Connective Tissue Disease (CTD)

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer


Connective tissue diseases are complex illnesses that can affect any part of the body, such as dermatomyositis/polymyositis, lupus, mixed connective tissue disease, rheumatoid arthritis, scleroderma, and Sjogren's.

Connective Tissue Disease. A connective tissue disease is any disease that has the connective tissues of the body as a primary target of pathology.

Thoracic Manifestations of Connective Tissue Diseases. The most important thoracic manifestations of CTDs are interstitial lung disease and pulmonary arterial hypertension, with nonspecific interstitial pneumonia being the most common pattern of interstitial lung disease. PubMed, Curr Probl Diagn Radiol, 2014 Aug 14.


Acute Respiratory Events in Connective Tissue Disorders (CTDs). In CTDs the lungs significantly contribute to morbidity and mortality, since they constitute a common site of disease involvement. PubMed, Respiration, 03/04/2016.

Investigation of Lung Involvement in Connective Tissue Disorders (CTD). The investigation of lung involvement in CTDs requires adequate skills in the ambit of a multidisciplinary approach and an extended spectrum of diagnostic tools. PubMed, Respiration, 06/26/2015. (Also see Pulmonary Fibrosis Diagnosis)

Clinical characteristics and survival in idiopathic pulmonary fibrosis (IPF) and connective tissue disease-associated usual interstitial pneumonia (CTD-UIP). Although the survival of CTD-UIP patients was similar compared with that of IPF/UIP patients, it appears that Undifferentiated Connective Tissue Disease influences the survival rate of CTD-UIP patients. Journal of Thoracic Disease, 04/03/2015. (Also see Pulmonary Fibrosis Prognosis)

Histopathology of Lung Disease in the Connective Tissue Diseases (CTD). This article will cover the pulmonary pathologies seen in rheumatoid arthritis, systemic sclerosis, myositis, systemic lupus erythematosus, Sjögren syndrome, and mixed CTD. Rheumatic Disease Clinics of North America, 03/06/2015. (Also see Pulmonary Fibrosis Diagnosis)

Interstitial lung disease (ILD) in connective tissue disease (CTD) - mechanisms and management. Data from treatment trials in systemic sclerosis support the use of immunosuppressive therapy, with the treatment benefit largely relating to the prevention of progression of lung disease. PubMed, Nat Rev Rheumatol, 2014 Sep 30. (Also see Pulmonary Fibrosis Treatments)

Interstitial Lung Disease (ILD) in Undifferentiated Forms of Connective Tissue Disease(CTD). There is much uncertainty and controversy surrounding the suggestive forms of CTD-associated ILD (CTD-ILD) and prospective studies are needed to provide a better understanding of the natural history of these cohorts. PubMed, Arthritis Care Res, 2014 Jul 21. (Also see Lung Involvement Prognosis)

Prevalence of pulmonary arterial hypertension (PAH) in patients with connective tissue diseases: a systematic review of the literature. Prevalence of PAH in patients with connective tissue diseases was substantially higher than that of idiopathic PAH based on pooled prevalence estimates. PubMed, Clin Rheumatol, 2013 Jun 20. (Also see Pulmonary Hypertension Research)

Connective Tissue Disease-Associated Interstitial Lung Disease (CTD): A Focused Review. The prognosis of patients with CTD having respiratory failure is often quite poor, highlighting the need for prompt and thorough clinical assessments to determine the underlying etiology and implementation of appropriate therapeutic strategies. PubMed, J Intensive Care Med, 2013 Dec 25. (Also see Lung Involvement Prognosis)

Reconciling Healthcare Professional and Patient Perspectives in the Development of Disease Activity and Response Criteria in Connective Tissue Disease-related Interstitial Lung Diseases (CTD-ILD). Efforts described here demonstrate unequivocally the value and influence of patient involvement on core set development. PubMed, J Rheumatol, 2014 Feb 1. (Also see Pulmonary Fibrosis)

Frequency of Pleural Effusions in Patients with Pulmonary Arterial Hypertension Associated with Connective Tissue Diseases (CTD) Pleural effusions frequently accumulate in patients with pulmonary arterial hypertension associated with CTD, and are associated with right heart failure. Chest, [2011]. (Also see Pleural Effusions, and Pulmonary Hypertension)


Connective tissue disease (CTD) increases risk for cardiovascular problems. The findings show that CTD is associated with higher prevalence of atherosclerotic cardiovascular disease and these insights could be used to improve how patients with a wide range of connective tissue diseases are evaluated. University of Chicago Medical Center, 02/05/2016.

How are cancer and connective tissue diseases (CTDs) related to sarcoidosis? The association between sarcoidosis and both cancer and CTDs may yield important insights into the immunopathogenesis of all three diseases. PubMed, Curr Opin Pulm Med, 2015 Sep;21(5):517-24. (Also see Sarcoidosis and Cancer)

Concomitant sarcoidosis and a connective tissue disease (CTD). A significant portion of patients with CTDs and sarcoidosis may actually not have the latter disease and the diagnosis of a concomitant connective tissue disease and sarcoidosis must be made with extreme caution. PubMed, Respir Med, 2013 Sep;107(9):1453-9. (Also see Sarcoidosis)


Determining the necessity for right heart catheterization (RHC) in pulmonary hypertension (PH) associated with connective tissue diseases (CTD) assessed by echocardiography. These findings demonstrate the necessity for RHC and differences in prognosis of PH in CTD. Wiley Online Library, 10/07/2015. (Also see Pulmonary Hypertension Diagnosis)

Frequency of development of connective tissue disease (CTD) in statin-users versus nonusers. Statins have pleiotropic properties that may affect the development of (CTD). Statin use was associated with a lower risk of CTD. PubMed, Am J Cardiol, 09/15/2013. (Also see Statins)

Latest advances in connective tissue disorders. Genetic studies suggest a role for the innate immune system in disease pathogenesis, suggesting further future targets for biological therapies over the next few years. Therapeutic Advances in Musculoskeletal Disease, 2013 August; 5(4): 234–249.

Silicone breast implants and connective tissue disease: no association. Any claims that remain regarding an association between cosmetic breast implants and CTDs are not supported by the scientific literature but rather are a residual byproduct of the unprecedented large-scale product liability litigation in the USA. Semin Immunopathol, [2011]. (Also see Causes of Scleroderma: Artificial Joints and Breast Implants)

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