AB0650 Lung Manifestations of Diffuse Cutaneous Systemic Sclerosis Patients from India

Abstract

BackgroundLung manifestations in diffuse cutaneous systemic sclerosis (dCSS) are well known. Data on these manifestations in Indian patients are scant or unknown. We aim to present the various lung manifestations in patients with dCSS.ObjectivesTo study the clinical and radiological characteristics of pulmonary symptoms such as cough, dyspnea, or hemoptysis, in patients, previously diagnosed with dCSS, in accordance with ACR 2013 classification criteria.MethodsThis was a cross-sectional, point of care study, done at the Government General and Chest Hospital, Hyderabad, Telangana, India. The study was conducted from April 2019 to March 2020 and patients with a confirmed diagnosis of dCSS were included. Patients with known heart disease, diabetes mellitus, cancer, obesity, acute respiratory infections, and HIV infection were excluded.7 patients were eligible for inclusion. All patients gave their consent to participate in this study. The study was also approved by the institutional ethics committee. All imaging studies were read and confirmed by consultant radiologists at our hospital.ResultsAll patients received a confirmed diagnosis of dCSS less than 2 years from the time of presentation to our hospital. The shortest duration from diagnosis of dCSS to first manifestation of pulmonary symptoms was 6 months.All patients were females. The mean age was 52 ± 14.6 years. All patients had chronic cough, lasting ≥ 1 month. 6 out of 7 patients had exertional dyspnea lasting for ≥ 1 month. None of the 7 patients reported hemoptysis, wheezing, or having a fever. Only 1 patient had anemia, and none had leukocytosis. All patients had their chest x-rays reported as normal.6/7 (85.7%) patients had subpleural reticular shadowing limited to the lower lobes on HRCT chest, consistent with usual interstitial pneumonia (UIP) pattern. 3/6 (50%) patients with reticular shadowing also had honeycombing suggesting advanced/severe UIP. None of the patients had traction bronchiectasis. 2/6 patients (33.3%) with UIP, also had non subpleural ground glass opacities (GGOs) in the middle and lower zones, suggesting an overlap between UIP and NSIP (non-specific interstitial pneumonia).Findings on ECG, 2D-Echo, spirometry and bronchoalveolar lavage (BAL) are summarized in Table 1.Table 1.S.No.Age (years)SexDuration from Diagnosis to first pulmonary manifestationPulmonary symptomsCBCChest X-rayHRCT ChestECG2D-EchoSpirometryBALPatient 144F16 monthsCough + Dyspnea – 2 monthsNormalNormalAdvanced UIPNormalNormalRestrictiveNormalPatient 256F18 monthsCough + Dyspnea – 3 monthsNormalNormalUIPNormalNormalNormalNeutrophilicPatient 361F12 monthsCough + Dyspnea – 1 monthNormalNormalAdvanced UIPNormalRVSP = 48 mm HgRestrictiveLymphocyticPatient 470F21 monthsCough + Dyspnea – 2 monthsNormalNormalNormalNormalNormalNormalLymphocyticPatient 524F18 monthsCough + Dyspnea – 2 monthsNormalNormalAdvanced UIP + NSIP (overlap)NormalRVSP = 51 mm HgRestrictiveNeutrophilicPatient 655F12 monthsCough + Dyspnea – 1 monthNormalNormalUIPNormalNormalNormalNot PerformedPatient 754F6 monthsDyspnea – 1 monthAnemia +NormalUIP + NSIP (overlap)NormalNormalRestrictiveNeutrophilicNotes in the column ‘BAL’ indicate the predominant cell type noted on cytology.RVSP = Right Ventricular Systolic PressureConclusionAlthough limited by a small sample size, UIP appeared to be the most common lung pathology in patients with dCSS.The duration from diagnosis of dCSS to first manifestation of pulmonary symptoms is much shorter in comparison to limited cutaneous systemic sclerosis [1]PAH is commoner with limited cutaneous systemic sclerosis than with dCSS. [2]BAL was neither useful for diagnosis nor required in preparing a therapeutic plan for these patients.References[1]Steen VD. Clinical manifestations of systemic sclerosis. InSeminars in cutaneous medicine and surgery 1998 Mar 1 (Vol. 17, No. 1, pp. 48-54).[2]Mathai S, Kroening K, Hummers L, Zaiman A, Girgis R, Hassoun P. Differences between limited and diffuse systemic sclerosis-related pulmonary arterial hypertension.Disclosure of InterestsNone declared