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Connective Tissue Disease-Associated Interstitial Lung Disease

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Abstract

Various systemic autoimmune connective tissue disorders are associated with interstitial lung disease (ILD). Rheumatoid arthritis involves the joints; thoracic manifestations include ILD, diseases of the pleura, small or large airways, and pulmonary arteries. Systemic lupus erythematosus (SLE) is a relapsing–remitting chronic autoimmune disease that can affect multiple organs. Pulmonary involvement is more commonly seen in SLE than in any other connective tissue diseases. Sjogren syndrome occurs due to inflammatory lymphocytic infiltration of glandular and extraglandular organs. It is characterized by diminished function of exocrine glands that lead to eye and mouth dryness (sicca syndrome). ILD and small airway disease are the most common forms of lung involvement in Sjogren. Systemic sclerosis is a connective tissue disorder with multisystem involvement characterized by dysregulation of the immune system, and excess collagen formation leading to fibrosis of skin, vascular system, and multiple organs. ILD is more likely to develop in diffuse cutaneous systemic sclerosis than in the limited cutaneous entity. Dermatomyositis (DM) and polymyositis (PM) belong to a group of idiopathic inflammatory myopathies, which are connective tissue disorders that mostly involve the skeletal muscle. ILD is the major determinant of mortality and morbidity in patients with DM and PM. Ankylosing spondylitis (AS) is a chronic progressive autoimmune disorder involving the spine, the sacroiliac joints, and less commonly the peripheral joints. Pulmonary apical fibrosis is one of the commonest pulmonary features seen in AS. Relapsing polychondritis (RP) is a rare relapsing–remitting autoimmune disorder that causes inflammation predominantly of cartilaginous tissues. Airway involvement in the form of sub-glottic stenosis, tracheobronchomalacia, thickened and calcified tracheal wall with sparing of the posterior membranous wall, and tracheal stenosis which can be localized or diffuse are frequently seen pulmonary features in RP. Finally, mixed connective tissue disease comprises combination or overlap of systemic sclerosis, SLE, and DM/PM.

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Authors and Affiliations

  1. Division of Pulmonary, Critical Care and Sleep Medicine, UTHealth Houston–McGovern Medical School, Houston, TX, USA

    Reeba Mathew & Sungryong Noh

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  1. Reeba Mathew
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  2. Sungryong Noh
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Correspondence to Reeba Mathew .

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  1. Department of Pathology, The University of Texas MD Anderson Cancer, Houston, TX, USA

    Cesar A. Moran

  2. Department of Thoracic Imaging, The University of Texas MD Anderson Cancer, Houston, TX, USA

    Mylene T. Truong

  3. Department of Thoracic Imaging, The University of Texas MD Anderson Cancer, Houston, TX, USA

    Patricia M. de Groot

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Mathew, R., Noh, S. (2023). Connective Tissue Disease-Associated Interstitial Lung Disease. In: Moran, C.A., Truong, M.T., de Groot, P.M. (eds) The Thorax. Springer, Cham. https://doi.org/10.1007/978-3-031-21040-2_19

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