scholarly journals SAT0535 CLINICAL COURSE IN PATIENTS WITH INTERSTITIAL PNEUMONIA WITH AUTOIMMUNE FEATURES (IPAF) IN A MULTIDISCIPLINARY CONSULTATION.

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1225.1-1225
Author(s):  
I. Perez ◽  
C. Vadillo ◽  
M. A. Nieto ◽  
D. Freites ◽  
Z. Rosales ◽  
...  
Keyword(s):  
Incidence Rate ◽  
Interstitial Pneumonia ◽  
Usual Interstitial Pneumonia ◽  
Classification Criteria ◽  
Therapeutic Management ◽  
European Respiratory Society ◽  
Respiratory Impairment ◽  
Radiological Pattern ◽  
Over Time

Background:A proportion of patients with Interstitial Lung Disease (ILD) show autoimmune characteristics but do not completely meet the classification criteria for a definitive connective tissue disease. In order to unify the nomenclature and criteria to define this condition, the classification of patients with Interstitial Pneumonia with Autoimmune Features (IPAF) has recently been adopted (Fisher, et al).Objectives:To describe the sociodemographic, clinical, functional characteristics and therapeutic management of IPAF patients in clinical practice and to evaluate the incidence rate of functional respiratory impairment over time.Methods:A longitudinal observational study was performed. Patients with IPAF classification criteria (Fischer et al) were included from the time of ILD diagnosis (Feb 2017 to Sept 2018) and followed until loss of follow-up or end of the study (Oct 2019), in a multidisciplinary team, carried by a pneumologist and a rheumatologist in a Tertiary Hospital in Madrid. Main outcome: relative functional respiratory impairment: defined as decline in percent predicted forced vital capacity (FVC%) of ≥ 5% compared to the previous visit. Respiratory function was measured at baseline and every 6 months. Covariates: a) sociodemographic, b) clinical, c) radiological pattern (non-specific interstitial pneumonia [NSIP]; usual interstitial pneumonia [UIP], others); d) FVC%, DLCO%; e) laboratory tests; f) therapy used (glucorticosteroids, disease modifying antirheumatic drugs (DMARDs) and Biologic Agents). Statistical analysis: description of the sociodemographic, clinical, radiological, functional and treatment characteristics of the patients. Survival techniques were used to estimate the incidence rate (IR) of relative functional respiratory impairment, expressed per 100 patient-semester with their respective confidence interval [95 % CI].Results:17 patients were included with a mean follow-up of 3 ± 1.5 years, 70.6% were women with a mean age of 65±10 years. The most frequent IPAF classification criteria were: a) clinical: arthritis (50%), Raynaud`s phenomenon (33%) and mechanical hands (17%); b) serological: 65% had ANA ≥1/360; 31% FR> 40; 30% Anti-Ro positive; c) morphologic: 59% presented NSIP pattern and 29.4% was UIP. The baseline median FVC% and DLCO% were 89 [83-107.7] and 63 [50-79.8] respectively. During the study period, 94% received treatment: 87.5% glucorticosteroids, 68.5% mycophenolate, 56% azathioprine, 18.7% cyclophosphamide iv and 33% antifibrotics. During the follow-up (104.6 patient - semester), 15 patients presented relative functional respiratory impairment, with an IR of 23.8 [16.1-35.3]. After 14 months from IPAF diagnosis 50% of the patients had relative functional respiratory impairment. At the end of the follow-up, 50% showed a worsening of the DLCO%.Conclusion:IPAF patients are mostly women in their sixties. The most frequent clinical criteria are arthritis and Raynaud`s phenomenon and the serological were FR and ANAs. The most frequent radiological pattern was NSIP. The therapeutic management is mainly with glucorticosteroids, mycophenolate and azathioprine. At the beginning, patients have a slightly diminished lung function. These patients have significant functional impairment over time that will impact in their prognosis. Longitudinal and multicenter studies are necessary to advance in the knowledge and management of these patients.References:[1]Fischer A, Antoniou KM, Brown KK, Cadranel J, Corte TJ, du Bois RM, et al. An official European Respiratory Society/American Thoracic Society research statement: interstitial pneumonia with autoimmune features. Eur Respir J [Internet]. 2015 Oct;46(4):976–87. Available from:http://www.ncbi.nlm.nih.gov/pubmed/26160873Disclosure of Interests:None declared

scholarly journals POS1435 CLINICAL CHARACTERIZATION AND PREDICTIVE FACTORS FOR PROGRESSION IN A COHORT OF ILD PATIENTS WITH FEATURES OF AUTOIMMUNITY: ARE IPAF CRITERIA SUFFICIENT?

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1002.1-1002
Author(s):  
F. Bozzao ◽  
P. Tomietto ◽  
E. Baratella ◽  
F. Giudici ◽  
M. Kodric ◽  
...  
Keyword(s):  
Interstitial Pneumonia ◽  
Predictive Factors ◽  
Usual Interstitial Pneumonia ◽  
Final Diagnosis ◽  
Classification Criteria ◽  
Radiological Progression ◽  
Nonspecific Interstitial Pneumonia ◽  
The Mean ◽  
Functional Deterioration ◽  
Worse Prognosis

Background:It is unknown whether patients with interstitial lung disease (ILD) and only some features of autoimmunity have a different natural history from those with a defined connective tissue disease (CTD-ILD). The classification criteria for “ILD with autoimmune features” (IPAF) may not be able to characterize all these patients, especially those with a usual interstitial pneumonia (UIP) pattern [1].Objectives:To determine clinical characteristics and predictive factors for progression in a cohort of ILD patients with features of autoimmunity, through the application of classification criteria for IPAF and specific CTD, whenever possible.Methods:We retrospectively selected a cohort of consecutive patients with ILD as onset manifestation and features of autoimmunity (at least 1 autoantibody and/or 1 clinical sign/symptom), evaluated by our multidisciplinary unit from March 2009 to March 2020. All the final diagnoses were revised according to the latest CTD and IPAF criteria. Patients were followed up for 33 (16.5-69.5) months.Results:Of the 101 patients enrolled (67.4±10.9 yrs, F/M ratio 65/36), 53 (52.5%) and 37 (36.6%) respectively satisfied the CTD and IPAF criteria. Eleven patients (10.9%) did not satisfy IPAF criteria because of only 1 item (clinical or serologic) within the IPAF domains and a UIP pattern; we defined this group as “autoimmune” UIP (AI-UIP). All the 8 patients initially classified as undifferentiated CTD had sufficient IPAF criteria. Among the IPAF patients (68.2±10.1 years, F/M ratio 20/17), the most common findings were: Nonspecific interstitial pneumonia pattern (56.8%), antinuclear antibodies positivity (43.2%) and arthritis (24.3%). The combination of a positive morphologic and serologic domain was the most common to reach the diagnosis (48.6%). Some IPAF patients had features not included in IPAF criteria, such as non-anti-synthetase myositis-specific antibodies (21.6%), objective sicca syndrome (13.5%) and anti-myeloperoxidase antibodies (2.7%). Over a median of 17 months, 2 IPAF patients (5.4%) developed a definite UIP pattern, while 4 (10.8%) a specific CTD. Comparing the IPAF, CTD-ILD and AI-UIP groups, no statistically significant differences were found in the mean age, sex distribution, smoking habits and mean duration of the disease. However, IPAF patients had a significantly higher prevalence of arterial hypertension and left-sided heart failure and a lower predominance of UIP pattern as expected (10.8% vs. 32.1% vs. 100%, p<0.01). Although no differences were found at the diagnosis, at 1 year the proportion of IPAF patients with radiological progression of the fibrosis and/or functional deterioration (defined by a decline in FVC of ≥ 10% and/or DLCO of ≥ 15% predicted) was lower to that of CTD-ILD and AI-UIP (17.1% vs. 31.4% vs. 63.6%, p 0.01). Fewer IPAF patients needed oxygen support (8.6% vs. 31.4% vs. 36.4, p 0.02). Considering the overall 101 patients, having an IPAF and a UIP pattern respectively predicted a slower (OR: 0.37, p 0.04) and a faster (OR: 3.56, p 0.01) ILD progression at the multivariate analysis.Conclusion:In our cohort, IPAF criteria were useful to identify a subset of patients with a slower ILD progression and a possible evolution to CTD (10-15% of cases) [2]. These criteria do not characterize all the patients with a UIP pattern and limited features of autoimmunity, which seem to have a worse prognosis, independently from the final diagnosis. Further studies are needed to clarify if the prognosis of AI-UIP is different from that of idiopathic pulmonary fibrosis.References:[1]Graney, et al. Ann Am Thorac Soc 2019;16(5):525-33.[2]Sebastiani, et al. Biomedicines 2021,9,17.Disclosure of Interests:None declared

scholarly journals FRI0495 FOLLOW UP OF INTERSTITIAL PNEUMONIA WITH AUTOIMMUNE FEATURES – THE EXPERIENCE OF ONE CENTRE

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 846.3-846
Author(s):  
N. Madeira ◽  
M. Alvarenga Santos ◽  
L. Cunha Miranda ◽  
S. Clemente ◽  
S. Furtado
Keyword(s):  
Interstitial Pneumonia ◽  
Respiratory Symptoms ◽  
Interstitial Lung Diseases ◽  
European Respiratory Society ◽  
High Resolution Computed Tomography ◽  
Disease Evolution ◽  
Clinical Criteria ◽  
Clinical Evolution ◽  
Relative Decline

Background:Interstitial Lung Diseases (ILD) may present features suggesting an underlying autoimmune process, which seem to differentiate them from idiopathic interstitial pneumonias, although without fully meeting the classification criteria (CC) for a specific connective tissue disease. Different terms had been used to describe these conditions and, to reach a consensus, the European Respiratory Society/American Thoracic Society proposed the CC for an entity named Interstitial Pneumonia with Autoimmune Features (IPAF). Clinical evolution and prognosis of this entity are still poorly understood.Objectives:To evaluate clinical evolution and prognosis of a population of patients with IPAF.Methods:Retrospective analysis of clinical files of patients followed by the Pulmonology Department since 02/2012 until 06/2019, who met the CC for IPAF, regarding clinical, functional and radiological evolution. Patients were considered to have a progressive phenotype in 24±3 months from their 1stevaluation if they fulfil 1 of the 4 criteria: relative decline in FVC ≥10% predicted; relative decline in FVC ≥5–<10% predicted and worsened respiratory symptoms; relative decline in FVC ≥5–<10% predicted and increased extent of fibrosis on High-resolution Computed Tomography (HRCT); worsened respiratory symptoms and increased extent of fibrosis on HRCT.Results:22 (7.4%) of 296 ILD patients met IPAF CC. 59.0% were female with an age at the 1stevaluation of 66.7±12.4 years. They were all non-smokers (63.6%) or ex-smokers (36.4%). Serologic and morphologic criteria were both present in 21 (95.4%) and clinical criteria in 5 patients (22.7%). Antinuclear antibodies (ANA) were identified in 19, rheumatoid factor in 4, SSA in 3 and anti-Jo-1 in 1 patient. HRCT patterns were identified in 21 patients: 15 nonspecific interstitial pneumonia (NSIP), 5 organizing pneumonia (OP) and 2 lymphocytic interstitial pneumonia (LIP). One NSIP and 1 LIP identified on HRCT were confirmed by histopathology. Three patients had inflammatory arthritis and 2 had Raynaud’s phenomenon. Immunosuppressive therapy was introduced in most cases (18 patients, including systemic corticotherapy in 17, azathioprine in 4, mycophenolate mofetil in 1), azithromycin was prescribed in 2 patients and 3 remained without therapy. Regarding the follow up at 24±3 months from the 1stevaluation (3 patients were excluded due to too recent follow-up), 4 patients (18.2%) had progressive phenotype, 7 (31.8%) had a favourable evolution and 3 (13.6%) patients had died. During a follow-up of 31.1±19.8 months, this number rose to 6 patients (27.3%), all of them died by respiratory cause and had NSIP pattern. No differences were found in age, last FVC, therapy and time of disease evolution between those who died and the others.Conclusion:Our study showed that a small proportion of IPAF patients had a progressive phenotype and the NSIP pattern seemed to be a poor prognosis factor for survival.References:[1]Ito Y, Arita M, Kumagai S, et al. Serological and morphological prognostic factors in patients with interstitial pneumonia with autoimmune features. BMC Pulm Med 2017; 17:111 10.1186/s12890-017-0453-zDisclosure of Interests:None declared

scholarly journals Interstitial Lung Disease and Anti-Myeloperoxidase Antibodies: Not a Simple Association

2021 ◽  
Vol 10 (12) ◽  
pp. 2548
Author(s):  
Marco Sebastiani ◽  
Fabrizio Luppi ◽  
Gianluca Sambataro ◽  
Diego Castillo Villegas ◽  
Stefania Cerri ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Rheumatic Disease ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Usual Interstitial Pneumonia ◽  
Clinical History ◽  
High Resolution Computed Tomography ◽  
Function Testing ◽  
And Diffusion

Anti-neutrophil cytoplasmic antibodies (ANCA), mainly anti-myeloperoxidase (MPO) antibodies, have been frequently identified in patients with idiopathic pulmonary fibrosis (IPF). However, their role remains unclear, and only 7–23% of these patients develops clinically overt vasculitis. We aimed to investigate the clinical, serological, and radiological features and prognosis of anti-MPO-positive interstitial lung disease (ILD) patients. Fifty-eight consecutive patients firstly referred for idiopathic interstitial pneumonia and showing serological positivity of anti-MPO antibodies were retrospectively enrolled. For each patient, clinical data, lung function testing, chest high-resolution computed tomography (HRCT) pattern, and survival were recorded. Thirteen patients developed a rheumatic disease during a median follow-up of 39 months. Usual interstitial pneumonia (UIP) was the most frequent ILD pattern, significantly influencing the patients’ survival. In fact, while the 52-week survival of the overall population was 71.4 ± 7.5%, significantly higher than IPF, survivals of anti-MPO patients with UIP pattern and IPF were similar. Forced vital capacity and diffusion lung capacity for CO significantly declined in 37.7 and 41.5% of cases, respectively, while disease progression at chest HRCT was observed in 45.2%. A careful clinical history and evaluation should always be performed in ILD patients with anti-MPO antibodies to quickly identify patients who are developing a systemic rheumatic disease.

scholarly journals SAT0529 CLINICAL CHARACTERISTICS AND TREATMENT PATTERNS IN A PATIENT GROUP WITH INTERSTITIAL LUNG DISEASE.

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1222.2-1222
Author(s):  
R. Ortega Castro ◽  
P. S. Laura ◽  
F. U. Pilar ◽  
J. Calvo Gutierrez ◽  
A. Requejo-Jimenez ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Smoking Habit ◽  
Inflammatory Myopathy ◽  
Usual Interstitial Pneumonia ◽  
Treatment Patterns ◽  
Common Symptom ◽  
Radiological Pattern ◽  
Mechanic’S Hands

Background:Diffuse interstitial lung disease (ILD) is frequently associated with connective tissue diseases (CTD) and is one of the main causes of morbidity and mortality in these patients. Recently, the concept of Interstitial Pneumonia with Autoimmune Features (IPAF) has been defined to characterize ILD associated with systemic manifestations limited to subtle serological and clinical autoimmune abnormalities and not fulfilling the international criteria for the diagnosis of a given CTD.Objectives:The objective of this study is to describe the clinical, serological and radiological characteristics, as well as the treatment patterns of patients with ILD referred to a Rheumatology Service for suspected CTDMethods:Observational, cross-sectional study of 43 patients with ILD referred for evaluation to the medical consultation of CTD of the Rheumatology service at the Reina Sofía Hospital. Patients were classified as patients with defined CTD, patient with IPAF and patients with other types of pneumopathy. We conducted a descriptive study of all patients and compared the clinical-analytical-radiological characteristics and treatment patterns of the first two groups.Results:Of the 43 patients, 67.40% were women with a mean age at diagnosis of 65.65 (10.42) years and 53.50% of smoking patientsOf the total of patients, 16 (37.2%) were included in the CTD group, 17 (39.5%) met criteria for IPAF and 10 (23.3%) had another type of pneumopathy.In the CTD group scleroderma was the most frequent disease (6/16), followed by inflammatory myopathy (4/16), Sjögren’s syndrome (3/16), rheumatoid arthritis (2/16) and polymyalgia rheumatic (1/16). In this group of patients, the most common symptom was Raynaud’s phenomenon (RP) (7/16), followed by arthritis (7/16) and mechanic’s hands (3/16). Regarding the most frequently antibodies were ANA (100%), anti-RO (41.7%), anti-citrullinated protein antibodies (30%) and rheumatoid factor (RF) (28.6%).In patients with IPAF, as in the CTD group, the most observed clinical criterion was RP (5/17), followed by arthritis (1/17) and mechanic’s hands (1/17). Among the serological criteria the most common antibodies were ANA (100%), followed by anti-RO (33.3%), anti-RNA synthetase (28.6%) and RF (22.2%).Regarding the radiological pattern, in both groups the most frequent was nonspecific interstitial pneumonia, followed by the indeterminate pattern and usual interstitial pneumonia (UIP) in third place. There were no significant differences by gender and age, between the group of CTD and IPAF, observing in both groups a predominance of women with a similar mean age, being the upper smoking habit in the IPAF group (70.6% vs 31.5%, p= 0.02). Regarding the treatment used, the use of immunosuppressants (IS) was more frequent in CTD group (56.3% vs 11.8%, p = 0.007).Conclusion:The clinical-serological and radiological characteristics were similar among patients with IPAF and CTD, which supports the notion of a similar pathophysiology in both groups. In our cohort patients with CTD received IS more frequently than IPAF group, however, future work would be necessary to assess whether the response to treatment is similar in these populations and if IS can benefit patients with IPAF to long term. In addition, it could be useful to include the UIP pattern within the IPAF classification criteria, not currently included, since it is the third most frequent radiological pattern.References:[1]Respirology, 21 (2016), pp. 245-258[2]Eur Respir J, 46 (2015), pp. 976-987Disclosure of Interests:Rafaela Ortega Castro: None declared, Pérez Sánchez Laura: None declared, Font Ugalde Pilar: None declared, Jerusalem Calvo Gutierrez: None declared, Antonio Requejo-Jimenez: None declared, Simona Espejo-Pérez: None declared, Teresa Gonzalez-Serrano: None declared, María del Carmen Castro Villegas: None declared, Gómez García Ignacio: None declared, Alejandro Escudero Contreras: None declared, Eduardo Collantes Estevez Grant/research support from: ROCHE and Pfizer, Speakers bureau: ROCHE, Lilly, Bristol and Celgene, Maria A Aguirre: None declared

scholarly journals THU0305 PREVALENCE AND CLINICAL OUTCOME OF INTERSTITIAL LUNG DISEASE IN ANCA ASSOCIATED VASCULITIS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 381.2-381
Author(s):  
J. Fernandes Serodio ◽  
J. Hernández-Rodríguez ◽  
G. Espígol-Frigolé ◽  
M. Alba ◽  
J. Marco-Hernández ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Cox Regression ◽  
Clinical Course ◽  
Usual Interstitial Pneumonia ◽  
Classification Criteria ◽  
Lung Involvement ◽  
Anca Associated Vasculitis ◽  
Inflammatory Infiltrates

Background:Lung involvement is frequent in ANCA-associated vasculitis (AAV). Classical lung manifestations consist of capillaritis with lung haemorrhage, inflammatory infiltrates and nodules. Interstitial lung disease (ILD) is increasingly recognized among patients with AAV. However, little is known concerning risk factors and clinical course of these patients.Objectives:The aim of our study was to characterize the prevalence and clinical course of ILD in patients with AAV.Methods:We have performed a clinical retrospective single-centre observational analysis (1990-2019) of all patients with the diagnosis of microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA) diagnosed according to 2018 Draft Classification Criteria for GPA and MPA1. Demographic, clinical and immunologic data were reviewed. Radiologic pattern of ILD were assessed by high-resolution-CT. Main outcome evaluated was overall-all survival.Results:The study population consisted of 123 patients, 56% female, aged 59.3±18.2 years old at the time of diagnosis. Clinical diagnosis was of MPA in 54% of patients and GPA in 46%. While 108 (88%) ANCA positive patients had PR3 (n=25) or MPO (n=83), 15 (12%) patients had negative or atypical ANCA. Any lung involvement was present in 82 (71%) and ILD was identified in 24 (20%) of all patients. ILD pattern was of usual interstitial pneumonia (UIP) in 12 patients, non-specified interstitial pneumonia (NSIP) in 9 and chronic organizing pneumonia (OP) in 3. There was an association between the presence of ILD and ANCA specificity: MPO were present in 100% of patients with UIP and in 75% of patients with NSIP/OP (p=0.017). Bronchiectasis were more prevalent among patients with ILD (19/24; p<0.001). During the median follow-up time period of 68 (23-126) months, mortality was of 42% among patients with ILD-AAV compared with 11% in no ILD-AAV (log-rank p=0.0001). On the multivariate Cox regression model, ILD was an independent predictor of mortality HR 2.95 (95%CI 1.09-7.96; p=0.033).Conclusion:ILD is a frequent manifestation of MPA and GPA patients. The presence of ILD, particularly UIP, is associated with ANCA-MPO and is a predictor of mortality. Therefore, a better management of fibrotic lung involvement in AAV is warranted.References:[1]Robson JC, Grayson PC, Ponte C, et al. Draft classification criteria for the ANCA associated vasculitides. Ann Rheum Dis 2018;77 (suppl 2):60-1.Disclosure of Interests:João Fernandes Serodio: None declared, José Hernández-Rodríguez: None declared, Georgina Espígol-Frigolé: None declared, Marco Alba: None declared, Javier Marco-Hernández: None declared, Marcelo Sánchez: None declared, Fernanda Hernández-González: None declared, Jacobo Sellarés: None declared, Maria C. Cid Grant/research support from: Kiniksa Pharmaceuticals, Consultant of: Janssen, Abbvie, Roche, GSK, Speakers bureau: Vifor, Sergio Prieto-González: None declared

Abatacept in interstitial lung disease associated with rheumatoid arthritis: national multicenter study of 263 patients

Rheumatology ◽  
2020 ◽  
Vol 59 (12) ◽  
pp. 3906-3916 ◽  
Author(s):  
Carlos Fernández-Díaz ◽  
Santos Castañeda ◽  
Rafael B Melero-González ◽  
Francisco Ortiz-Sanjuán ◽  
Antonio Juan-Mas ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Standard Dose ◽  
Usual Interstitial Pneumonia ◽  
Point Change ◽  
Lung Capacity ◽  
Sparing Effect ◽  
Severe Degree

Abstract Objective To assess the efficacy of abatacept (ABA) in RA patients with interstitial lung disease (ILD) (RA-ILD). Methods This was an observational, multicentre study of RA-ILD patients treated with at least one dose of ABA. ILD was diagnosed by high-resolution CT (HRCT). We analysed the following variables at baseline (ABA initiation), 12 months and at the end of the follow-up: Modified Medical Research Council (MMRC) scale (1-point change), forced vital capacity (FVC) or diffusion lung capacity for carbon monoxide (DLCO) (improvement or worsening ≥10%), HRCT, DAS on 28 joints evaluated using the ESR (DAS28ESR) and CS-sparing effect. Results We studied 263 RA-ILD patients [150 women/113 men; mean (s.d.) age 64.6 (10) years]. At baseline, they had a median duration of ILD of 1 (interquartile range 0.25–3.44) years, moderate or severe degree of dyspnoea (MMRC grade 2, 3 or 4) (40.3%), FVC (% of the predicted) mean (s.d.) 85.9 (21.8)%, DLCO (% of the predicted) 65.7 (18.3) and DAS28ESR 4.5 (1.5). The ILD patterns were: usual interstitial pneumonia (UIP) (40.3%), non-specific interstitial pneumonia (NSIP) (31.9%) and others (27.8%). ABA was prescribed at standard dose, i.v. (25.5%) or s.c. (74.5%). After a median follow-up of 12 (6–36) months the following variables did not show worsening: dyspnoea (MMRC) (91.9%); FVC (87.7%); DLCO (90.6%); and chest HRCT (76.6%). A significant improvement of DAS28ESR from 4.5 (1.5) to 3.1 (1.3) at the end of follow-up (P &lt; 0.001) and a CS-sparing effect from a median 7.5 (5–10) to 5 (2.5–7.5) mg/day at the end of follow-up (P &lt; 0.001) was also observed. ABA was withdrawn in 62 (23.6%) patients due to adverse events (n = 30), articular inefficacy (n = 27), ILD worsening (n = 3) and other causes (n = 2). Conclusion ABA may be an effective and safe treatment for patients with RA-ILD.

scholarly journals Rituximab in the Treatment of Interstitial Lung Disease Associated with Autoimmune Diseases: Experience from a Single Referral Center and Literature Review

2020 ◽  
Vol 9 (10) ◽  
pp. 3070
Author(s):  
Belén Atienza-Mateo ◽  
Sara Remuzgo-Martínez ◽  
Diana Prieto-Peña ◽  
Víctor Manuel Mora Cuesta ◽  
David Iturbe-Fernández ◽  
...  
Keyword(s):  
Literature Review ◽  
Interstitial Pneumonia ◽  
Therapeutic Option ◽  
Pulmonary Function Tests ◽  
Usual Interstitial Pneumonia ◽  
Inflammatory Myositis ◽  
Idiopathic Inflammatory Myositis ◽  
Referral Center ◽  
Radiological Pattern ◽  
One Year

In the present study, we aimed to report our experience with rituximab (RTX) in the treatment of patients with ILD associated with AD (AD-ILD) at a single center. For this purpose, clinical characteristics, radiological findings, and pulmonary function tests (PFTs) of RTX-treated AD-ILD-patients seen from May 2016 until March 2020 at a referral center for individuals with ILD were retrospectively reviewed. Additionally, an updated literature review was conducted. A total of 26 patients (mean age 58.3 ± 11.1 years at ILD diagnosis) was included. The most common ADs related to ILD were systemic sclerosis, idiopathic inflammatory myositis (including anti-synthetase syndrome) and rheumatoid arthritis. Non-specific interstitial pneumonia (n = 12) and usual interstitial pneumonia (n = 11) were the predominant radiological patterns. The sustained improvement in PFTs was observed from the start of RTX, with a statistically significant increase in DLCO from basal to one year after RTX (mean + 4.2%, p = 0.024). Overall, there were no differences when comparing PFT outcome according to the radiological pattern or the specific type of AD. In conclusion, RTX constitutes a good therapeutic option to preserve lung function in patients with AD-ILD, regardless of the radiological pattern or the underlying AD.

scholarly journals Predictors of mortality in rheumatoid arthritis-associated interstitial lung disease

2015 ◽  
Vol 47 (2) ◽  
pp. 588-596 ◽  
Author(s):  
Joshua J. Solomon ◽  
Jonathan H. Chung ◽  
Gregory P. Cosgrove ◽  
M. Kristen Demoruelle ◽  
Evans R. Fernandez-Perez ◽  
...  
Keyword(s):  
Rheumatoid Arthritis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Usual Interstitial Pneumonia ◽  
High Resolution Computed Tomography ◽  
Predictors Of Mortality ◽  
Risk Of Death ◽  
Increased Risk ◽  
Over Time

Interstitial lung disease (ILD) is a common pulmonary manifestation of rheumatoid arthritis. There is lack of clarity around predictors of mortality and disease behaviour over time in these patients.We identified rheumatoid arthritis-related interstitial lung disease (RA-ILD) patients evaluated at National Jewish Health (Denver, CO, USA) from 1995 to 2013 whose baseline high-resolution computed tomography (HRCT) scans showed either a nonspecific interstitial pneumonia (NSIP) or a “definite” or “possible” usual interstitial pneumonia (UIP) pattern. We used univariate, multivariate and longitudinal analytical methods to identify clinical predictors of mortality and to model disease behaviour over time.The cohort included 137 subjects; 108 had UIP on HRCT (RA-UIP) and 29 had NSIP on HRCT (RA-NSIP). Those with RA-UIP had a shorter survival time than those with RA-NSIP (log rank p=0.02). In a model controlling for age, sex, smoking and HRCT pattern, a lower baseline % predicted forced vital capacity (FVC % pred) (HR 1.46; p<0.0001) and a 10% decline in FVC % pred from baseline to any time during follow up (HR 2.57; p<0.0001) were independently associated with an increased risk of death.Data from this study suggest that in RA-ILD, disease progression and survival differ between subgroups defined by HRCT pattern; however, when controlling for potentially influential variables, pulmonary physiology, but not HRCT pattern, independently predicts mortality.

Prevalence and incidence of pulmonary arterial hypertension: 10-year follow-up of an unselected systemic sclerosis cohort

2017 ◽  
Vol 2 (3) ◽  
pp. 196-202 ◽  
Author(s):  
Els Vandecasteele ◽  
Karin Melsens ◽  
Kristof Thevissen ◽  
Michel De Pauw ◽  
Ellen Deschepper ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Incidence Rate ◽  
Specific Treatment ◽  
Early Screening ◽  
European Respiratory Society ◽  
Pulmonary Arterial ◽  
Incident Cases

Introduction Early screening for pulmonary arterial hypertension (PAH) in systemic sclerosis (SSc) improves outcome. Therefore, we evaluated the screening for PAH during the 10-year follow-up of an unselected prospective SSc cohort by calculating the prevalence and the incidence rate of PAH and we compared the screening before and after implementation of the 2009 European Society of Cardiology/European Respiratory Society (ESC/ERS) guidelines. Methods Data were evaluated from each SSc-specific visit of 362 consecutive SSc patients included in the SSc Cohort of the Ghent University between May 2006 and December 2015. Results Of the 362 included patients, 23.2% had limited SSc, 59.9% limited cutaneous SSc and 16.9% diffuse cutaneous SSc. At baseline, one patient was already on PAH-specific treatment and eight patients were diagnosed with PAH, implicating a baseline PAH prevalence of 2.5% (9/362). During follow-up (median of 18 months [interquartile range: 0-54 months]), nine patients were diagnosed with incidental PAH, resulting in an incidence rate of 9.3/1000 person-years, 95% confidence intervals (95% CI): 4.3-17.7. Before the ESC/ERS guidelines, five PAH patients, all already diagnosed with prevalent PAH, were included in the cohort. After 2009, 13 patients (4 prevalent cases) were diagnosed with PAH, making the yearly incidence around 1% (0.82%-2.00%). Conclusions During 10-year follow-up in a cohort of 362 unselected SSc patients, the cumulative prevalence of PAH is 5% (18/362) and the incidence rate 9.3/1000 person-years, 95% CI: 4.3-17.7. Before implementation of the 2009 ESC/ERS screening algorithm, there were no incident cases.

scholarly journals An official European Respiratory Society/American Thoracic Society research statement: interstitial pneumonia with autoimmune features

2015 ◽  
Vol 46 (4) ◽  
pp. 976-987 ◽  
Author(s):  
Aryeh Fischer ◽  
Katerina M. Antoniou ◽  
Kevin K. Brown ◽  
Jacques Cadranel ◽  
Tamera J. Corte ◽  
...  
Keyword(s):  
Connective Tissue ◽  
Interstitial Pneumonia ◽  
Connective Tissue Disease ◽  
Task Force ◽  
American Thoracic Society ◽  
Classification Criteria ◽  
Future Research ◽  
Autoimmune Process ◽  
European Respiratory Society ◽  
Society Research

Many patients with an idiopathic interstitial pneumonia (IIP) have clinical features that suggest an underlying autoimmune process but do not meet established criteria for a connective tissue disease (CTD). Researchers have proposed differing criteria and terms to describe these patients, and lack of consensus over nomenclature and classification limits the ability to conduct prospective studies of a uniform cohort.The “European Respiratory Society/American Thoracic Society Task Force on Undifferentiated Forms of Connective Tissue Disease-associated Interstitial Lung Disease” was formed to create consensus regarding the nomenclature and classification criteria for patients with IIP and features of autoimmunity.The task force proposes the term “interstitial pneumonia with autoimmune features” (IPAF) and offers classification criteria organised around the presence of a combination of features from three domains: a clinical domain consisting of specific extra-thoracic features, a serologic domain consisting of specific autoantibodies, and a morphologic domain consisting of specific chest imaging, histopathologic or pulmonary physiologic features.A designation of IPAF should be used to identify individuals with IIP and features suggestive of, but not definitive for, a CTD. With IPAF, a sound platform has been provided from which to launch the requisite future research investigations of a more uniform cohort.

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