title: Pulmonary involvement in systemic sclerosis: A clinical profile.
abstract: BACKGROUND
  Systemic sclerosis is a generalized disorder of connective tissue affecting skin and internal organs. Lung involvement accounts for significant morbidity and is a leading cause of mortality in patients.
  
  
  OBJECTIVE
  This study intends to study the frequency of occurrence of pulmonary involvement in progressive systemic sclerosis (PSS) and to describe the clinical and radiological picture of pulmonary involvement in PSS.
  
  
  METHODS
  This was a descriptive cross-sectional study. A detailed history, modified Rodnan score, clinical examination, routine investigation, antinuclear antibody, immuno biot, chest X-ray (CXR), pulmonary function test (PFT), and 6 min walk test (6MWT) were performed on all patients. High resolution computed tomography was done on those who consented.
  
  
  RESULTS
  Hundred subjects with PSS were included in the study; 90 were females and 10 were males. Common presenting complaints were skin thickening in 98% and Raynaud's phenomenon in 98%. Skin thickening of digits beyond metacarpo phalangeal was seen in 98%, face and neck in 92%, and hands in 92%. Chest wall thickening was seen in 40 subjects (40%). 90 (90%) of the studied subjects had pulmonary involvement, longer duration of disease was significantly associated with pulmonary involvement (P < 0.05). Dyspnea, cough, bilateral crepitations, CXR, Borg score, and Rodnan score was found to be significantly associated with severe pulmonary involvement (P < 0.05).
  
  
  CONCLUSIONS
  The prevalence of pulmonary involvement in this cohort study was 90%. Almost 1/3(rd) of patients, that is 29 (29%) were detected to have pulmonary involvement despite being asymptomatic for respiratory complaints, hence early screening and evaluation is recommended. PFT and 6MWT are noninvasive, cost-effective, and easily available screening tests which can be used in resource-limited settings.
authors.affiliation: Department of Pulmonary Medicine, St John's Medical College Hospital, Bengaluru, Karnataka, India.
authors.forename: A S
authors.initials: AS
authors.lastname: Deepa
authors.affiliation: Department of Pulmonary Medicine, St John's Medical College Hospital, Bengaluru, Karnataka, India.
authors.forename: Roopa Premanand
authors.initials: RP
authors.lastname: Rachel
authors.affiliation: Department of Pulmonary Medicine, St John's Medical College Hospital, Bengaluru, Karnataka, India.
authors.forename: Priya
authors.initials: P
authors.lastname: Ramchandran
authors.affiliation: Department of Pulmonary Medicine, St John's Medical College Hospital, Bengaluru, Karnataka, India.
authors.forename: Uma
authors.initials: U
authors.lastname: Devaraj
authors.affiliation: Department of Pulmonary Medicine, St John's Medical College Hospital, Bengaluru, Karnataka, India.
authors.forename: S Abhilash
authors.initials: SA
authors.lastname: Arnold
authors.affiliation: Department of Pulmonary Medicine, St John's Medical College Hospital, Bengaluru, Karnataka, India.
authors.forename: Vineeta
authors.initials: V
authors.lastname: Shobha
authors.affiliation: Department of Pulmonary Medicine, St John's Medical College Hospital, Bengaluru, Karnataka, India.
authors.forename: George
authors.initials: G
authors.lastname: D'souza
keywords: Diffusing capacity of the lungs for carbon monoxide (DLCO); forced expiratory volume at 1 s; forced vital capacity; metacarpo phalangeal; mixed connective tissue disorder; systemic sclerosis