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VOLUME 9 , ISSUE 2 ( July-December, 2020 ) > List of Articles

Original Article

Interstitial Pneumonia with Autoimmune Features – An Observational Study in a Tertiary Care Institute from South India

Ravi Charan Avala, Narendra K Narahari, Anu Kapoor, Bhaskar Kakarla, Rajasekhar Varma, Paramjyothi Kruparao Gongati

Keywords : Autoantibody profile, autoimmune features, connective tissue disease, interstitial pneumonia, nonspecific interstitial pneumonia

Citation Information : Avala RC, Narahari NK, Kapoor A, Kakarla B, Varma R, Gongati PK. 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.

DOI: 10.4103/ijrc.ijrc_13_20

License: CC BY-NC-SA 4.0

Published Online: 08-12-2022

Copyright Statement:  Copyright © 2020; Indian Journal of Respiratory Care.


Background: Often, patients with idiopathic interstitial pneumonia (IIP) have certain specific clinical features to suggest an autoimmune disease but do not justify the current rheumatologic classification systems to fit into a diagnosis of connective tissue disease (CTD)-associated IIP. There is a great paucity of clinical, serological, and radiological data of these patients from India. Aim: The aim was to study the clinicoradiological and autoantibody profile in patients with interstitial pneumonia with autoimmune features (IPAF). Methodology: It was a prospective, observational study conducted in a tertiary care center between December 2015 and December 2016. A total of 30 patients who satisfied the criteria for IPAF according to the American Thoracic Society/European Respiratory Society research were included in the study. Results: All 30 patients satisfied IPAF criteria, but they did not meet the immunological criteria for CTD. Majority of them were female (86.67%) and nonsmokers. The mean age was 52.5 ± 14.5 years. The most common clinical symptom was inflammatory arthritis in 20 (66.67%) patients, followed by Raynaud's phenomenon in 5 (16%) patients. Nonspecific interstitial pneumonia was the most common radiological pattern seen in 20 (66.67%) patients, while antinuclear antibody (1:320) was the most common autoantibody positive in 18 (60.0%) patients, followed by rheumatoid arthritis factor in 15 (50%) patients. Conclusions: High female predominance along with distinct imaging, histologic and serological characteristic features are seen in patients with IPAF as compared to those with IIP. Further studies in patients with IPAF are needed to understand the natural history and its management.

  1. Fischer A, Antoniou KM, Brown KK, Cadranel J, Corte TJ, du Bois RM, et al. An official European Respiratory Society/American Thoracic Society research statement: Interstitial pneumonia with autoimmune features. Eur Respir J 2015;46:976.87.
  2. Raghu G, Remy.Jardin M, Myers JL, Richeldi L, Ryerson CJ, Lederer DJ, et al. American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Latin American Thoracic Society. Diagnosis of idiopathic pulmonary fibrosis: an Official ATS/ERS/JRS/ALAT clinical practice guideline. Am J Respir Crit Care Med 2018;198: e44.68.
  3. Lee CT, Oldham JM. Interstitial pneumonia with autoimmune features: Overview of proposed criteria and recent cohort characterization. Clin Pulm Med 2017;24:191.6.
  4. Fischer A, du Bois R. Interstitial lung disease in connective tissue disorders. Lancet 2012;380:689.98.
  5. Fischer A, West SG, Swigris JJ, Brown KK, du Bois RM. Connective tissue disease.associated interstitial lung disease: A call for clarification. Chest 2010;138:251.6.
  6. Oldham JM, Adegunsoye A, Valenzi E, Lee C, Witt L, Chen L, et al. Characterization of patients with interstitial pneumonia with autoimmune features, Eur Respir J 2016;47:1622.4.
  7. Chartrand S, Lee JS, Swigris JJ, Stanchev L, Fischer A. Clinical characteristics and natural history of autoimmune forms of interstitial lung disease: A experience. Lung 2019;197:709.13.
  8. Ferri C, Manfredi A, Sebastiani M, Colaci M, Giuggioli D, Vacchi C, et al. Interstitial pneumonia with autoimmune features and undifferentiated connective tissue disease: Our interdisciplinary rheumatology.pneumology experience, and review of the literature. Autoimmun Rev 2016;15:61.70.
  9. Sharma R, Talwar D, Tuteja M. Clinico.radiological and autoimmune profile correlation in patients with autoimmune featured interstitial lung disease (AIFILD): An observational study in Indian scenario. Chest 2016;149:A456.
  10. Collins BF, Spiekerman CF, Shaw MA, Ho LA, Hayes J, Spada CA, et al. Idiopathic interstitial pneumonia associated with autoantibodies: A large case series followed over 1 Year. Chest 2017;152:103.12.
  11. Ahmad K, Barba T, Gamondes D, Ginoux M, Khouatra C, Spagnolo P, et al. Interstitial pneumonia with autoimmune features: Clinical, radiologic, and histological characteristics and outcome in a series of 57 patients. Respir Med 2017;123:56.62.
  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;19:120.
  13. Hu Y, Wang LS, Wei YR, Du SS, Du YK, He X, et al. Clinical characteristics of connective tissue disease.associated interstitial lung disease in 1,044 Chinese patients. Chest 2016;149:201.8.
  14. Oldham JM, Danoff SK. COUNTERPOINT: Does interstitial pneumonia with autoimmune features represent a distinct class of patients with idiopathic interstitial pneumonia? No. Chest 2019;155:260.3.
  15. Graney BA, Fischer A. Interstitial pneumonia with autoimmune features. Ann Am Thorac Soc 2019;16:525.33.
  16. Kono M, Nakamura Y, Enomoto N, Hashimoto D, Fujisawa T, Inui N, et al. Usual interstitial pneumonia preceding collagen vascular disease: A retrospective case control study of patients initially diagnosed with idiopathic pulmonary fibrosis. PLoS One 2014;9:e94775.
  17. Kono M, Nakamura Y, Yoshimura K, Enomoto Y, Oyama Y, Hozumi H, et al. Nonspecific interstitial pneumonia preceding diagnosis of collagen vascular disease. Respir Med 2016;117:40.7.
  18. Kamiya H, Panlaqui OM. Systematic review and meta.analysis of the prognosis and prognostic factors of interstitial pneumonia with autoimmune features. BMJ Open 2019;9:e031444.
  19. Yoshimura K, Kono M, Enomoto Y, Nishimoto K, Oyama Y, Yasui H, et al. Distinctive characteristics and prognostic significance of interstitial pneumonia with autoimmune features in patients with chronic fibrosing interstitial pneumonia. Respir Med 2018;137:167.75.
  20. Fernandes L, Nasser M, Ahmad K, Cottin V. Interstitial pneumonia with autoimmune features (IPAF). Front Med (Lausanne) 2019;6:209.
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