Obstructive Lung Diseases |

Pulmonary Fibrosis Preceding Skin Involvement in Scleroderma FREE TO VIEW

Sugeet Jagpal, MD; Anthony Scardella, MD
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Robert Wood Johnson Medical School, New Brunswick, NJ

Chest. 2013;144(4_MeetingAbstracts):664A. doi:10.1378/chest.1702421
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SESSION TITLE: Interstitial Lung Disease Case Report Posters I

SESSION TYPE: Affiliate Case Report Poster

PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM

INTRODUCTION: Scleroderma can present solely as advanced pulmonary fibrosis.

CASE PRESENTATION: A 44 year old African American woman was evaluated in the emergency department complaining of sudden dyspnea that began after using a cleaning product which contained hypochlorous acid, dodecyl sulfate, phosphoric acid, and sodium hydroxide. CT scan of the chest initially demonstrated patchy ground glass opacities and interlobular septal thickening. She was asked to see a pulmonologist and she presented to clinic with nonproductive cough, wheezing, and dyspnea with exertion. The working diagnosis at this time was reactive airways dysfunction syndrome secondary to a chemical exposure; however, pulmonary function tests as well as a repeat CT chest were ordered to further evaluate her symptoms (Image 1, Table 1). At a subsequent visit the patient reported resolution of her symptoms consistent with resolution of the groundglass opacities on CT chest. Her dyspnea, however, recurred several months later. Pulmonary function tests and chest imaging were repeated and demonstrated deterioration (Table 1, Image 1). The patient was referred for a surgical lung biopsy and pathology demonstrated a normal lung juxtaposed with fibrotic lung consistent with usual interstitial pneumonia. This prompted a serologic evaluation for collagen vascular disease. The ANA titer was diffusely positive (1:2560) and her Scl-70 IgG antibody was highly positive (>8). The patient was started on systemic steroids, referred to rheumatology, and scleroderma was confirmed. The patient was enrolled in a trial comparing the immunomodulatory agents mycophenolate and cyclophosphomide. Since diagnosis the patient has developed Raynaud’s phenomenon but does not have classic skin manifestations, digital ulcers, or gastrointestinal complaints.

DISCUSSION: In scleroderma, pulmonary involvement can include interstitial lung disease, pulmonary hypertension, aspiration pneumonia and alveolar hemorrhage (1). Pulmonary fibrosis can be present in up to 90% of patients with end stage scleroderma (1). Antitopoisomerase antibodies (Scl-70) are associated with increased risk of skin involvement, pulmonary fibrosis, and cardiac involvement (2). It is known that patients who initially present with only pulmonary involvement can progress to develop typical scleroderma (3).

CONCLUSIONS: Pulmonary fibrosis can be the initial manifestation of scleroderma, preceding skin involvement.

Reference #1: Hant, F. (2010). Pulmonary manisfestations of scleroderma and mixed connective tissue disease. Clinics in Chest Medicine, 433-439.

Reference #2: Hanke, K. (2009). Diagnostic value of anti-topoisomerase antibodies in a large multicentric cohort. Arthritis Research & Therapies, R28.

Reference #3: Lomeo, R. (1989). Progressive systemic sclerosis sine scleroderma presenting as pulmonary interstitial fibrosis. American Journal of Medicine, 525-527.

DISCLOSURE: The following authors have nothing to disclose: Sugeet Jagpal, Anthony Scardella

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