HRCT Findings and Clinical Features in Non-specific and Usual Interstitial Pneumonia with Connective Tissue Diseases

2007 ◽  
Vol 14 (3) ◽  
pp. 208
Author(s):  
Joong Kyong Ahn ◽  
Eun-Mi Koh ◽  
You Sun Lee ◽  
Hoon-Suk Cha ◽  
Man Pyo Chung ◽  
...  
Keyword(s):  
Connective Tissue ◽  
Interstitial Pneumonia ◽  
Clinical Features ◽  
Usual Interstitial Pneumonia ◽  
Connective Tissue Diseases

scholarly journals Morphologic Aspects of Interstitial Pneumonia With Autoimmune Features

2018 ◽  
Vol 142 (9) ◽  
pp. 1080-1089 ◽  
Author(s):  
Ellen Caroline Toledo do Nascimento ◽  
Bruno Guedes Baldi ◽  
Marcio Valente Yamada Sawamura ◽  
Marisa Dolhnikoff
Keyword(s):  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Connective Tissue Disease ◽  
Usual Interstitial Pneumonia ◽  
Connective Tissue Diseases ◽  
Distinct Subgroup ◽  
Management Protocol ◽  
The Impact

Context.— Interstitial lung disease, a common complication observed in several connective tissue diseases, causes significant morbidity and mortality. Similar to individuals with connective tissue diseases, a significant subgroup of patients with clinical and serologic characteristics suggestive of autoimmunity but without confirmed specific connective tissue disease presents with associated interstitial lung disease. These patients have been classified using different controversial nomenclatures, such as undifferentiated connective tissue disease–associated interstitial lung disease, lung-dominant connective tissue disease, and autoimmune featured interstitial lung disease. The need for a better understanding and standardization of this entity, interstitial lung disease with autoimmune features, and the need for an adequate management protocol for patients resulted in the introduction of a new terminology in 2015: interstitial pneumonia with autoimmune features. This new classification requires a better comprehension of its diagnostic impact and the influence of its morphologic aspects on the prognosis of patients. Objective.— To review the diagnostic criteria for interstitial pneumonia with autoimmune features, with an emphasis on morphologic aspects. Data Sources.— The review is based on the available literature, and on pathologic, radiologic, and clinical experience. Conclusions.— The interstitial pneumonia with autoimmune features classification seems to identify a distinct subgroup of patients with different prognoses. Studies show that nonspecific interstitial pneumonia and usual interstitial pneumonia are the most prevalent morphologic patterns and show discrepant results on the impact of the usual interstitial pneumonia pattern on survival. Prospective investigations are necessary to better define this subgroup and to determine the prognosis and appropriate clinical management of these patients.

scholarly journals Clinico-radiological profile of connective tissue disease related-interstitial lung diseases from a tertiary care centre of India: a cross sectional study

2021 ◽  
Author(s):  
Mehul Agarwal ◽  
Manohar Lal Gupta ◽  
Kunal Deokar ◽  
Benhur Joel Shadrach ◽  
Neha Bharti ◽  
...  
Keyword(s):  
Connective Tissue ◽  
Interstitial Pneumonia ◽  
Lung Diseases ◽  
Tertiary Care ◽  
Usual Interstitial Pneumonia ◽  
Connective Tissue Diseases ◽  
Clinical Manifestations ◽  
Interstitial Lung Diseases ◽  
Western India ◽  
Tertiary Care Centre

Interstitial lung diseases (ILDs) are a frequently occurring pulmonary manifestation in patients of connective tissue diseases (CTD). Detailed understanding of this subset of lung diseases is vital, hence the study was conducted to analyze the clinico-radiological characteristics of CTD-ILD. The present study was conducted between March 2017 to February 2018 at a tertiary care teaching hospital from western India. A total of 100 patients having respiratory symptoms suggestive of ILD, who were either diagnosed cases of CTD or had clinical manifestations of underlying CTD, were included in the study.  27% of patients belonged to age group 41-50 years and 78% were females. Chief respiratory complaints were dyspnea and cough. Clubbing was present in 29% patients. The most common CTDs were rheumatoid arthritis [RA] (26%), systemic sclerosis [SSC] (21%), mixed connective tissue disorder [MCTD] (19%) and Sjogren’s syndrome [SS] (16%). Restrictive defect on spirometry was seen in 58% cases and was most significant among patients with RA (65%) and SS (62%). Pulmonary arterial hypertension was seen in 40% cases and was most evident in MCTD (78%) and SSC patients (58%). Chest radiograph was normal in 47% of subjects. Most common radiological pattern on high resolution computed tomography (HRCT) thorax was non-specific interstitial pneumonia (42%) followed by usual interstitial pneumonia (21%). ILD is a common manifestation of CTD. Work-up for an underlying CTD should be offered to all ILD patients and vice versa.

scholarly journals AB0605 CLINICAL PROFILE AND CHEST HIGH-RESOLUTION COMPUTED TOMOGRAPHY (HRCT) FINDINGS IN PATIENTS WITH CONNECTIVE TISSUE DISEASES AND INTERSTITIAL LUNG DISEASE: EXPERIENCE OF A SINGLE REFERENCE RHEUMATOLOGY CENTER

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1598.2-1599
Author(s):  
I. Rusu ◽  
L. Muntean ◽  
M. M. Tamas ◽  
I. Felea ◽  
L. Damian ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Systemic Sclerosis ◽  
High Resolution ◽  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Connective Tissue Diseases ◽  
High Resolution Computed Tomography ◽  
Hrct Patterns

Background:Interstitial lung disease (ILD) is a common manifestation of connective tissue diseases (CTDs), and is associated with significant morbidity and mortality. Chest high-resolution computed tomography (HRCT) play an important role in the diagnosis of ILD and may provide prognostic information.Objectives:We aimed to characterize the clinical profile and chest HRCT abnormalities and patterns of patients diagnosed with CTDs and ILD.Methods:In this retrospective, observational study we included 80 consecutive patients with CTDs and ILD referred to a tertiary rheumatology center between 2015 and 2019. From hospital charts we collected clinical data, immunologic profile, chest HRCT findings. HRCT patterns were defined according to new international recommendations.Results:Out of 80 patients, 64 (80%) were women, with a mean age of 55 years old. The most common CTD associated with ILD was systemic sclerosis (38.8%), followed by polymyositis (22.5%) and rheumatoid arthritis (18.8%). The majority of patients had dyspnea on exertion (71.3%), bibasilar inspiratory crackles were present in 56.3% patients and 10% had clubbing fingers. Antinuclear antibodies (ANA) were present in 78.8% patients, and the most frequently detected autoantibodies against extractable nuclear antigen were anti-Scl 70 (28.8%), followed by anti-SSA (anti-Ro, 17.5%), anti-Ro52 (11.3%) and anti-Jo (7.5%). Intravenous cyclophosphamide therapy for 6-12 months was used in 35% of patients, while 5% of patients were treated with mycophenolate mofetil.The most frequent HRCT abnormalities were reticular abnormalities and ground glass opacity. Non-specific interstitial pneumonia (NSIP) was identified in 46.3% CTDs patients. A pattern suggestive of usual interstitial pneumonia (UIP) was present in 32.5% patients, mainly in patients with systemic sclerosis. In 21.3% patients the HRCT showed reticulo-nodular pattern, micronodules and other abnormalities, not diagnostic for UIP or NSIP pattern.Conclusion:Nonspecific interstitial pneumonia (NSIP) is the most common HRCT pattern associated with CTDs. Further prospective longitudinal studies are needed in order to determine the clinical and prognostic significance of various HRCT patterns encountered in CTD-associated ILD and for better patient management.References:[1]Ohno Y, Koyama H, Yoshikaua T, Seki S. State-of-the-Art Imaging of the Lung for Connective Tissue Disease (CTD). Curr Rheumatol Rep. 2015;17(12):69.[2]Walsh SLF, Devaraj A, Enghelmeyer JI, Kishi K, Silva RS, Patel N, et al. Role of imaging in progressive-fibrosing interstitial lung diseases. Eur Respir Rev. 2018;27(150)Disclosure of Interests:None declared

Effect of Cotreatment with Corticosteroids in Connective Tissue Related Lung Disease (CTD-ILD) Patients on Mycophenolate Mofetil (MMF)

2021 ◽  
Author(s):  
Roberto Caricchio ◽  
Erin R Narewski ◽  
Ryan Townsend ◽  
Stephen Codella ◽  
Jin Sun Kim ◽  
...  
Keyword(s):  
Mycophenolate Mofetil ◽  
Connective Tissue ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Connective Tissue Disease ◽  
Pulmonary Function Tests ◽  
Usual Interstitial Pneumonia ◽  
University Hospital ◽  
Inflammatory Myositis ◽  
Idiopathic Inflammatory Myositis

Abstract Introduction: Connective Tissue Disease Related Interstitial Lung Disease (CTD-ILD) is often treated with immunosuppressant medications; common among these is Mycophenolate Mofetil (MMF). We hypothesized that co-treatment with corticosteroids would impact disease progression.Methods: We examined a consecutive cohort of CTD-ILD patients followed at Temple University Hospital in Philadelphia, PA since 2015 who had pulmonary function tests (PFTs) performed by American Thoracic Society (ATS)/European Respiratory Society (ERS) Criteria at least one year apart. All patients were treated for CTD-ILD with MMF used either as sole therapy or as combination therapy with prednisone. Univariate logistic analyses were performed revealing the odds ratio (OR) for improvement or worsening of several PFT values (including forced vital capacity (FVC), diffusion capacity of carbon monoxide (DLCO), and six-minute walk (6MW)) greater than the minimal clinically important difference (MCID) for each value.Results: We included 103 patients (74 women) with an average age of 60 ± 11 years, 49% of our cohort were current or former smokers, and mean BMI was 29 ± 7 kg/m2. Patients were observed on treatment for an average of 23 months. CTD distribution included 25% mixed connective tissue disease (MCTD), 24% systemic sclerosis (SSc), 17% rheumatoid arthritis (RA), 14% systemic lupus erythematosus (SLE), 10% other idiopathic inflammatory myositis (IIM) syndromes, 7% Antisynthetase Syndrome, 5% Sjӧgren’s syndrome. Non-specific interstitial pneumonia (NSIP) was the majority (45%) ILD pattern noted, Usual Interstitial Pneumonia (UIP) 35%, and other types were less prevalent (20%). The majority of patients received corticosteroids as co-treatment with MMF (75 patients (72%)) with a mean daily dose of 15 ± 16 mg of prednisone. Mean daily MMF dose was 1144 ± 675 mg. Glucocorticoid treatment was not associated with significant improvements in PFT values, including FVC, DLCO, and 6MW distance walked.Conclusion: In this small cohort, patients with CTD-ILD receiving MMF did not demonstrate improved lung function when receiving co-treatment with corticosteroids, but larger prospective studies are needed to better elucidate the effect of corticosteroids on this vulnerable group of patients.

Lymphocytic infiltrations of the lung

2020 ◽  
pp. 4241-4244
Author(s):  
S. J. Bourke
Keyword(s):  
Connective Tissue ◽  
B Cell ◽  
Interstitial Pneumonia ◽  
Lymphoid Tissue ◽  
Poor Prognosis ◽  
Cell Lymphoma ◽  
Connective Tissue Diseases ◽  
B Cell Lymphoma ◽  
Low Grade ◽  
High Grade

Lymphocytic infiltrations of the lung arise from the proliferation of bronchus-associated lymphoid tissue, resulting in a spectrum of rare conditions ranging from benign polyclonal lymphoid interstitial pneumonia to monoclonal primary malignant lymphomas of the lung. Lymphoid interstitial pneumonia is most commonly seen in Sjögren’s syndrome or other connective tissue diseases, and in association with HIV infection, and is characterized by reticulonodular shadowing on CT imaging and (usually) a good response to corticosteroids. Primary pulmonary lymphomas fall into three categories: lymphomatoid granulomatosis, low-grade B-cell lymphoma, and high-grade B-cell lymphoma. The latter require treatment with cytotoxic drugs and have a poor prognosis.

scholarly journals Interstitial pneumonia with autoimmune features and platypnea-orthopnea syndrome

2019 ◽  
Vol 12 (9) ◽  
pp. e230948
Author(s):  
Uthara Mathew ◽  
Ankit Mittal ◽  
Surabhi Vyas ◽  
Animesh Ray
Keyword(s):  
Systematic Review ◽  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Interstitial Pneumonia ◽  
Immunosuppressive Treatment ◽  
Usual Interstitial Pneumonia ◽  
Lower Lobe ◽  
Autoimmune Disorder ◽  
Shortness Of Breath ◽  
Review Of Literature

Interstitial pneumonia with autoimmune features (IPAF) is a recently proposed terminology for interstitial lung disease (ILD) with evidence of autoimmunity that does not meet the criteria for a defined connective tissue disease (CTD). Although ILD is well recognised in patients with established CTD, it is rarely the sole presenting feature of CTD. We report a case of 22-year-old male patient, who presented with progressive shortness of breath for 2 months and had features suggestive of platypnea-orthodeoxia syndrome (POS). Imaging revealed ILD with usual interstitial pneumonia pattern. Patient had features of autoimmune disorder but did not fulfil the criteria for any CTD and hence was labelled as IPAF. His POS was attributed predominantly to the lower lobe disease. The patient responded well to immunosuppressive treatment. A systematic review of literature of all cases with POS due to pulmonary parenchymal involvement has also been done.

An analysis of the clinical features of lung cancer in patients with connective tissue diseases

2017 ◽  
Vol 55 (2) ◽  
pp. 153-160 ◽  
Author(s):  
Atsuro Saijo ◽  
Masaki Hanibuchi ◽  
Hisatsugu Goto ◽  
Yuko Toyoda ◽  
Toshifumi Tezuka ◽  
...  
Keyword(s):  
Lung Cancer ◽  
Connective Tissue ◽  
Clinical Features ◽  
Connective Tissue Diseases
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