SESSION TITLE: Interstitial Lung Disease Case Report Posters
SESSION TYPE: Affiliate Case Report Poster
PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM
INTRODUCTION: Interstitial Lung disease (ILD) is generally a late feature in patients with progressive systemic sclerosis (PSS). We report a rare case of 30 year old female patient who presented with acute respiratory distress syndrome, noted to have diffuse alveolar damage (DAD) on tranbronchial lung biopsy and elevated serum Scl-70 antibodies with no clinical evidence of Scleroderma
CASE PRESENTATION: 30 year old non-smoker female patient with presented with progressively worsening shortness of breath, productive cough, and fever of three weeks duration. Physical exam revealed bilateral crackles on lung exam. Computerized tomography (CT) of the chest showed widespread nodular densities with mediastinal and hilar lymphadenopathy. Complete infective work-up was negative, and immunological studies revealed only positive Scl-70 and ANA reflex antibodies. Transbronchial biopsy was consistent with acute lung injury, with features of organizing DAD. Hospital course was complicated by worsening hypoxic respiratory failure requiring mechanical ventilation. She was empirically treated with high dose corticosteroid therapy with diagnosis of acute interstitial pneumonitis (AIP) with success. Four week follow-up revealed near radiological resolution of her infiltrates with remarkable clinical improvement
DISCUSSION: Pulmonary parenchymal abnormalities have been noted in PSS even in the absence of classic cutaneous involvement. Pleural thickening and fibrosis, interstitial fibrosis, and peripheral lower lobe honeycomb changes are less commonly noted. DAD and acute AIP that is not associated with immune complex deposition or infection has rarely been reported in association of scleroderma, although it is well known to occur with systemic lupus erythematosus (SLE), mixed CTD with SLE features, Rheumatoid arthritis, and polymyositis-dermatomyocitis. Scl-70 in many studies has been noted to be highly specific for scleroderma; however our patient has no clinical features of the disease at this time.
CONCLUSIONS: Dyspnea and pulmonary interstitial fibrosis may precede development of onset of skin and vascular changes in PSS. Open lung biopsy performed early in the course may not yield definitive diagnosis. A thorough clinical exam and continued clinical follow-up to look for nail fold abnormalities along with antinuclear body and esophagram study is essential to facilitate early diagnosis of PSS.
Reference #1: Parambil JG etal. Diffuse alveolar damage: uncommon manifestation of pulmonary involvement in patients with connective tissue disorders
DISCLOSURE: The following authors have nothing to disclose: Mohammed Moizuddin, Muhammed Imtiaz, Mohammed Nayeem, Ria Gripaldo
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