Spectrum of diffuse parenchymal lung disease with special reference to idiopathic pulmonary fibrosis: experience at Charlotte Maxeke Johannesburg Academic Hospital
Date
2017
Authors
Ndikundisani Ananius, Tshiovhe
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Abstract
Background
Diffuse parenchymal lung diseases (DPLD) encompass a group of diseases with a wide range
of causes with varied presentations and prognosis. The known causes include occupational or
environmental exposure, drug induced lung diseases, hypersensitivity pneumonitis and
connective tissue disease (CTD). Among the DPLD with unknown cause, idiopathic pulmonary
fibrosis (IPF) is the commonest and has the worst outcome. The earlier publications from this
country described cryptogenic fibrosing alveolitis. With subsequent characterisation of
idiopathic interstitial pneumonia (IIP), and an increase in the burden of IPF reported worldwide,
we evaluated the clinical features of patients with IPF in the South African context.
Objectives
To evaluate the clinical spectrum of DPLD encountered in Johannesburg, South Africa, and to
describe the clinical profile of patients with IPF.
Methods
A retrospective record review of patient files was conducted who attended the Charlotte
Maxeke Johannesburg Academic Hospital (CMJAH) Respiratory Clinic in the past 5 years
from January 2011 to December 2015. Patients with DPLD were identified and the diagnoses
were noted. The records of patients with IPF were further analysed.
v
Results
We identified 132 patients with DPLD. Sarcoidosis (37.8%), IPF (21.2%), connective tissue
associated diffuse parenchymal lung disease (CTD-DPLD) (14.3%), and hypersensitivity
pneumonitis (HP) (9.8%) were the four most common subtypes. IPF was seen in all racial
groups. Of the 28 patients with IPF in our cohort, there was a slight female predominance
(1.3:1). The mean age of the patients in our study was 63.8 years and the majority were Whites.
Cough (96.4%), dyspnoea (92.8%) and bilateral crackles (96.4%) were the commonest clinical
features. The majority of patients (78.5%) were diagnosed by high resolution computerised
tomography (HRCT) scan.
Conclusion
IPF is the second most common DPLD disease encountered after sarcoidosis at the CMJAH.
IPF is seen in all racial groups in Johannesburg, South Africa, and the characteristics of patients
with IPF are similar to those seen in other parts of the world.
Description
A research report submitted to the University of the Witwatersrand, Johannesburg,
in fulfilment for the requirements of the degree of
Master of Medicine in Internal Medicine 2017