Abstract
Background: Interstitial lung diseases (ILD) comprise a diverse group of heterogeneous entities. Epidemiology, clinical profile and prognosis of interstitial lung diseases widely vary globally. Little data are available on ILD in Sri Lanka. Objective and methodology: A single center descriptive study conducted at Teaching hospital-Kandy, Sri Lanka among diagnosed ILD patients from 2007-2018. Demographic, clinical and radiological data were collected retrospectively to analyse clinical profile, aetiology, classification and outcome of interstitial lung diseases. Results: 302 subjects were analyzed (mean age 59.5 years, female 61.3%). Idiopathic interstitial pneumonias (IIP) were the commonest (42.3%, N=128) followed by secondary ILD due to known aetiologies(40.7%, N=123), hypersensitivity pneumonitis (14.6%, N=44) and sarcoidosis (2%, N=6). Majority of IIPs were nonspecific interstitial pneumonia (NSIP)(46.8%, N=60), followed by idiopathic pulmonary fibrosis (IPF)(28.1%, N=36). Majority of secondary ILDs were due to connective tissue diseases (87%, N= 107). Shortness of breath and cough were the commonest presenting symptoms, found in 271 (90.3%) and 250 (83.3%) patients respectively. High resolutions computerized tomography (HRCT) was performed in all, but histology was available in 54 (17.8%). Lung functions tests (LFT) were normal in 71 (26.3%), but demonstrated restrictive pattern in 182 (67.4%). Mean percentage predicated forced vital capacity (FVC) was 66.91 ± 18.7% while mean percentage predicted forced expiratory volume at 01 second (FEV1) was 69.92 ± 20.0%. Therewas no significant change in LFT during follow up. Infective exacerbations needing hospitalization was the commonest complication seen in 86 (40.3%). Data on follow up radiological investigations were noted in 143 (47.5%), in which 59 (41.2%) demonstrated radiological improvement, while 34 (23.7%) had progressive changes and 50 (34.9%) had HRCT changes similar to previous images. 184 patients were found surviving, while 43 were dead. Higher mean age, male gender, current or previous smoking, lower distance achieved at 6-minute walking test, or any history of hospitalizations due to infective exacerbations were noted to be associated significantly in patients with mortality. Conclusion: IIP and secondary ILDs were similar in prevalence in the cohort of diagnosed ILD patients from central Sri Lanka. Idiopathic NSIP outnumbered IPF in the sample.
Highlights
Interstitial lung diseases (ILDs), synonymous with diffuse parenchymal lung diseases are a heterogeneous group of clinical, radiological and pathological entities which primarily affect the pulmonary interstitium
Majority of secondary ILDs were due to connective tissue diseases (87%, N= 107)
interstitial pneumonias (IIP) and secondary ILDs were similar in prevalence in the cohort of diagnosed ILD patients from central Sri Lanka
Summary
Interstitial lung diseases (ILDs), synonymous with diffuse parenchymal lung diseases are a heterogeneous group of clinical, radiological and pathological entities which primarily affect the pulmonary interstitium. This group of disorders is associated with variable degrees of pulmonary inflammation and fibrosis, leading to common functional outcome such as restricted lung volumes and impaired gas exchange [1]. The available data on epidemiology of ILDs varies significantly across the globe. This may represent the real difference attributed to genetic profile, environmental factors, occupational exposure, smoking habits and sociocultural practices, but may be due to differences in study designs, disease definition and classification [2].
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