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Review
. 2024 Mar 22;50(1):e20230132.
doi: 10.36416/1806-3756/e20230132. eCollection 2024.

Connective tissue disease-associated interstitial lung disease

[Article in English, Portuguese]
Karin Mueller Storrer  1 , Carolina de Souza Müller  1 , Maxwell Cássio de Albuquerque Pessoa  1 , Carlos Alberto de Castro Pereira  2
Affiliations
Review

Connective tissue disease-associated interstitial lung disease

[Article in English, Portuguese]
Karin Mueller Storrer et al. J Bras Pneumol. .

Abstract
in English, Portuguese

Connective tissue disease-associated interstitial lung disease (CTD-ILD) represents a group of systemic autoimmune disorders characterized by immune-mediated organ dysfunction. Systemic sclerosis, rheumatoid arthritis, idiopathic inflammatory myositis, and Sjögren's syndrome are the most common CTDs that present with pulmonary involvement, as well as with interstitial pneumonia with autoimmune features. The frequency of CTD-ILD varies according to the type of CTD, but the overall incidence is 15%, causing an important impact on morbidity and mortality. The decision of which CTD patient should be investigated for ILD is unclear for many CTDs. Besides that, the clinical spectrum can range from asymptomatic findings on imaging to respiratory failure and death. A significant proportion of patients will present with a more severe and progressive disease, and, for those, immunosuppression with corticosteroids and cytotoxic medications are the mainstay of pharmacological treatment. In this review, we summarized the approach to diagnosis and treatment of CTD-ILD, highlighting recent advances in therapeutics for the various forms of CTD.

Doença pulmonar intersticial associada à doença do tecido conjuntivo (DPI-DTC) representa um grupo de distúrbios autoimunes sistêmicos caracterizados por disfunção de órgãos imunomediada. Esclerose sistêmica, artrite reumatoide, miosite inflamatória idiopática e síndrome de Sjögren são as DTC mais comuns que apresentam acometimento pulmonar, bem como pneumonia intersticial com achados autoimunes. A frequência de DPI-DTC varia de acordo com o tipo de DTC, mas a incidência total é de 15%, causando um impacto importante na morbidade e mortalidade. A decisão sobre qual paciente com DTC deve ser investigado para DPI não é clara para muitas DTC. Além disso, o espectro clínico pode variar desde achados assintomáticos em exames de imagem até insuficiência respiratória e morte. Parte significativa dos pacientes apresentará doença mais grave e progressiva, e, para esses pacientes, imunossupressão com corticosteroides e medicamentos citotóxicos são a base do tratamento farmacológico. Nesta revisão, resumimos a abordagem do diagnóstico e tratamento de DPI-DTC, destacando os recentes avanços na terapêutica para as diversas formas de DTC.

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Conflict of interest statement

CONFLICTS OF INTEREST: None declared.

Figures

Figure 1
Figure 1. HRCT scans (in A) and capillaroscopy features (in B) in a patient with systemic sclerosis. NSIP: nonspecific interstitial pneumonia.
Figure 2
Figure 2. Treatment algorithm for systemic sclerosis-associated interstitial lung disease (SSc-ILD) based on evidence and expert opinion. PFT: pulmonary function test; MMF: mycophenolate; and CPR: C-reactive protein. Modified from Roofeh et al.
Figure 3
Figure 3. HRCT features in a patient with rheumatoid arthritis. UIP: usual interstitial pneumonia.
Figure 4
Figure 4. Treatment algorithm for rheumatoid arthritis-associated interstitial lung disease (RA-ILD) based on evidence and expert opinion. DMARDs: disease-modifying anti-rheumatic drugs; and MMF: mycophenolate.
Figure 5
Figure 5. HRCT scans (in A) and cutaneous features (in B) in idiopathic inflammatory myopathy (IIM). NSIP: nonspecific interstitial pneumonia.
Figure 6
Figure 6. Treatment algorithm for idiopathic inflammatory myositis-associated interstitial lung disease (IIM-ILD) based on evidence and expert opinion. MMF: mycophenolate. Modified from Barba et al. and Morisset et al.
Figure 7
Figure 7. Suggested additional therapies for treatment of connective tissue disease-associated interstitial lung disease.
See this image and copyright information in PMC

References

    1. Fischer A, du Bois R. Interstitial lung disease in connective tissue disorders [published correction appears in. Lancet. 2012;380(9848):1148–1148. doi: 10.1016/S0140-6736(12)61079-4. - DOI - PubMed
    1. Atzeni F, Gerardi MC, Barilaro G, Masala IF, Benucci M, Sarzi-Puttini P. Interstitial lung disease in systemic autoimmune rheumatic diseases a comprehensive review. Expert Rev Clin Immunol. 2018;14(1):69–82. doi: 10.1080/1744666X.2018.1411190. - DOI - PubMed
    1. Hunninghake GM, Goldin JG, Kadoch MA, Kropski JA, Rosas IO, Wells AU. Detection and Early Referral of Patients With Interstitial Lung Abnormalities An Expert Survey Initiative. Chest. 2022;161(2):470–482. doi: 10.1016/j.chest.2021.06.035. - DOI - PMC - PubMed
    1. Smith V, Scirè CA, Talarico R, Airo P, Alexander T, Allanore Y. Systemic sclerosis state of the art on clinical practice guidelines. RMD Open. 2018;4(Suppl 1):e000782. doi: 10.1136/rmdopen-2018-000782. - DOI - PMC - PubMed
    1. Hoffmann-Vold AM, Maher TM, Philpot EE, Ashrafzadeh A, Barake R, Barsotti S. The identification and management of interstitial lung disease in systemic sclerosis evidence-based European consensus statements. Lancet Rheumatol. 2020;2(2):e71–e83. doi: 10.1016/S2665-9913(19)30144-4. - DOI - PubMed

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