Nonspecific Interstitial Pneumonia Pattern Research Articles

High resolution computed tomography in systemic sclerosis: From diagnosis to follow-up.

Early diagnosis of interstitial lung disease (ILD) and pulmonary hypertension (PH) is crucial in systemic sclerosis (SSc) for both management and treatment. However, diagnosing SSc-ILD can be challenging because symptoms of lung involvement are often non-specific at the early stages of disease. High-resolution computed tomography (HRCT) of the chest is recognized as the most accurate imaging modality for baseline and follow-up evaluation of SSc-ILD. Key features of SSc-ILD on HRCT include a non-specific interstitial pneumonia (NSIP) pattern, with peripheral ground-glass opacities and extensive traction bronchiectasis. Less common HRCT manifestations include usual interstitial pneumonia (UIP) pattern, followed by diffuse alveolar damage (DAD), diffuse alveolar hemorrhage (DAH) and organizing pneumonia (OP). The extent of disease on HRCT is known to relate with prognosis and serial assessments can be helpful in monitoring disease progression or treatment response. We discuss the main chest computed tomography (CT) manifestations of SSc, highlighting the role of imaging at both baseline and follow-up evaluations.

Diagnosis of pulmonary sarcoidosis comorbid with non-specific interstitial pneumonia: a case report

BackgroundAlthough the imaging manifestations of pulmonary sarcoidosis have been described in detail in previous studies, a consensus has not been reached on the imaging presentation of non-specific interstitial pneumonia (NSIP) lookalike pattern as a distinct pattern in the diagnosis of pulmonary sarcoidosis in high-resolution computed tomography (HRCT). No cases of pulmonary sarcoidosis comorbid with NSIP have been reported.Case presentationA 53-year-old male presented to the hospital with a five-year history of recurrent coughing up sputum and a four-year history of shortness of breath. In addition to the typical features of pulmonary sarcoidosis, the patient’s HRCT also showed unexpected interstitial changes in the lower lobes of both lungs, suggesting an NSIP pattern. Histopathology of the lung tissue in this region confirmed well-formed noncaseating epithelioid granulomas and pathological modifications of NSIP. After a rigorous exclusion diagnosis combining the patient’s clinical features, radiological and pathological findings, we diagnosed this patient with pulmonary sarcoidosis comorbid with NSIP.ConclusionsThis suggests that NSIP may act as a rare comorbidity of pulmonary sarcoidosis thereby resulting in the patient’s HRCT presenting differently from routine sarcoidosis imaging.

UNRAVELING THE UNEXPECTED: SARCOIDOSIS WITH NSIP PATTERN

UNRAVELING THE UNEXPECTED: SARCOIDOSIS WITH NSIP PATTERN

Pathological features of connective tissue disease-associated interstitial lung disease in transbronchial cryobiopsies.

Transbronchial cryobiopsies are increasingly used for the diagnosis of interstitial lung disease (ILD), but there is a lack of published information on the features of specific ILD in cryobiopsies. Here we attempt to provide pathological guidelines for separating usual interstitial pneumonia (UIP) of idiopathic pulmonary fibrosis (IPF), fibrotic hypersensitivity pneumonitis (FHP) and connective tissue disease-associated ILD (CTD-ILD) in cryobiopsies. We examined 120 cryobiopsies from patients with multidisciplinary discussion (MDD)-established CTD-ILD and compared them to a prior series of 121 biopsies from patients with MDD-established IPF or FHP. A non-specific interstitial pneumonia (NSIP) pattern alone was seen in 36 of 120 (30%) CTD-ILD, three of 83 (3.6%) FHP and two of 38 (5.2%) IPF cases, statistically favouring a diagnosis of CTD-ILD. The combination of NSIP + OP was present in 29 of 120 (24%) CTD-ILD, two of 83 (2.4%) FHP and none of 38 (0%) IPF cases, favouring a diagnosis of CTD-ILD. A UIP pattern, defined as fibroblast foci plus any of patchy old fibrosis/fibrosis with architectural distortion/honeycombing, was identified in 28 of 120 (23%) CTD-ILD, 45 of 83 (54%) FHP and 27 of 38 (71%) IPF cases and supported a diagnosis of FHP or IPF. The number of lymphoid aggregates/mm2 and fibroblast foci/mm2 was not different in IPF, CTD-ILD or FHP cases with a UIP pattern. Interstitial giant cells supported a diagnosis of FHP or CTD-ILD over IPF, but were infrequent. In the correct clinical/radiological context the pathological findings of NSIP, and particularly NSIP plus OP, favour a diagnosis of CTD-ILD in a cryobiopsy, but CTD-ILD with a UIP pattern, FHP with a UIP pattern and IPF generally cannot be distinguished.

Exploring rheumatoid arthritis associated interstitial lung disease a retrospective study from two Saudi tertiary care centers

BackgroundInterstitial lung disease (ILD) is an increasingly recognized complication of rheumatoid arthritis (RA) and is associated with significant morbidity and mortality. Many risk factors for RA-related ILD were reported. The current study aims to explore the features and risk factors of Saudi patients with RA-ILD.MethodsThis is a multicenter, retrospective, observational study of patients with RA-ILD. Clinical and radiological data from patients with RA-ILD were obtained from electronic medical records, including demographics, clinical characteristics, laboratory tests, pulmonary function tests, ECHO, and HRCT images.ResultOut of 732 patients, 57 had RA-ILD. The mean age at the time of ILD diagnosis was 61.9 (± 12.2) years. RA-ILD diagnosis was significantly less among females (p = 0.008). Patients who ever smoked had significantly more RA-ILD (p < 0.001). Patients with RA-ILD were more likely to present with medical comorbidities, namely diabetes (p < 0.001), hypertension (p < 0.001), ischemic heart disease (p < 0.001), and osteoarthritis (p = 0.030). The multivariate analysis revealed that the age (OR: 1.035, 95% CI: 48.45–52.86, p = 0.0001); gender (OR: 2.581, CI: 1.77–1.86, p = 0.001), DM (OR: 2.498, 95% Cl: 1.65–1.76, P = 0.0001), HTN (OR: 1.975, 95% Cl: 1.61–1.74, P = 0.019), IHD (OR: 6.043, 95% Cl: 1.89–1.93, P = 0.0001) have a significant positive association with RA-ILD. No significant differences were observed between seropositive parameters with or without RA-ILD (p > 0.05). The most common symptoms of RA-ILD were cough (55.6%) and dyspnea (30.2%), and the most common ILD pattern was Non-specific Interstitial Pneumonia (NSIP) (55.6%) followed by Usual Interstitial Pneumonia (UIP) (38.9%). Traction bronchiectasis (75.5%) and glass ground opacities (73.6%) were also observed. The mean FVC and DLCO at baseline were 64.6% and 53.3%, respectively.ConclusionIn this cohort of patients, Saudi RA-ILD patients had a predominant NSIP pattern conversely to what is seen globally. These findings could be explained by the lower rates of smoking in our patient population. Future prospective national studies are needed to confirm the current findings and better evaluate RA-ILD epidemiology and risk factors.

Antifibrotics in the Management of Rheumatoid Arthritis-Associated Interstitial Lung Disease: Prospective Real-World Experience From an Interstitial Lung Disease Clinic in India.

Interstitial lung disease (ILD) in rheumatoid arthritis (RA) is a serious complication with varied prevalence ranging from 4% to as high as 68%, with varied presentation. Immunosuppressants and antifibrotics are used in the management of RA ILD. The clinicodemographic profile and presentation in our country need to be further explored. We assessed the efficacy and safety profile of antifibrotic drugs in combination with immunosuppressants among RA ILD patients. A prospective observational study was conducted in the Interstitial Lung Disease (ILD) Clinic in the Department of Pulmonary Medicine, All India Institute of Medical Sciences Raipur, India, between January 2022 to January 2023. RA patients with dyspnea and chronic cough were referred to us for evaluation of ILD. Patients underwent clinical examination, complete lung function study including spirometry, single breath diffusion capacity for carbon monoxide (DLCO), six-minute walk test, and high-resolution computed tomography of the thorax. Quality of life was assessed using the King's Brief Interstitial Lung Disease (KBILD) questionnaire. Two hundred eighteen RA patients were evaluated and out of these, 43 (20.8%) had features of ILD on high-resolution computed tomogram (HRCT) thorax. Twenty-six (2.18%) met the inclusion criteria for starting antifibrotics. The mean ± SD. age of the patients was 52.96 ± 14.04 and the majority (77%) were females. Fourteen (53.38%) patients had usual interstitial pneumonia (UIP)/probable UIP pattern and 12 (46.22%) had nonspecific interstitial pneumonia (NSIP) patterns on HRCT. Out of 26 patients, 24 (92.3%) were started on antifibrotics. Fourteen (53.8%) patients were on nintedanib and 10 (38.4%) were on pirfenidone. The mean ± SD forced vital capacity (FVC)% predictedwas 62.5 ± 20.04.The mean ± SD. The DLCO percentage predicted was 54.4 ± 22.8. Twenty-two (84.6%) patients did not experience any side effects. The mean ± SD. KBILD score was 59.9 ± 11.17 and was similar in both sexes. In our study, the prevalence of RA ILD was nearly 20.8% and more common in females. Twenty-four (2%) patients were included for antifibrotic treatment. There was an improvement in lung function at the end of six months, but the change was not significant. All patients tolerated antifibrotics well without any serious adverse events.

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 &gt;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.

P029 Successful treatment of Rapidly progressive MDA-5 with dual combination therapy as a bridge to lung transplantation

Abstract Background/Aims Anti-melanoma differentiation-associated gene 5 (MDA5) is known to be associated with rapidly progressive interstitial lung disease (RP-ILD) and is fatal in most cases. The aim of this case report is to highlight the importance of early detection and aggressive treatment in improving the survival of these patients. Methods A 48-year-old Caucasian man, ex-smoker with 20 pack year history with well-controlled asthma, GORD, hiatus hernia and chondrocalcinosis in right knee on hydroxychloroquine was noted to have finger clubbing and decreased air entry at the right lung base on routine follow up. He worked in print finishing for 18 years. Results Investigations: CXR-increased interstitial shadowing. CT chest-extensive fibrosis NSIP pattern with honey combing, traction bronchiectasis throughout both lung fields and ground glass changes at the lower lobes. FVC 2.99 (63% predicted), FEV1 2.82 (73% predicted), FEV1/FVC ratio 0.95%, TLCO 2.75 (25% predicted), KCO 0.70 (46% predicted). CK- 76, Myositis blot- MDA 5 +ve, c ANCA +ve, PR3&amp;MPO -ve and ANA -ve. CRP 70. Bronchoscopy-normal. He received IV methyl prednisolone 1 gm x 3 followed by prednisolone 60 mg daily and tapering dose along with Cyclophosphamide IVI 1 gm x 6 cycles. Long term home oxygen was prescribed. As he did not improve on initial immunosuppressive treatment, Rituximab IVI 1 gm x 2 was administered within two months of initial therapy. He was referred for lung transplant. As maintenance, he was on mycophenolate 1.5 gm BD, prednisolone 10 mg OD, Co-trimoxazole 960 mg x 3 days per week and rituximab 1 gm at six months and 12 months. He had bilateral lung transplant in Cambridge after two years from the diagnosis. Unfortunately, he developed organizing pneumonia, primary graft dysfunction requiring a brief ITU admission and tracheostomy soon after the transplant. His current medications include Prednisolone 5mg, Mycophenolate 2g Tacrolimus 3mg mane, 2mg nocte, Azithromycin 250mg three times weekly and Septrin 480mg od. He is now managing well, cycling 10-15 miles daily. He no longer requires ambulatory oxygen. His most recent FEV1 4.1L, FVC4.9L and resting saturation 97%. Dual combination aggressive immunosuppressive therapy led to significant disease control in this patient, enabling him to be enlisted for and finally to receive a double lung transplant within a reasonable time limit. It has been reported that patients with MDA 5 ILD have a 1-year survival of 67.5% and a 3-year survival of 46.7%. Conclusion Key Learning Points: Patients with MDA5-ILD have significantly high mortality rate but early diagnosis and optimal treatment combination can lead to favourable outcomes. These patients should be monitored for opportunistic infections. Patient commitment is important. Our patient stopped smoking, reduced weight to improve fitness to optimise the transplant success. Disclosure K. Yein: None. S. Sadiq: None. S. Zeb: None. A. Ahmed: None. E. Price: None.

Characteristics of chest high-resolution computed tomography in patients with anti-aminoacyl-tRNA synthetase antibody-positive interstitial lung disease.

Anti-aminoacyl-tRNA synthetase (ARS) antibodies form a condition called Antisynthetase syndrome (ASSD). While interstitial lung disease (ILD) is a particularly frequent manifestation of ASSD and is closely associated with morbidity and mortality, few studies have been conducted on its characteristics on high-resolution computed tomography (HRCT). In this study, we clarified the HRCT findings in patients with anti-ARS antibody-positive ILD (ARS-ILD). Methods: The HRCT findings at the time of the ILD diagnosis in 24 ARS-ILD patients were retrospectively evaluated by 2 pulmonologists and one radiologist. We also assessed the clinical symptoms, physical examination findings, and laboratory data including the type of anti-ARS antibodies. For a further analysis, the data of patients were divided into two groups: the polymyositis (PM)/dermatomyositis (DM) group and the non-PM/DM group. The ratio of men to women was almost 1:1. The median age at the time of the diagnosis was 53 years old. Anti-glycyl (anti-EJ) and anti-histidyl (anti-Jo-1) antibodies were more common than others. An analysis of the HRCT patterns of 23 ARS-ILD patients showed that the most common pattern was the nonspecific interstitial pneumonia (NSIP) pattern. The second most common pattern was the usual interstitial pneumonia (UIP) pattern. Between the PM/DM and non-PM/DM groups, no clear trends were noted in the age, sex ratio, proportion of HRCT patterns, or type of anti-ARS antibodies. This retrospective study demonstrated that ARS-ILD patients, regardless of myositis symptoms, most often showed the NSIP pattern on HRCT, as previously reported. However, unlike previous reports, the UIP pattern on HRCT was not rare.

Clinical features and prognostic analysis of checkpoint inhibitor pneumonitis in patients with non-small cell lung cancer

Objective: To describe the clinical characteristics of patients with non-small cell lung cancer (NSCLC) who developed checkpoint inhibitor pneumonitis (CIP) and to explore potential prognostic factors. Methods: NSCLC patients who were complicated with CIP after immune checkpoint inhibitors (ICIs) therapy in our institute were enrolled in this study from 1 July 2018 to 30 November 2022. Clinical data of NSCLC-CIP patients were collected, including clinical and radiological features and their outcomes. Results: Among the 70 enrolled NSCLC-CIP patients, there were 57 males (81%) and 13 females (19%). The mean age at the diagnosis of CIP was (65.2±6.3) years. There were 46 smokers (66%), 26 patients (37%) with emphysema, 19 patients (27%) with previous interstitial lung disease, and 26 patients (37%) with a history of thoracic radiation. The mean interval from the first application of checkpoint inhibitor to the onset of CIP was (122.7±106.9) days (range: 2-458 days). The main chest CT manifestations were coincided with non-specific interstitial pneumonia (NSIP) pattern and organizing pneumonia (OP) pattern. Most patients had grade 2 (21 cases) or grade 3 (34 cases) CIP. Seventeen patients had been concurrent with other immune-related adverse events such as rash, hepatitis, colitis, and thyroiditis. Half of the enrolled patients (36 patients/51%) had fever, and most patients had elevated C-reactive protein (52 patients/72%) and all patients had elevated erythrocyte sedimentation rate (70 patients/100%). Serum lactate dehydrogenase was elevated in 34 CIP patients. Prednisone≥1 mg·kg-1·d-1 (or equivalent) was the most commonly used initial treatment in CIP patients (50 patients/71.4%). Complications with pulmonary infections (OR=4.44, P=0.03), use of anti-fungal drugs (OR=5.10, P=0.03) or therapeutic dose of sulfamethoxazole (OR=4.86, P=0.04), longer duration of prednisone≥1 mg·kg-1·d-1 (or equivalent) (Z=-2.33, P=0.02) were probable potential risk factors for poor prognosis. Conclusions: Older males with smoking history might be predisposed to develop NSCLC-CIPs after ICIs therapy. NSIP pattern and OP pattern were common chest CT manifestations. Complications with pulmonary infections (especially fungal infection or Pneumocystis jirovecii pneumonia), longer duration, longer duration of high-dose corticosteroids were likely potential risk factors for poor prognosis.

Clinical Characteristics and Outcomes of Interstitial Lung Disease in Primary Sjögren's Syndrome: A Retrospective Cohort Study.

To describe the characteristics of primary Sjögren's syndrome (pSS) patients with interstitial lung disease (ILD) and to assess treatment response. All patients of pSS from 2010 to 2019 were retrospectively identified. Lung function tests, high resolution computed tomography (HRCT) findings, and treatment outcomes were analysed. Out of 550 patients with pSS, ILD was detected in 33 patients (frequency of 6 %). The mean(±SD) age at the diagnosis of pSS was 50 (± 9.3) years. 28/33(84.8%) were females. ILD onset preceded pSS diagnosis in 2 (6%) patients, simultaneously diagnosed in 21 (63.6%) patients and developed after pSS onset in 10 (30.3%) patients. 5 patients (15.15 %) were asymptomatic for ILD. Non-specific interstitial pneumonia (NSIP) accounted for the most frequent ILD subtype, in 15 patients (45.5%). Mycophenolate mofetil (MMF) was the most frequently used steroid sparing agent, in 25 patients (75.7%). 7 patients were lost to follow up. Response was seen in 22 patients, whereas 3 patients were non responders. There was one mortality due to lower respiratory tract infection-related sepsis. Presence of sicca symptoms [91.5% vs 8.7% (p<0.001)], NSIP pattern of ILD [90% vs 10% (p = 0.002)], and absence of Raynaud's phenomenon [91.7% vs 8.3% (p<0.001)] were significantly associated with responder status when compared to non-responders. ILD in primary Sjögren's syndrome is not an uncommon entity, and immunosuppression with steroids along with steroid-sparing agents led to good clinical outcomes of ILD in a majority of the patients in our cohort.

RADIOLOGICAL EVALUATION OF INTERSTITIAL LUNG DISEASES ON HIGH-RESOLUTION COMPUTED TOMOGRAPHY IN TERTIARY CARE CENTER OF NORTH INDIA

Objective: This study assessed the various high-resolution computed tomography (HRCT) patterns of interstitial lung diseases (ILDs) and to differentially diagnose ILD based on their clinical findings and distribution patterns. Methods: The study was conducted in the Department of Radiodiagnosis, Government Medical College and Rajindra Hospital, Patiala, Punjab, India. Forty-three patients with clinical suspicion of ILD were enrolled in the study. HRCT scans of the chest were done in all the cases taken in the study. Results: The mean age (±he age group of 51–60 years. We found slight female preponderance with females accounting for 53.5% and males accounting for 46.5% of the caseload. The most common presenting complaint was dyspnea on exertion (83.7%) followed by cough in 69.8%. The most frequent HRCT finding was septal thickening (90.7%), followed by tractional bronchiectasis (83.7%), fibrotic changes (72.1%), ground-glass opacities (65.1%), and honeycombing (58.1%). Based on HRCT findings, the most common HRCT pattern was the typical usual interstitial pneumonia pattern (58.1%), followed by the non-specific interstitial pneumonia pattern. The final diagnoses were made based on clinic-radiological findings and by the exclusion of other possibilities. The most common ILD reported was idiopathic pulmonary fibrosis (39.5%), followed by connective tissue disorder-ILD (20.9%), and SR-ILD (13.9%). This is followed by idiopathic non-specific interstitial pneumonia (11.6%), hypersensitivity pneumonitis (6.9%), and cryptogenic organizing pneumonia (2.3%). Conclusion: HRCT is a valuable technique for evaluating various ILDs even when chest X-rays are normal. It can differentially diagnose ILDs based on their clinical findings and distribution patterns.

Histopathological significance of connective tissue disease-associated interstitial lung disease in transbronchial lung cryobiopsy specimens

Differentiating between idiopathic interstitial pneumonia (IIP) and secondary interstitial pneumonia, particularly connective tissue disease-associated interstitial lung disease (CTD-ILD), can be challenging histopathologically, and there may be discrepancies among pathologists. While surgical lung biopsy has traditionally been considered the gold standard for diagnosing interstitial pneumonia, the usefulness of transbronchial lung cryobiopsy (TBLC) has been reported. If TBLC could effectively distinguish between primary and secondary diseases, it would provide a less invasive option for patients. The aim of this study was to identify specific pathologic findings in TBLC specimens that could assist in distinguishing CTD-ILD from IIP. A total of 93 underwent TBLC at Tenri Hospital between 2018 and 2022. We retrospectively reviewed cases of CTD-ILD exhibiting a nonspecific interstitial pneumonia (NSIP) pattern (CTD-NSIP) and cases of NSIP with an unknown etiology (NSIP-UE), as determined through multidisciplinary discussion. Nineteen patients with CTD-NSIP and 26 patients with NSIP-UE were included in the study for clinicopathological analysis. The CTD-NSIP group had a significantly higher proportion of female patients compared to the NSIP-UE group (79% vs. 31%; p = 0.002). The presence of both fresh and old intraluminal fibrosis within the same TBLC specimen was significantly more frequent in CTD-NSIP group than in the NSIP-UE group (p = 0.023). The presence of an NSIP pattern with co-existing fresh and old intraluminal fibrosis in TBLC specimens raised suspicion for CTD-ILD.

Anti-Synthetase Syndrome: A Rare and Challenging Diagnosis for Interstitial Lung Disease (ILD): A Case Report

Background: ASA syndrome is a rare and complex immune-mediated entity with heterogeneously diverse presentations diagnosed by the presence of antibodies directed against aminoacyl tRNA synthetase (most common anti-JO-1). The clinical manifestations range from myositis, interstitial lung disease (NSIP PATTERN), non-erosive arthritis, unexplained recurrent fever, Raynaud’s phenomenon, mechanics hands involving mainly skin, respiratory and musculoskeletal systems. Case Report: We report a case of 37-year-old female who had multiple admissions for polyarthritis which was symmetrical, progressive, involving large and small joints associated with morning stiffness, relieved by medication temporarily, with subsequent development of respiratory symptoms like cough, fever off and on, shortness of breath, exertional dyspnea, along with cracks on fingers of hands (mechanics hands). Symptoms worsened gradually with time, affecting her daily routine activities like combing hair, changing clothes, exertional dyspnea on taking few steps, debilitating her quality of life. Extensive and detailed workup was done, Anti-Jo-1 antibodies and Anti-SSA/Ro were positive, CPK level raised, serum aldolase raised, HRCT chest showed interstitial lung disease most likely NSIP, ANA negative, ACE levels normal, Bronchial biopsy showed benign respiratory mucosa, no evidence of malignancy was found. Patient was diagnosed with anti-synthetase antibody syndrome and started on pulse therapy with methylprednisolone. Conclusion: This article explores the clinical manifestations, diagnostic criteria, treatment, progression and prognosis of the disease course.

“Etiology and CT pattern in patients with interstial lung disease”: shift in the trends?

Background: Interstitial lung disease (ILD) also called as diffuse parenchymal lung diseases (DPLD) are “a diverse group of pulmonary infiltrations which result in disruption of the distal lung parenchyma characterized by both inflammation and fibrosis of lung parenchyma and is associated with wide variety of clinical situations.” The understanding of etiology and the underlying CT pattern is helpful in diagnosis, treatment initiation, assessment of response, disease prognosis, and setting up appropriate interventions to improve survival and the quality of life. Methods: A prospective observational analysis of “Radiological and Etiological patterns” in patients presenting with features of the ILD in a tertiary care hospital in South India. Results: The study consisted of 104 patients, with mean age group of 48.03 years, with female 54% of the study population. The commonest high-resolution computed tomography (HRCT) thorax pattern was usual interstitial pneumonia (UIP) (n = 52, 50%). The commonest etiology of the ILD was connective tissue disorders (CTD) (n = 59, 56.73%). Most common CTD was systemic sclerosis (n = 23, 22.11%) and rheumatoid arthritis (n = 20, 19.23%). Second most common etiology was Idiopathic Pulmonary Fibrosis (IPF) (n=15, 14.42%). The UIP pattern was observed in 59.32% patients with CTD-ILD (n = 35) and in 100% patients with the IPF. Conclusion: Connective tissue disorders was the commonest etiology (n = 59, 56.73%), followed by idiopathic etiology (n = 22, 21.15%). Unlike other studies where NSIP pattern was the commonest pattern in CTD-ILD, it was observed in our study that UIP pattern was the most common CT pattern (n = 52, 50%) in connective tissue disorders as well as other etiology of the ILDs.

CT Patterns of Interstitial Lung Disease in Patients with Plaque Psoriasis: A Retrospective Case Series Study.

Background and Objectives: Recently published articles reported an association between psoriasis and interstitial lung diseases (ILDs). The aim of this study is to evaluate the differences in ILD computed tomography (CT) patterns between smoker and never smoker plaque psoriasis (PP) patients under topical treatment without psoriatic arthritis (PA), inflammatory bowel disease (IBD) or connective tissue diseases (CTDs). Matherials and Methods: Two radiologists evaluated chest CT examinations of 65 patients (33 smokers, 32 never smokers) with PP. Results: Usual interstitial pneumonia (UIP) pattern was diagnosed in 36 patients, nonspecific interstitial pneumonia pattern in 19, hypersensitivity pneumonitis in 7 and pleuropulmonary fibroelastosis (PPFE) in 3 patients. UIP pattern showed a statistically significant higher frequency in smoker patients (p = 0.0351). Respiratory symptoms were reported in 80% of patients. Conclusions: ILDs seems to represent a new comorbidity associated with psoriasis. Moreover, a statistically significant association between smokers and UIP pattern in PP patients is found. Respiratory symptoms should be evaluated in PP patients, in collaboration with a radiologist and a pneumologist. However, further studies are required to better understand the epidemiology of ILDs in PP patients.

Abatacept in usual and in non-specific interstitial pneumonia associated with rheumatoid arthritis

ObjectiveTo compare the effectiveness of abatacept (ABA) in Rheumatoid Arthritis-associated Interstitial Lung Disease (RA-ILD) according to the radiological patterns of usual (UIP) or non-specific interstitial pneumonia (NSIP). MethodsFrom an observational longitudinal multicentre study of 263 RA-ILD patients treated with ABA, those with UIP or NSIP were selected. Lung function, chest high resolution computerised tomography (HRCT) and dyspnoea were recorded and compared in both groups from baseline to the end of follow-up (progression definitions: improvement or worsening >10% of FVC or DLCO, changes in HRCT extension and 1-point change in the mMRC scale, respectively). Differences between final and baseline visits were calculated as the average difference (95% CI) through mixed effects models regression. ResultsWe studied 190 patients with UIP (n=106) and NSIP (n=84). General features were similar in both groups except for older age, positive rheumatoid factor, and previous sulfasalazine therapy, which were more frequent in patients with UIP. ILD duration up to ABA initiation was relatively short: median 16 [4-50] and 11 [2-36] months (p=0.36) in UIP and NSIP, respectively. Mean baseline FVC and DLCO were 82% and 63% in UIP and 89% and 65% in NSIP, respectively. Both parameters remained stable during 24 months with ABA. HRCT lesions and dyspnoea improved/stabilized in 73.1% and 90.5% and 72.9% and 94.6% of UIP and NSIP patterns, respectively. ConclusionABA seems equally effective in stabilizing dyspnoea, lung function and radiological impairment in both UIP and NSIP patterns of RA-ILD. Early administration of ABA may prevent RA-ILD progression, regardless of the radiological pattern.

Nailfold capillaroscopy findings of interstitial pneumonia with autoimmune features.

We evaluated nailfold capillaroscopy (NFC) of interstitial pneumonia with autoimmune features (IPAF) and compared it with that of patients with connective tissue disease-interstitial lung disease (CTD-ILD) and idiopathic interstitial pneumonia (IIP). Patients with newly diagnosed as ILD were evaluated using NFC. Baseline demographic, clinical, serological, and high-resolution CT findings were collected. NFC was semi-quantitatively scored with six domains ranging from 0 to 18. In addition, the overall patterns (scleroderma/non-scleroderma patterns) were determined. A total of 81 patients (31 with CTD-ILD, 18 with IPAF, and 32 with IIP) were included. The non-specific interstitial pneumonia pattern was the most common ILD pattern in the CTD-ILD and IPAF groups, whereas the usual interstitial pneumonia pattern was the most common in the IIP group. The semi-quantitative score of the CTD-ILD group was higher than that of the IPAF or IIP groups (5.8 vs 4.2 vs 3.0, p < 0.001, respectively). Giant capillaries and haemorrhages were more frequently present in the CTD-ILD and IPAF groups than in the IIP group. A scleroderma pattern was present in 27.8% of the IPAF group, whereas none of the IIP patients showed a scleroderma pattern. NFC findings may be useful in classifying patients with ILD into CTD-ILD/IPAF/IIP.

Risk factors and prognosis of interstitial lung disease for primary Sjögren syndrome patients: A retrospective case‒control study.

To determine the prevalence, clinical features, risk factors, and prognosis of interstitial lung disease (ILD) in patients with primary Sjogren's syndrome (pSS). Data from 274 pSS patients from August 2013 to August 2022 were reviewed. The clinical features of pSS with ILD were revealed. Logistic regression was used to determine risk factors for ILD in pSS patients. Survival analysis and Cox regression were used to analyse the prognosis and prognostic factors of pSS patients. In pSS patients, the prevalence of ILD was 22.3% (61/274). The pSS patients with ILD were characterized by a late onset and long disease course, with a nonspecific interstitial pneumonia (NSIP) pattern as the predominant high-resolution computed tomography (HRCT) finding. Logistic regression results indicated that an age over 50years old (OR 4.786, 95% CI 1.602-14.299; P = 0.005), purpuric rash (OR 4.695, 95% CI 1.537-14.339; P = 0.007), AMA-M2 antibody positivity (OR 2.582, 95% CI 1.166-5.722; P = 0.019), and diabetes (OR 2.514, 95% CI 1.025-6.167; P = 0.044) were risk factors for ILD in pSS patients. Cox regression results showed that advanced age (HR 1.240, 95% CI 1.088-1.413; P = 0.001) and cancer history (HR 8.411, 95% CI 1.771-39.934; P = 0.007) were risk factors for pSS patient survival. This study showed that pSS patients with ILD tended to have a late onset and long course of pSS. An age over 50years, purpuric rash, AMA-M2 antibody positivity, and diabetes were risk factors for ILD in pSS patients. Advanced age and cancer history were prognostic factors in pSS patients. Key Points • This study showed that pSS patients with ILD tended to have a late-onset and lengthy course of pSS, with the NSIP pattern as the predominant lung image. • The risk factors for ILD in pSS patients determined in this study were an age over 50years, purpuric rash, AMA-M2 antibody positivity, and diabetes. • The prognostic risk factors for pSS patients were advanced age and cancer history.

Nonspecific interstitial pneumonia pattern is a frequent finding in patients with post-acute COVID-19 syndrome treated with bilateral orthotopic lung transplantation: current best evidence

Patients withpost-acute COVID-19 (PA-COVID) syndrome or long COVID-19 syndrome develop persistent symptoms and complications that last beyond 4 weeks of the initial infection. There is limited information regarding the pulmonary pathology in PA-COVID patients who require bilateral orthotopic lung transplantation (BOLT). Our experience with 40 lung explants from 20 PA-COVID patients who underwent BOLT is described. Clinicopathologic findings are correlated with best evidence from literature. The lung parenchyma showed bronchiectasis (n=20) and severe interstitial fibrosis with areas resembling the nonspecific interstitial pneumonia (NSIP) pattern of fibrosis (n=20), interstitial fibrosis not otherwise specified (n=20), and fibrotic cysts (n=9). None of the explants exhibited a usual interstitial pneumoniapattern of fibrosis. Other parenchymal changes included multinucleated giant cells (n=17), hemosiderosis (n=16), peribronchiolar metaplasia (n=19), obliterative bronchiolitis (n=6), and microscopic honeycombing (n=5). Vascular abnormalities included thrombosis of a lobar artery (n=1) and microscopic thrombi in small vessels (n=7). Systematic literature review identified 7 articles reporting the presence in 12 patients of interstitial fibrosis showing the NSIP pattern (n=3), organizing pneumonia/diffuse alveolar damage(n=4) and not otherwise specified (n=3) patterns. All but one of these studies also reported the presence of multinucleated giant cells and none of the studies reported the presence of severe vascular abnormalities. PA-COVID patients undergoing BOLT show a pattern of fibrosis that resembles a mixed cellular-fibrotic NSIP pattern and generally lack severe vascular complications. As the NSIP pattern of fibrosis is often associated with autoimmune diseases, additional studies are needed to understand the mechanism of disease and learn whether this information can be used for therapeutic purposes.