Spectrum of Interstitial Lung Disease Patterns in Connective Tissue Diseases and their Outcomes: a Retrospective Analysis

Amar Lal; Muhammad Ahmed Saeed; Muhammad Rafaqat Hameed; Maryam Aamer; Umbreen Arshad; Hiba Shariq

doi:10.51253/pafmj.v76iSUPPL-1.13831

Authors

Amar Lal Department of Rheumatology, Central Park Teaching Hospital Lahore, Pakistan https://orcid.org/0009-0004-0264-8801
Muhammad Ahmed Saeed Department of Rheumatology, Central Park Teaching Hospital Lahore, Pakistan https://orcid.org/0000-0003-4771-4441
Muhammad Rafaqat Hameed Department of Rheumatology, Central Park Teaching Hospital Lahore, Pakistan
Maryam Aamer Department of Rheumatology, Central Park Teaching Hospital Lahore, Pakistan
Umbreen Arshad Department of Rheumatology, Central Park Teaching Hospital Lahore, Pakistan
Hiba Shariq Department of Rheumatology, Central Park Teaching Hospital Lahore, Pakistan

DOI:

https://doi.org/10.51253/pafmj.v76iSUPPL-1.13831

Keywords:

Interstitial lung disease, Mixed connective tissue disease, Pakistan, Retrospective study

Abstract

Objective: To evaluate patterns of connective tissue disease-associated interstitial lung disease and to evaluate disease outcomes across various CTD subtypes in a tertiary care setting in Lahore, Pakistan.

Study Design: Observational Retrospective Study

Place and Duration of the Study: Rheumatology Department, Central Park Teaching Hospital, and its affiliated Clinics, Lahore, Pakistan, from 2021 to 2025.

Methodology: The data of 174 patients diagnosed with CTD-ILD was reviewed. Inclusion criteria included adult patients with a confirmed CTD diagnosis (as per ACR/EULAR criteria) and HRCT-confirmed ILD. Data on demographics, serology, HRCT patterns, and pulmonary function tests (PFTs), and outcomes were analyzed using SPSS v27. The statistical significance was set at p ≤ 0.05.

Results: Among 174 patients, 93.7% were female, with a mean age of 49.4 ± 13.7 years. The most common CTD was systemic sclerosis (30.5%), followed by rheumatoid arthritis (21.8%) and mixed connective tissue disease (MCTD) (19.5%). NSIP was the predominant HRCT pattern (61.5%), followed by UIP (23.0%). Disease activity varied, with active disease most common in SLE (62.5%) and systemic sclerosis (47.2%). PFTs showed moderate to severe restriction in a majority of patients, particularly those with myositis, systemic sclerosis, and SLE (p < 0.001).

Conclusion: CTD-ILD presents with heterogeneous clinical, serological, and radiological profiles. NSIP is the most frequent ILD pattern across CTDs. Timely identification of ILD patterns and targeted immunosuppressive therapy may improve patient outcomes.

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Author Biography

Muhammad Rafaqat Hameed, Department of Rheumatology, Central Park Teaching Hospital Lahore, Pakistan

References

1. Yoo H, Hino T, Hwang J, Franks TJ, Han J, Im Y, et al. Connective tissue disease-related interstitial lung disease (CTD-ILD) and interstitial lung abnormality (ILA): Evolving concept of CT findings, pathology and management. Eur J Radiol 2022; 9(1): 100419.

https://doi.org/10.1016/j.ejro.2022.100419

2. Joy GM, Arbiv OA, Wong CK, Lok SD, Adderley NA, Doboszet KM, et al. Prevalence, imaging patterns and risk factors of interstitial lung disease in connective tissue disease: a systematic review and meta-analysis. Eur Respir Rev 2023; 32: 220210. https://doi.org/10.1183/16000617.0210-2022

3. Zubairi ABS, Ahmad H, Hassan M, Siddiqui F, Iqbal N, Riaz M, et al. Comparative analysis of connective tissue disease-associated interstitial lung disease and idiopathic pulmonary fibrosis from a tertiary care centre in Pakistan. J Pak Med Assoc 2021; 71(10): 2330-2334.

https://doi.org/10.47391/JPMA.01-107

4. Vacchi C, Sebastiani M, Cassone G, Cerri S, Casa GD, Salvarani C, et al. Therapeutic options for the treatment of interstitial lung disease related to connective tissue diseases. a narrative review. J Clin Med 2020; 9(2): 407.https://doi.org/10.3390/jcm9020407

5. Mai C. Connective tissue disease‑associated interstitial lung disease (CTD‑ILD). J Belgian Soc Radiol 2024; 108(1): 99. https://doi.org/10.5334/jbsr.3780

6. Jeganathan N, Sathananthan M. Connective tissue disease-related interstitial lung disease: prevalence, patterns, predictors, prognosis, and treatment. Lung 2020; 198(1): 735-759.

https://doi.org/10.1007/s00408-020-00383-w

7. Jafri S, Ahmed N, Saifullah N, Musheer M. Epidemiology and clinico-radiological features of interstitial lung diseases. Pak J Med Sci 2020; 36(3):365-370.

https://doi.org/10.12669/pjms.36.3.1046

8. Shahzad T. Connective tissue disease associated interstitial lung disease- an acute exacerbation in emergency room. J Ayub Med Coll Abbottabad 2019; 31(1):127-128.

9. Olaosebikan H, Adeyeye O, Akintayo R, Akpabio A, Adenitan A, Adelowo O, et al. Connective tissue disease--associated interstitial lung disease: an underreported cause of interstitial lung disease in Sub-Saharan Africa. Clin Rheumatol 2021; 40(9): 3455-3460. https://doi.org/10.1007/s10067-020-05336-5

10. Hazarika K, Sahoo RR, Mohindra N, Wakhlu A, Manoj M, Bafna P, et al. Clinical, radiologic and serologic profile of patients with interstitial pneumonia with autoimmune features: a cross-sectional study. Rheumatol Int 2022; 42(8):1431-1441.

https://doi.org/10.1007/s00296-021-04883-7

11. Ahmad H, Imran M, Zeb A, Din IU, Zia S. Clinical, Serological and Radiological Profile of Patients with Autoimmune Disease Associated Interstitial Lung Disease: Autoimmune Disease Associated Interstitial Lung Disease. Pak J Health Sci 2025; 6(4): 36-42. https://doi.org/10.54393/pjhs.v6i4.2864

12. Lim JU, Gil BM, Kang HS, Oh J, Kim YH, Kwon SS. Interstitial pneumonia with autoimmune features show better survival and less exacerbations compared to idiopathic pulmonary fibrosis. BMC Pulm Med 2019; 19(1): 120.: https://doi.org/10.1186/s12890-019-0868-9

13. Karampeli M, Thomas K, Tseronis D, Aggelakos M, Kassara D, Havatza K, et al. AB1216 interstitial pneumonia with autoimmune features (IPAF): a single center, prospective study. Ann of Rheum Dis 2020; 79(1): 1899-900. https://doi.org/10.1136/annrheumdis-2020-eular.2753

14. Atienza-Mateo B, Remuzgo-Martínez S, Mora Cuesta VM, Iturbe-Fernández D, Fernández-Rozas S, Prieto-Peña D, et al. The spectrum of interstitial lung disease associated with autoimmune diseases: data of a 3.6-year prospective study from a referral center of interstitial lung disease and lung transplantation. J Clin Med 2020; 9(6): 1606. https://doi.org/10.3390/jcm9061606

15. Zubairi ABS, Ansarie M, Mahmud T, Ashraf S, Rao NA, Javaid A, et al. National Registry of Interstitial Lung Disease from Pakistan. Cureus 2021; 13(4): e14684.

https://doi.org/10.7759/cureus.14684

16. Avala R, Narahari N, Kapoor A, Kakarla B, Varma R, Gongati P. Interstitial Pneumonia with Autoimmune Features-An Observational Study in A Tertiary Care Institute from South India. Indian J Respir Care 2020; 9(2): 209-215. https://doi.org/10.4103/ijrc.ijrc_13_20

17. Lu M, Gong L, Huang C, Ye M, Wang H, Liu Y, et al. Analysis of Clinical Characteristics of Connective Tissue Disease-Associated Interstitial Lung Disease in 161 Patients: A Retrospective Study. Int J Gen Med 2022; 15: 8617-8625. https://doi.org/10.2147/IJGM.S391146

18. Chartrand S, Swigris JJ, Stanchev L, Lee JS, Brown KK, Fischer A. Clinical features and natural history of interstitial pneumonia with autoimmune features: A single center experience. Resp Med 2016; 119: 150-154. http://doi.org/10.1016/j.rmed.2016.09.002

19. Ye Y, Sing CW, Hubbard R, Lam DCL, Li HL, Li GH, et al. Prevalence, incidence, and survival analysis of interstitial lung diseases in Hong Kong: a 16-year population-based cohort study. Lancet Reg Health West Pac 2023; 42: 100871. https://doi.org/10.1016/j.lanwpc.2023.100871

20. Vahidy S, Agyeman J, Zheng B, Donohoe K, Hambly N, Johannson KA, et al. Characteristics and risk factors of interstitial pneumonia with autoimmune features. Respir Med 2024; 221: 107500. https://doi.org/10.1016/j.rmed.2023.107500

21. Seetha AP, Musthafa S, Venugopal KP, Gangadharan H. The Spectrum of Interstitial Lung Disease in a Tertiary Care Center in South India. Pulmon 2025; 27(1):24-7. https://doi.org/10.4103/pulmon.pulmon_37_24

22. Vivero F, Campins F, Lancellotti D, Malfante P, Babini S, Sebastiani J, et al. Autoimmune interstitial lung disease in Latin-America. Clin Immunol 2019; 199: 52-56.

https://doi.org/10.1016/j.clim.2018.12.013

23. Oliveira RP, Ribeiro R, Melo L, Grima B, Oliveira S, Alves JD. Connective tissue disease-associated interstitial lung disease. Pulmonology 2022; 28(2): 113-118.

https://doi.org/10.1016/j.pulmoe.2020.01.004

24. Nayebirad S, Mohamadi A, Yousefi-Koma H, Javadi M, Farahmand K, Atef-Yekta R, et al. Association of anti-Ro52 autoantibody with interstitial lung disease in autoimmune diseases: a systematic review and meta-analysis. BMJ Open Respir Res 2023; 10(1): e002076.

https://doi.org/10.1136/bmjresp-2023-002076