# Clinical Profile of Patients with Interstitial Lung Disease with Underlying Autoimmune Rheumatic Disease Presenting to a Tertiary Care Setting: A Descriptive Cross-sectional Study

**Authors:** Ujjwol Prasad Risal, Rakshya Pandey, Raju Prasad Pangeni

PMC · DOI: 10.31729/jnma.v63i292.9268 · 2025-12-31

## TL;DR

This study describes the clinical features of patients with interstitial lung disease linked to autoimmune rheumatic diseases in a hospital in Nepal.

## Contribution

The study provides a detailed clinical profile of autoimmune rheumatic disease-related interstitial lung disease in a specific geographic and healthcare setting.

## Key findings

- Rheumatoid arthritis was the most common autoimmune rheumatic disease associated with interstitial lung disease.
- Usual interstitial pneumonia was the most frequently observed radiographic pattern.
- Corticosteroids were the most commonly used treatment among the patients.

## Abstract

Interstitial lung diseases are a diverse group of disorders that display considerable overlap clinically, physiologically, and radiologically. A significant subset of these patients has autoimmune rheumatic diseases. This study aimed to characterize the clinical profile of patients with autoimmune rheumatic disease-related interstitial lung disease, focusing on demographic features, radiographic patterns, and treatment practices.

A descriptive cross-sectional study was conducted at the Hospital for Advanced Medicine and Surgery in Kathmandu, Nepal, from May 2022 to May 2023. Patients with interstitial lung disease confirmed by high-resolution computed tomography and a rheumatologist-verified diagnosis of autoimmune rheumatic disease were included. Demographic, clinical, laboratory, imaging, and pulmonary function data were collected and analysed.

A total of 36 patients were included, comprising 28 (77.80%) females and 8 (22.20%) males, with a mean age of 58.92 ± 15.89 years. Rheumatoid arthritis was present in 18 (50%) cases, while undifferentiated systemic rheumatic disease was seen in 7 (19.44 %). Extrapulmonary manifestations were the initial presentation in 24 (66.7%) of cases. Usual interstitial pneumonia was identified in 22 (61.1%) patients. The mean forced vital capacity was 65.90 ± 25.38%, and the mean diffusion capacity of the lungs for carbon monoxide was 47.22 ± 23.21%. Methotrexate and hydroxychloroquine were each used by 6 (16.70%) patients, while corticosteroids were used by 11 (30.60%) patients.

Interstitial lung disease is a significant complication of autoimmune rheumatic diseases. Early identification through regular clinical assessment and imaging is essential to prevent disease progression and improve outcomes.

## Linked entities

- **Diseases:** interstitial lung disease (MONDO:0015925), rheumatoid arthritis (MONDO:0008383)

## Full-text entities

- **Genes:** IFIH1 (interferon induced with helicase C domain 1) [NCBI Gene 64135] {aka AGS7, Hlcd, IDDM19, IMD95, MDA-5, MDA5}, TRIM21 (tripartite motif containing 21) [NCBI Gene 6737] {aka RNF81, RO52, Ro/SSA, SSA, SSA1, TRIM21/Ro52}, SNRNP70 (small nuclear ribonucleoprotein U1 subunit 70) [NCBI Gene 6625] {aka RNPU1Z, RPU1, SNRP70, Snp1, U1-70K, U170K}, SSB (small RNA binding exonuclease protection factor La) [NCBI Gene 6741] {aka LARP3, La, La/SSB, SSB/La}
- **Diseases:** adrenal insufficiency (MESH:D000309), PSS (MESH:D012859), diabetes mellitus (MESH:D003920), lung injury (MESH:D055370), IIMs (OMIM:300896), fibrosis (MESH:D005355), SLE (MESH:D008180), MCTD (MESH:D008947), Systemic sclerosis (MESH:D012595), Rheumatoid (MESH:D011695), AIRDs (MESH:C537066), Clubbing (MESH:D003025), IIM (MESH:D009220), pneumonia (MESH:D011014), small vessel vasculitis (MESH:C565222), UIP (MESH:D054990), inflammatory joint disease (MESH:D007592), cough (MESH:D003371), Autoimmune Rheumatic Disease (MESH:D012216), autoimmune hemolytic anemia (MESH:D000744), HAMS (MESH:D003428), Palindromic Rheumatism (MESH:C538103), RA (MESH:D001172), pulmonary symptom (MESH:D012818), connective tissue diseases (MESH:D003240), hypothyroidism (MESH:D007037), Hypertension (MESH:D006973), pemphigus foliaceous (MESH:D010392), bronchiolitis (MESH:D001988), Pulmonary hypertension (MESH:D006976), ILD (MESH:D017563), systemic vasculitides (MESH:D056647), RP (MESH:D011928)
- **Chemicals:** carbon monoxide (MESH:D002248), tofacitinib (MESH:C479163), cyclic citrullinated peptide (MESH:C487763), Methotrexate (MESH:D008727), rituximab (MESH:D000069283), leflunomide (MESH:D000077339), prednisolone (MESH:D011239), hydroxychloroquine (MESH:D006886), steroids (MESH:D013256)
- **Species:** Homo sapiens (human, species) [taxon 9606]

---
Source: https://tomesphere.com/paper/PMC12906751