monitoring of ILD associated with SS.

Ressonância magnética pulmonar é semelhante à tomografia de tórax para detectar inflamação em pacientes com esclerose sistêmica

Interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH) are prevalent complications of systemic sclerosis (SS) and are currently the leading causes of death related to the disease. The accurate recognition of these conditions is therefore of utmost importance for patient management.A study was carried out with 24 SS patients being followed at the Rheumatology Department of the Hospital de Clínicas of Universidade Federal do Paraná (UFPR) and 14 healthy volunteers, with the objective of evaluating the usefulness of lung magnetic resonance imaging (MRI) when assessing ILD in SS patients. The results obtained with lung MRI were compared to those obtained by computed tomography (CT) of the chest, currently considered the examination of choice when investigating ILD in SS patients.The assessed population was predominantly composed of women with a mean age of 50 years, limited cutaneous SS, and a disease duration of approximately 7 years. In most cases, there was agreement between the findings on chest CT and lung MRI. Considering it is a radiation‐free examination and capable of accurately identifying areas of lung tissue inflammatory involvement, lung MRI showed to be a useful examination, and further studies are needed to assess whether there is an advantage in using lung MRI instead of chest CT when assessing ILD activity in SS patients.

Detection of Interstitial Lung Disease in Systemic Sclerosis through Partitioning of Lung Transfer for Carbon Monoxide

Background: Interstitial lung disease (ILD) is a leading cause of death in systemic sclerosis (SSc). Sensitivities and specificities of the current pulmonary function tests (PFTs) for the detection of ILD in SSc are poor. Objective: To determine whether diffusion capacity of the lungs for carbon monoxide (DLCO) partitioned into membrane conductance for CO (DmCO) and alveolar capillary blood volume (Vcap) could provide more sensitive clues to ILD than current PFTs. Methods: DmCO and Vcap were determined in 35 consecutive SSc patients in whom a cardiac and/or pulmonary vascular abnormality had been rejected according to the recommended screening algorithm. ILD was diagnosed with high-resolution computed tomography. Results: Among 35 patients [6 men; median age (first–third quartile) 61.9 years (49.5–67.7)], 22 had no ILD and 13 did. Total lung capacity (TLC), vital capacity and DLCO [percentage of predicted value (%pred)] were lower in patients with ILD [86 (82–103) vs. 106 (98–112), p = 0.01, 96 (88–112) vs. 114 (104–121), p = 0.04, and 67 (59–81) vs. 80 (71–94), p = 0.02, respectively]. DmCO (%pred) and the ratio of DmCO to Vcap were much lower in patients with ILD [54 (48–72) vs. 83 (66–92), p < 0.001, and 0.22 (0.21–0.27) vs. 0.40 (0.35–0.53), p < 0.0001, respectively]. According to receiver operating characteristic analysis, the DmCO:Vcap ratio displayed higher sensitivity and specificity than TLC, vital capacity and DLCO in identifying ILD in our study group (p < 0.01). Conclusions: These results suggest that the partitioning of DLCO might be of interest for identifying ILD in SSc patients.