scholarly journals Connective Tissue Disease, Interstitial Lung Disease, and Pulmonary Hypertension (CTD PH-ILD): A Distinct Entity and Potential Opportunity

2021 ◽  
Vol 20 (4) ◽  
pp. 109-118
Author(s):  
Denise Gabrielle A. Sese ◽  
Kristin B. Highland
Keyword(s):  
Pulmonary Hypertension ◽  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Connective Tissue Disease ◽  
Treatment Options ◽  
Connective Tissue Diseases ◽  
Multisystem Disorder ◽  
Disease Pathogenesis ◽  
Increased Risk

Connective tissue diseases are a multisystem disorder that can cause impairments in quality of life, shorten life expectancy, and increase the risk of mortality at a younger age. These patients have an increased risk for the development of pulmonary hypertension through several mechanisms including pulmonary arterial hypertension and pulmonary hypertension associated with interstitial lung disease. This review aims to discuss the various presentations of connective tissue disease associated with pulmonary hypertension associated with interstitial lung disease, demographics, and survival. It gives an overview of accepted mechanisms of disease pathogenesis, discusses advances in diagnostics, and treatment options. Despite a deeper understanding of disease pathogenesis, treatment for this remains limited to prevention of disease progression. The identification of the primary disease driver requires careful evaluation of the disease phenotype and is a potential target for treatment and prevention of death.

scholarly journals Treatment of Pulmonary Hypertension in Patients with Connective Tissue Disease and Interstitial Lung Disease

2010 ◽  
Vol 17 (6) ◽  
pp. 282-286 ◽  
Author(s):  
Shikha Mittoo ◽  
Thomas Jacob ◽  
Andrea Craig ◽  
Zoheir Bshouty
Keyword(s):  
Pulmonary Hypertension ◽  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Connective Tissue Disease ◽  
Term Treatment ◽  
Kaplan Meier ◽  
Long Term Treatment ◽  
Survival Differences

BACKGROUND: Pulmonary hypertension (PH) in patients with connective tissue disease (CTD) can occur in isolation or concomitantly with interstitial lung disease (ILD). Targeted therapies for PH can mitigate clinical deterioration in CTD patients with isolated PH; however, the effect of these therapies in CTD patients with PH and ILD (CTD-PH-ILD) are poorly characterized.OBJECTIVE: To investigate outcomes following long-term treatment of PH in patients with CTD-PH-ILD.METHODS: A retrospective evaluation of 13 CTD-PH-ILD patients who were treated with bosentan, sildenafil or bosentan plus sildenafil, was conducted. Immunosuppressants were prescribed as indicated. Patients underwent pulmonary function testing and assessment of 6 min walk distance at the time of treatment initiation and during follow-up. Patients were followed until time of death, lung transplantation or the end of the study. Kaplan-Meier estimates of survival were calculated and log-rank testing was used to analyze survival differences according to CTD subtype.RESULTS: Thirteen patients (seven with systemic sclerosis [SSc], four with overlap syndrome, and two with rheumatoid arthritis) were followed for a mean (± SD) duration of 33.8±21.7 months. The survival estimate at a median duration of 34 months was 85%; two patients with SSc died. Mortality rates were greater among patients with SSc versus other CTD subtypes (P=0.04). No changes from baseline to follow-up in mean forced vital capacity or exercise capacity, and no treatment-related toxicity, were observed.CONCLUSION: Treatment using PH-specific therapies in patients with CTD, PH and ILD was well tolerated. Further studies to investigate the efficacy of PH-specific therapies in CTD-PH-ILD patients are warranted.

scholarly journals AB1136 THE DIAGNOSTIC VALUE OF SERUM KL-6 IN CONNECTIVE TISSUE DISEASE ASSOCIATED INTERSTITIAL LUNG DISEASE IN THE UYGUR POPULATION OF CHINA.

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1858.2-1858
Author(s):  
X. Wu ◽  
L. Wu ◽  
C. N. Luo ◽  
Y. M. Shi
Keyword(s):  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Connective Tissue Disease ◽  
Characteristic Curve ◽  
Connective Tissue Diseases ◽  
Nonsmall Cell Lung Cancer ◽  
Pulmonary Complications ◽  
Cross Sectional ◽  
Uygur Population

Background:Connective tissue diseases are a group of inflammatory, immune mediated disorders.Interstitial lung disease (ILD) is associated with significant morbidity and mortality.Currently,, scientists are still looking for serum markers to diagnose interstitial lung disease.Althought serum KL-6 level has been studied in ILD of various aetiologies and revealed to be an important serum marker for ILD,but differences in KL-6 expression related to ethnic and/or genetic variants may exist.Objectives:To evaluate the diagnosis of the serum Krebs von den Lungen-6 (KL-6) for CTD-ILD in the Uygur population of China.Methods:117 Patients with CTD-ILD (CTD-ILD group) and 182 patients with CTD (CTD group) who visited the department of rheumatology and immunology of People’s Hospital of Xinjiang Uygur Autonomous Region between January, 2015 and December, 2019 were included. Serum KL-6 levels were measured by chemiluminescent enzyme immunoassay kit.Results:The significantly higher levels of KL-6 were determined in the RA-ILD group than RA group [569(287.5,984)U/ml vs 194(152,266.5)U/ml](P<0.001)(figure 1).The optimal cutoff value of serum KL-6 for diagnosis of RA-ILD was 345.5 U/ml, and the sensitivity and specificity were71.8% and 90.1 %, respectively. Area Under the Curve (AUC) was 0.875.(figure 2)Figure 1.Comparison of serum KL-6 concentrations in CTD-ILD group and CTD group.Fig 2.Receiver-operating characteristic curve(ROC) of KL-6 for the diagnosis of CTD-ILDConclusion:The serum KL-6 is a important biomarker for the diagnosis of CTD-ILD and Serum KL-6 could be a clinically useful biomarker in screening CTD-ILD in the Uygur population of China.References:[1]Woodhead F, Wells A U, Desai S R. Pulmonary Complications of Connective Tissue Diseases[J]. Clinics in Chest Medicine, 2008, 29(29):149–164.Tanaka S, Hattori N, Ishikawa N, et al. Krebs von den Lungen-6 (KL-6) is a progn -ostic biomarker in patients with surgically resected nonsmall cell lung cancer. Int J Cancer 2012; 130:377–87.[2]Ogz E O, Kucuksahin O, Turgay M, et al. Association of serum KL-6 levels with interstitial lung disease in patients with connective tissue disease: a cross-sectional study. Clinical Rheumatology, 2016, 35(3):663-666.Disclosure of Interests:None declared

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 Treatment Options for Interstitial Lung disease associated with Connective Tissue Disease

2021 ◽  
Vol 5 (2) ◽  
pp. 12-18
Author(s):  
Moiz Ehtesham ◽  
Fatima Mahmood ◽  
Muhammad Asim Shabbir ◽  
Ruben-Wende Peredo
Keyword(s):  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Connective Tissue Disease ◽  
Treatment Options

scholarly journals Therapeutic Options for the Treatment of Interstitial Lung Disease Related to Connective Tissue Diseases. A Narrative Review

2020 ◽  
Vol 9 (2) ◽  
pp. 407 ◽  
Author(s):  
Caterina Vacchi ◽  
Marco Sebastiani ◽  
Giulia Cassone ◽  
Stefania Cerri ◽  
Giovanni Della Casa ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Connective Tissue Disease ◽  
Lupus Erythematosus ◽  
Therapeutic Option ◽  
Inflammatory Myopathy ◽  
Connective Tissue Diseases ◽  
Immunosuppressive Drugs ◽  
Pulmonary Complications

Interstitial lung disease (ILD) is one of the most serious pulmonary complications of connective tissue diseases (CTDs) and it is characterized by a deep impact on morbidity and mortality. Due to the poor knowledge of CTD-ILD’s natural history and due to the difficulties related to design of randomized control trials, there is a lack of prospective data about the prevalence, follow-up, and therapeutic efficacy. For these reasons, the choice of therapy for CTD-ILD is currently very challenging and still largely based on experts’ opinion. Treatment is often based on steroids and conventional immunosuppressive drugs, but the recent publication of the encouraging results of the INBUILD trial has highlighted a possible effective and safe use of antifibrotic drugs as a new therapeutic option for these subjects. Aim of this review is to summarize the available data and recent advances about therapeutic strategies for ILD in the context of various CTD, such as systemic sclerosis, idiopathic inflammatory myopathy and Sjogren syndrome, systemic lupus erythematosus, mixed connective tissue disease and undifferentiated connective tissue disease, and interstitial pneumonia with autoimmune features, focusing also on ongoing clinical trials.

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

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