Introduction: PLCH is a rare interstitial lung disease, linked to cigarette smoking and may be associated with respiratory failure and death. Limited UK data has been published and little knowledge exists of the diagnostic and treatment practices employed by UK physicians.
Aims and Objectives: Our study aims to characterise the epidemiological, clinical, histological, radiological and prognostic indicators in a UK cohort of patients with PLCH.
Methods: 112 cases from 53 centres (65 from the BTS British Orphan Lung Disease database and 47 new or previously unregistered). Consultants provided contact details; Patients were sent an information leaflet, consent form and questionnaire. Once consent obtained, consultants were sent a medical questionnaire. The patientsā€˜ GP provided current medication and medical history.
Results: Details on 95 patients (19 deceased, 7 lost to follow-up) received. Clinical information regarding 67 cases has been received; completed by the patient, consultant or both (31 males). Age at presentation 37.1 years (SD 14.4). Presenting symptoms: Dyspnoea 78%, cough 63%, constitutional symptoms 25%, pain 22% and pneumothorax 8%, Smoking status: Ex 71.7%, current 25.0% mean (SD) 19.9 (16.9) pack years. Cannabis use 9.6% Diagnosis: 93.8% patients had had an HRCT scan and 61% an open lung biopsy. Disease course: Symptoms resolved 23.3%, same 33.3% and 43.3% had slowly progressed. Treatment: None 56%, Steroids 29.6% immunosuppression/chemotherapy 26.5%. pleural surgery 18.7% and lung transplant 6%
Conclusions: This UK dataset indicates a high prevalence of smoking in our cohort and, despite advances in computed tomography a high percentage of patients are still diagnosed with an open lung biopsy.