scholarly journals THU0263 LUNG INVOLVEMENT IN PRIMARY SJÖGREN SYNDROME – AN UNDER-DIAGNOSED ENTITY

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 358.1-359
Author(s):  
G. Sogkas ◽  
S. Hirsch ◽  
K. Olsson ◽  
R. Schmidt ◽  
T. Witte ◽  
...  

Background:Interstitial lung disease (ILD) represents a frequent extra-glandular manifestation of primary Sjögren’s Syndrome (pSS). Limited published data regarding phenotyping and treatment exists. Advances in managing specific ILD phenotypes have not been comprehensively explored in patients with coexisting pSS.Objectives:This retrospective study aimed to phenotype lung diseases occurring in a well-described pSS cohort and describe treatment course and outcomes.Methods:Between April 2018 and September 2019, all pSS patients attending our Outpatient clinic were screened for possible lung involvement. Clinical, laboratory and computer tomography (CT) findings were analysed. Patients were classified according to CT findings into 5 groups: usual interstitial pneumonia (UIP), non-specific interstitial pneumonia (NSIP), desquamative interstitial pneumonia (DIP), combined pulmonary fibrosis and emphysema (CPFE) and unspecific-ILD.Results:Lung involvement was confirmed in 24/240 patients (10%). Clinically manifest pSS occurred later in patients with ILD vs. non-ILD (53.2 [42.0-61.7]vs.62.3 [55.6-68.8] years; p=0.0016). The commonest phenotype was UIP n=10 (41%), followed by NSIP n=7 (29%), DIP n=2 (8%), CPFE n=2 (8%) and unspecific-ILD n=3 (13%). Forced vital capacity (FVC) and carbon monoxide diffusion capacity (DLCO) appeared lower in UIP and DIP, without reaching a significant difference. Treatment focused universally on intensified immunosuppression, with 12/24 patients (50%) receiving cyclophosphamide. No anti-fibrotic treatments were used. Follow-up was median 13.2 [7.9-72.3] months, during which 6/24 (25%) patients exhibited a further decline in FVC. No deaths occurred.Conclusion:Lung involvement in pSS is heterogeneous. Better phenotyping and tailored treatment may improve outcomes and requires further evaluation in larger prospective studies.Disclosure of Interests:None declared

2021 ◽  
Vol 10 (12) ◽  
pp. 2548
Author(s):  
Marco Sebastiani ◽  
Fabrizio Luppi ◽  
Gianluca Sambataro ◽  
Diego Castillo Villegas ◽  
Stefania Cerri ◽  
...  

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.


Author(s):  
Eman Ragab ◽  
Asrar Helal Mahrous ◽  
Ghadeer Maher El Sheikh

Abstract Background High-resolution computed tomography (HRCT) has proved to be an important diagnostic tool throughout the COVID-19 pandemic outbreaks. Increasing number of the infected personnel and shortage of real-time transcriptase polymerase chain reaction (RT-PCR) as well as its lower sensitivity made the CT a backbone in diagnosis, assessment of severity, and follow-up of the cases. Results Two hundred forty patients were evaluated retrospectively for clinical, laboratory, and radiological expression in COVID-19 infection. One hundred eighty-six non-severe cases with home isolation and outpatient treatment and 54 severe cases needed hospitalization and oxygen support. Significant difference between both groups was encountered regarding the age, male gender, > 38° fever, dyspnea, chest pain, hypertension, ≤ 93 oxygen saturation, intensive care unit (ICU) admission, elevated D-dimer, high serum ferritin and troponin levels, and high CT-severity score (CT-SS) of the severe group. CT-SS showed a negative correlation with O2 saturation and patients’ outcome (r − 0.73/p 0.001 and r − 0.56/p 0.001, respectively). Bilateral peripherally distributed ground glass opacities (GGOs) were the commonest imaging feature similar to the literature. Conclusion Older age, male gender, smoking, hypertension, low O2 saturation, increased CT score, high serum ferritin, and high D-dimer level are the most significant risk factors for severe COVID-19 pneumonia. Follow-up of the recovered severe cases is recommended to depict possible post COVID-19 lung fibrosis.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1225.1-1225
Author(s):  
I. Perez ◽  
C. Vadillo ◽  
M. A. Nieto ◽  
D. Freites ◽  
Z. Rosales ◽  
...  

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


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 ◽  
...  

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


Author(s):  
Damla Serçe Unat ◽  
Aysu Ayrancı ◽  
Gulru Polat ◽  
Gülistan Karadeniz ◽  
Fatma Demirci Üçsular ◽  
...  

INTRODUCTION: Pulmonary thromboembolism (PTE) is a disease that mortal and hardly diagnosed. Since findings of PTE are nonspesific diagnosed can be so hard sometimes. Major risk factors are malignancy, immobility, previous surgery, thrombophilia, advanced age, and genetic factors. Studies on the relationship between gender and prognosis in PTE have very different results. In this study, we aimed to evaluate gender differences in terms of prognosis, intensive care admission, laboratory, and radiological tests. METHODS: We retrospectively analyzed the data of 348 patients diagnosed with Acute PTE between January 2012 and December 2015. All of these patients were patients who applied to the emergency department of our center, which is a third step chest diseases hospital. We used the PTE guideline jointly designed by the European Society of Cardiology (ESC) and the European Respiratory Society (ERS) for prognosis classifications. RESULTS: The mean age of the patients was 62.7, while the male patients were younger. Women's sPESI scores and mean ages were observed higher than men. Although women had more bad prognostic factors, no significant difference was found between both genders in terms of early mortality. DISCUSSION AND CONCLUSION: Although there is a difference between mean age and simplified Pulmonary Embolism Severity Index (sPESI) scores; no difference in early mortality between men and women. Gender studies in PTE are an area that is open to new studies, since the studies conducted on this subject give quite different results and these results may affect the follow-up protocols.


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 ◽  
...  

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.


2020 ◽  
Vol 8 (5) ◽  
pp. 786
Author(s):  
Alessandra Coscia ◽  
Agata Leone ◽  
Carlotta Rubino ◽  
Ganna Galitska ◽  
Matteo Biolatti ◽  
...  

Cytomegalovirus (CMV) is the leading cause of congenital infection. Its occurrence is phenotypically heterogeneous. The type of maternal infection, primary or non-primary, is an important factor related to the symptomatic disease, the primary infection was long considered the only cause of severe neonatal disease. We aimed to analyze the association of primary and non-primary infection with pathological outcomes in infants and with long-term sequelae at follow-up. This was a monocentric retrospective observational study on a population of 91 infants diagnosed with a CMV infection at the Neonatal Care Unit of Neonatology at the Sant’Anna Hospital of Turin during the period of June 2005 to December 2018. Infants underwent clinical, laboratory, and neuroradiological evaluations at birth. Subsequently, the patients were monitored in an auxological, neurodevelopment, and audiological follow-up. Regarding primary vs. non-primary infection, we found a higher percentage of incidence of symptomatic and neurological localized infection, as well as long-term sequelae in the latter. However, no significant difference between the two populations was found. We underline the possibility of re-infection in previously immunized mothers (non-primary infection) with unfavorable neonatal and long-term outcomes.


2019 ◽  
Vol 19 (1) ◽  
Author(s):  
Toshikazu Watanabe ◽  
Tomoyuki Minezawa ◽  
Midori Hasegawa ◽  
Yasuhiro Goto ◽  
Takuya Okamura ◽  
...  

Abstract Background Myeloperoxidase anti-neutrophil cytoplasmic antibody-related nephritis (MPO-ANCA nephritis) is occasionally accompanied by lung abnormalities such as pulmonary fibrosis. However, the clinical features of pulmonary fibrosis in patients with MPO-ANCA nephritis have not been well documented. This study was performed to compare the prognosis of a usual interstitial pneumonia (UIP) pattern of lung fibrosis in patients with MPO-ANCA nephritis with the prognosis of idiopathic pulmonary fibrosis (IPF). Methods We retrospectively reviewed the medical records of 126 patients with MPO-ANCA nephritis and identified 31 with a UIP pattern of lung fibrosis on high-resolution or thin-slice computed tomography (CT). We compared the characteristics and prognosis of these patients with those of 32 patients with IPF. In 18 patients from both groups, we assessed and compared the decline in lung volume over time using three-dimensional (3D) CT images reconstructed from thin-section CT data. Results The numbers of male and female patients were nearly equal among patients with MPO-ANCA nephritis exhibiting a UIP pattern; in contrast, significant male dominancy was observed among patients with IPF (p = 0.0021). Significantly fewer smokers were present among the patients with MPO-ANCA nephritis with a UIP pattern than among those with IPF (p = 0.0062). There was no significant difference in the median survival time between patients with MPO-ANCA nephritis with a UIP pattern (50.8 months) and IPF (55.8 months; p = 0.65). All patients with IPF in this cohort received antifibrotic therapy (pirfenidone or nintedanib). Almost half of the deaths that occurred in patients with MPO-ANCA nephritis with a UIP pattern were caused by non-respiratory-related events, whereas most deaths in patients with IPF were caused by respiratory failure such as acute exacerbation. In the 3D CT lung volume analyses, the rate of decline in lung volume was equivalent in both groups. Conclusions MPO-ANCA nephritis with a UIP pattern on CT may have an unfavorable prognosis equivalent to that of IPF with a UIP pattern treated with antifibrotic agents.


Thorax ◽  
2010 ◽  
Vol 66 (1) ◽  
pp. 61-65 ◽  
Author(s):  
M. Akira ◽  
Y. Inoue ◽  
T. Arai ◽  
T. Okuma ◽  
Y. Kawata

2021 ◽  
pp. 201010582110414
Author(s):  
Amin R. Soliman ◽  
Tarek Samy Abdelaziz ◽  
Ahmed Fathy

Background Coronavirus disease-19 (COVID-19) is an ongoing pandemic causing considerable fatalities worldwide. Vitamin D modulates the immune response through effects on various cells, such as: macrophages, B and T lymphocytes, neutrophils, and dendritic cells. Aim To explore whether supplementation of vitamin D, in the form of a single intramuscular cholecalciferol injection, to patients with diabetes, COVID-19, and low vitamin D levels could improve the prognosis of those patients. Methods This was a placebo-controlled randomized prospective study. The study has two arms as follows: the intervention arm (40 vitamin D deficient diabetes elderly patients that acquired SARS-CoV-2), compared to the control arm (16 elderly diabetes patients, with deficient vitamin D with SARS-CoV-2). Patients in the intervention arm were given vitamin D as a single intramuscular injection (200,000 IU); patients in the control arm were given placebo. The primary outcome was mortality within 6 weeks of the diagnosis of COVID-19. Clinical, laboratory, treatment, and outcome data were recorded after 6 weeks of follow-up. Results No significant difference in 6 weeks mortality was observed between patients who received vitamin D and patients who received placebo (17.5% vs 18.8%, p = 0.838). Age, presence of hypertension, and chronic obstructive pulmonary disease were independent predictors of mortality at 6 weeks. Conclusion Vitamin D supplementation did not reduce the severity or mortality of COVID-19 at 6 weeks. Further large scale studies are required to explore the effect of vitamin D therapy on survival in patients with diabetes mellitus who acquire COVID-19.


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