Idiopathic pulmonary fibrosis in Soweto, South Africa: A descriptive study

Abstract

Objectives: Idiopathic pulmonary fibrosis (IPF) is a specific form of age-related fibroproliferative interstitial pneumonia that is chronic, progressive, and carries a poor prognosis, with median survival of just 2.5–3.5 years from diagnosis. The exact etiology is unknown, but smoking is known to be risk factor. Symptoms and signs include progressive dyspnoea, cough, inspiratory “Velcro” crackles, and clubbing. At present, treatment options are limited; but include pulmonary rehabilitation, long-term domiciliary oxygen therapy, and the conditionally recommended pharmacological therapies pirfenidone and nintedanib. This study sought to describe the cohort of patients that attended the respiratory outpatient services at a tertiary-level hospital in South Africa during the period 2007– 2016. To the best of the authors’ knowledge, this is the first such descriptive study performed in Africa. Materials and Methods: This was a retrospective, descriptive, and record review, that included patients ≥18 years of age who fulfilled 2011 ATS/ERS/JRS/ALAT diagnostic criteria for IPF. Results: Data from 74 patients were used for analysis in this study, of which 60.8% were female. The mean age standard deviation was 64.4 (10.9) years and the majority (79.7%) were Black. Over half of the patients (40/74, 54.1%) were current or previous smokers, although there was no correlation between smoking history and age or baseline pulmonary function testing. All patients reported dyspnea, which was modified Medical Reseach Council (mMRC) Grade 3 or 4 in 80% of patients. High resolution computed tomography chest was reported as radiological usual interstitial pneumonia (UIP) in 72 patients (97.3%) and three patients underwent lung biopsy, all of which showed a UIP pattern. Fifty-eight patients (78.4%) had spirometry results available, with median forced vital capacity 67.3% of predicted; this was significantly higher in females. Median transfer factor of the lung for carbon monoxide was 39% predicted. Twenty-five patients (33.8%) received corticosteroids, of whom five (6.8%) received the prednisone-azathioprine-N-acetylcysteine regime. Three patients (4.1%) received nintedanib; two of whom showed slowing of decline in lung function, although no significant symptomatic improvement was reported. Mean duration of follow-up was 13.3 months, although females had significantly longer duration of follow-up than males. Conclusion: Despite a fairly small sample size and retrospective nature, this study contributes to the body of literature on IPF and highlights the need for additional studies in developing countries, particularly in Africa.