SAT0508 A New Method of Quantitative Analysis of Thoracic CT Images to Investigate Connective Tissue Disease (CTD)-Associated Interstitial Pneumonia

2013 ◽  
Vol 72 (Suppl 3) ◽  
pp. A754.1-A754
Author(s):  
N. Tosaka ◽  
J. Nishino ◽  
K. Kato ◽  
S. Fukaya ◽  
S. Yoshida
Keyword(s):  
Quantitative Analysis ◽  
Connective Tissue ◽  
Interstitial Pneumonia ◽  
Connective Tissue Disease ◽  
Ct Images ◽  
New Method ◽  
Thoracic Ct

scholarly journals Case Report: Successful Treatment of Refractory Interstitial Lung Disease With Cyclosporine A and Pirfenidone in a Child With SLE

2021 ◽  
Vol 12 ◽  
Author(s):  
Linxia Deng ◽  
Yaxian Chen ◽  
Xiufen Hu ◽  
Jianhua Zhou ◽  
Yu Zhang
Keyword(s):  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Young Children ◽  
Combination Therapy ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Connective Tissue Disease ◽  
Cyclosporine A ◽  
Ct Images ◽  
Work Up

Interstitial lung disease (ILD) as an initial manifestation of lupus is rare, especially in young children. Here, we report a case of a 3-year-old boy who presented with fever, shortness of breath, and facial erythema. Clinical examination suggested a diagnosis of active systemic lupus erythematosus (SLE) with butterfly rash, anemia, positive antinuclear antibody, positive anti-double-stranded DNA, and hypocomplementemia. On retrospective review of the patient’s records, multiple chest computed tomography (CT) images showed non-specific interstitial pneumonia + organizing pneumonia pattern, with no further autoimmune work-up during the visit to a respiratory department. In our opinion, persistent interstitial pneumonia may be a clue to connective tissue disease. The patient received steroid treatment for 1 year, and the radiological and immunological resolution was noted. However, he still suffered from cough and dyspnea. After a 1-year follow-up, he was hospitalized again for SLE relapse. While continuing corticosteroid therapy, the patient was given combination therapy consisting of cyclosporine A (CsA) and monthly-pulse cyclophosphamide for 6 months, and decreased proteinuria was noted. However, the patient’s respiratory symptoms and pulmonary radiologic findings did not improve significantly. With continued steroid therapy, the patient was started on a daily regimen of CsA and pirfenidone. Both drugs were sufficiently effective to allow gradual reduction of steroid dosage. After 2 years of treatment, marked improvements in symptoms, pulmonary function and chest CT images were observed. Our experience with this case emphasizes that prompt work-up for connective tissue disease (CTD) should be considered in young children with ILD, and pirfenidone might be a useful add-on therapy with immunosuppressive agents for refractory CTD-ILD in pediatric patients. Nevertheless, further clinical trials including larger numbers of patients need to assess the efficiency and safety of this combination therapy for refractory CTD-ILD.

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.

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.

scholarly journals Deep-inspiration breath-hold18F-FDG-PET/CT is useful for assessment of connective tissue disease associated interstitial pneumonia

2015 ◽  
Vol 26 (1) ◽  
pp. 121-127 ◽  
Author(s):  
Takeaki Uehara ◽  
Mitsuhiro Takeno ◽  
Maasa Hama ◽  
Ryusuke Yoshimi ◽  
Akiko Suda ◽  
...  
Keyword(s):  
Connective Tissue ◽  
Interstitial Pneumonia ◽  
Connective Tissue Disease ◽  
Fdg Pet ◽  
Deep Inspiration ◽  
Pet Ct ◽  
Fdg Pet Ct

scholarly journals Significance of connective tissue disease features in idiopathic interstitial pneumonia

2011 ◽  
Vol 39 (3) ◽  
pp. 661-668 ◽  
Author(s):  
T. J. Corte ◽  
S. J. Copley ◽  
S. R. Desai ◽  
C. J. Zappala ◽  
D. M. Hansell ◽  
...  
Keyword(s):  
Connective Tissue ◽  
Interstitial Pneumonia ◽  
Connective Tissue Disease ◽  
Idiopathic Interstitial Pneumonia

scholarly journals Centrilobular Fibrosis in Fibrotic (Chronic) Hypersensitivity Pneumonitis, Usual Interstitial Pneumonia, and Connective Tissue Disease–Associated Interstitial Lung Disease

2020 ◽  
Vol 144 (12) ◽  
pp. 1509-1516
Author(s):  
Andrew Churg
Keyword(s):  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Connective Tissue Disease ◽  
Hypersensitivity Pneumonitis ◽  
Interstitial Fibrosis ◽  
Usual Interstitial Pneumonia ◽  
Diffuse Fibrosis ◽  
Chronic Hypersensitivity Pneumonitis

Context.— Various pulmonary diseases can produce centrilobular (peribronchiolar) fibrosis, which may be isolated or associated with other patterns of more diffuse fibrosis. The major forms of interstitial lung disease in which centrilobular fibrosis is found are fibrotic (chronic) hypersensitivity pneumonitis, connective tissue disease–associated interstitial lung disease, and (a disputed issue) usual interstitial pneumonia/idiopathic interstitial fibrosis. Objective.— To review recent literature that addresses separation of these entities. Data Sources.— Data comprised recent publications. Conclusions.— In a specially constructed multidisciplinary discussion exercise, it was found that peribronchiolar metaplasia affecting more than half the bronchioles or more than 2 foci of peribronchiolar metaplasia per square centimeter of biopsy area was strongly associated with a confident diagnosis of fibrotic hypersensitivity pneumonitis. Giant cells or granulomas were only found in cases with a greater than 50% diagnostic confidence in hypersensitivity pneumonitis. Conversely, greater numbers of fibroblast foci per square centimeter and increasing measured amounts of subpleural fibrosis favored a diagnosis of usual interstitial pneumonia. Recent data also suggest that centrilobular fibrosis can be found in usual interstitial pneumonia, although the presence of centrilobular fibrosis statistically favors an alternate diagnosis. Connective tissue disease is a major confounder because many patterns are very similar to fibrotic hypersensitivity pneumonitis or usual interstitial pneumonia. Genetic abnormalities, such as the MUC5B minor allele overlap, in these conditions and at this point cannot be used for discrimination. Thus, the separation of fibrotic hypersensitivity pneumonitis and usual interstitial pneumonia remains a difficult problem. Accurate biopsy diagnosis of all of these diseases requires correlation with imaging and clinical findings, and is crucial for treatment.

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