scholarly journals Clinical features and outcome of acute exacerbation of interstitial pneumonia associated with connective tissue disease.

2016 ◽  
Vol 63 (3.4) ◽  
pp. 294-299 ◽  
Author(s):  
Yuko Toyoda ◽  
Masaki Hanibuchi ◽  
Jun Kishi ◽  
Hiroshi Kawano ◽  
Shun Morizumi ◽  
...  
Keyword(s):  
Connective Tissue ◽  
Acute Exacerbation ◽  
Interstitial Pneumonia ◽  
Connective Tissue Disease ◽  
Clinical Features

scholarly journals Differences in clinical features of acute exacerbation between connective tissue disease-associated interstitial pneumonia and idiopathic pulmonary fibrosis

2018 ◽  
Vol 16 ◽  
pp. 147997231880947 ◽  
Author(s):  
Noriyuki Enomoto ◽  
Yoshiyuki Oyama ◽  
Yasunori Enomoto ◽  
Hideki Yasui ◽  
Masato Karayama ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Connective Tissue ◽  
Pulmonary Fibrosis ◽  
Acute Exacerbation ◽  
Interstitial Pneumonia ◽  
Connective Tissue Disease ◽  
Clinical Features ◽  
Early Stage ◽  
Inflammatory Status ◽  
Cell Counts

Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is a devastating condition that frequently occurs in the advanced stage of IPF. However, the clinical features in AE of connective tissue disease-associated interstitial pneumonia (AE-CTD-IP) have not been well-established. The aim of this study was to clarify the clinical features of AE-CTD-IP and to compare them with those of AE-IPF. Fifteen AE-CTD-IP patients and 48 AE-IPF patients who were diagnosed and treated at our hospital were retrospectively studied. Compared with AE-IPF patients, AE-CTD-IP patients had a significantly higher %FVC (median, 94.8 vs. 56.3%; p < 0.001) and a lower extent of honeycombing on HRCT ( p = 0.020) within 1 year before AE. At AE, AE-CTD-IP patients showed higher white blood cell counts (12.0 vs. 9.9 × 103/μL; p = 0.023), higher CRP (10.2 vs. 6.7 mg/dL; p = 0.027), and longer period from admission to the beginning of AE treatment (4 vs. 1 days; p = 0.003) than AE-IPF patients. In addition, patients with AE-CTD-IP had poor prognosis as in those with AE-IPF (log-rank; p = 0.171). In conclusion, AE-CTD-IP occurred even in the early stage of IP and had more inflammatory status than in AE-IPF.

scholarly journals Recognition of Connective Tissue Disease-Related Interstitial Pneumonia Based on Histological Score—A Validation Study of an Online Diagnostic Decision Support Tool

Diagnostics ◽  
2021 ◽  
Vol 11 (8) ◽  
pp. 1359
Author(s):  
Mutsumi Ozasa ◽  
Yoshiaki Zaizen ◽  
Kazuhiro Tabata ◽  
Kensuke Kataoka ◽  
Shuntaro Sato ◽  
...  
Keyword(s):  
Connective Tissue ◽  
Interstitial Pneumonia ◽  
Connective Tissue Disease ◽  
Clinical Features ◽  
Decision Support Tool ◽  
Smoking History ◽  
Support Tool ◽  
Histological Score ◽  
Diagnostic Decision ◽  
Thoracoscopic Biopsy

Objectives: to evaluate the number of cases of idiopathic pulmonary fibrosis (IPF) that included histological features of connective tissue disease (CTD) and to check whether they demonstrated the clinical features of CTD, using a previously reported CTD-interstitial pneumonia (IP) index that histologically differentiates CTD-associated and idiopathic IP. Methods: patients diagnosed with IPF following video-assisted thoracoscopic biopsy through multidisciplinary team diagnosis between 2014 and 2017 were selected. Pathological observation was made by four pathologists who scored eight observational items needed for the CTD-IP index. Cases determined as CTD, by the CTD-IP index, were extracted, and their clinical features were compared. Results: a total of 94 cases of IPF were identified, of which 20 were classified into the CTD group using the CTD-IP index with reasonable interobserver agreement (k = 0.76). Cases pathologically classified into the CTD group were significantly associated with female sex, non-smoking history, autoantibody positivity, and CTD symptoms (p = 0.01, 0.03, 0.01, and 0.04, respectively). Conclusions: patients with IPF with pathological findings of CTD showed clinical characteristics similar to those of patients with CTD.

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.

Overlap/undifferentiated syndromes

2013 ◽  
pp. 1069-1078 ◽  
Author(s):  
Ariane Herrick
Keyword(s):  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Clinical Features ◽  
Lupus Erythematosus ◽  
Raynaud’S Phenomenon ◽  
Clinical Manifestations ◽  
Raynaud's Phenomenon ◽  
Overlap Syndromes ◽  
Clear Cut ◽  
Positive Treatment

Undifferentiated connective tissue disease (UCTD) and overlap syndromes both form part of the broad spectrum of connective tissue disease. They are difficult to define, as the boundaries between them and specific diseases such as systemic lupus erythematosus (SLE), systemic sclerosis (SSc), and myositis are often not clear-cut. This chapter gives a broad overview of diagnosis, clinical features, outcomes, and management. Patients with UCTD have clinical and/or serological features of connective tissue disease but do not fulfil the criteria for any one defined disease. Raynaud’s phenomenon and puffy fingers are often the presenting features but there are many possible others, including arthralgia, sicca symptoms, and breathlessness due to pulmonary fibrosis, usually in the context of a positive anti-nuclear antibody (ANA). A proportion of patients evolve into a defined connective tissue disease: in those who do, this is generally within 5 years of onset. Treatment is dependent upon the clinical features: for examplem vasodilators for Raynaud’s phenomenon, or hydroxychloroquine for arthralgia/arthritis. Patients with overlap syndromes have features of more than one defined connective tissue disease. Overlap syndromes are therefore highly heterogeneous as many combinations of clinical and serological features can occur. Mixed connective tissue disease (MCTD) is the overlap syndrome that has been most described and includes overlapping features of SLE, SSc, and myositis in patients who are anti-U1 ribonucleoprotein (RNP) antibody positive. Treatment is of the specific clinical manifestations. Patients with overlap syndromes should be kept under regular review to allow early identification of internal organ involvement.

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