SESSION TITLE: Interstitial Lung Disease Student/Resident Case Report Posters I
SESSION TYPE: Medical Student/Resident Case Report
PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM
INTRODUCTION: Antisynthetase Syndrome (ASS) is a lesser recognized Idiopathic Inflammatory Myopathy with nearly 89% showing Interstitial Lung Disease (ILD). However, in a small sub-population without evidence of myositis, the diagnosis may be critically delayed, hindering management of this rapidly progressive disease.
CASE PRESENTATION: Fifty-eight year old Caucasian male with type II diabetes mellitus and osteoarthritis presented with polyarticular arthritis involving bilateral knees, shoulders, wrists and hands; associated with severe dryness and cracking of skin. Pulmonary function tests (PFTs) done for progressive exertional dyspnea showed FEV1/FVC 76, FEV1 1.99 L (54%), TLC 76% DLCO 63% with 9% improvement (230 mL) to bronchodilators. He was treated with albuterol for presumed bronchial asthma, but progressively worsened after a brief response. At our center, examination revealed bilateral basal coarse crepitations and diffuse swelling of hand joints with leukocytosis 16,900/mm3 and elevated CPK 402 U/L. Septal thickening and honeycombing on CT scan and restrictive pattern on PFTs were consistent with ILD. Inflammatory arthritis, hyper-keratotic ‘mechanic’ hands and ILD prompted a diagnosis of ASS confirmed by serum Anti-Jo1 antibodies, despite the absence of myositis. Patient improved clinically with prednisone and methotrexate, and continues to be stable with non-progression of ILD.
DISCUSSION: Only 2-11% patients with ASS present with ILD in the absence of myositis and are diagnosed with Anti-Jo1 antibodies in the serum. Median interval of development of myositis after ILD is 6 months; however there are reports of up to 2 years. Anti-Jo1 antibody positive ILDs cause only modest serum CPK elevation that delays diagnosis in the absence of clinically evident myositis. These patients deteriorate faster in comparison to patients with myositis. However, once diagnosed, they have high steroid responsiveness which is postulated to be due to higher non-specific interstitial pneumonia without manifest alveolar damage. High resolution CT has replaced lung biopsy in establishing diagnosis. Steroids-resistant cases are treated with Azathioprine or Cyclophosphamide.
CONCLUSIONS: ILD with a suggestive clinical picture warrants a work-up for ASS despite the absence of myositis to ensure appropriate diagnosis and management.
Reference #1: Marie I, Josse S, Hatron PY, Dominique S, Hachulla E, Janvresse A, et al. Interstitial lung disease in anti-jo1 patients with antisynthetase syndrome. Arthritis Care Res (Hoboken). 2012 Nov 30. doi: 10.1002/acr.21895.
Reference #2: Plastiras SC, Soliotis FC, Vlachoyiannopoulos P, Tzelepis GE. Interstitial lung disease in a patient with antisynthetase syndrome and no myositis. Clin Rheumatol. 2007 Jan;26(1):108-11. Epub 2005 Nov 23.
Reference #3: Patel HC, Lauder NN. The Antisynthetase Syndrome. Am J Med. 2011 Sep:124(9):e3-4
DISCLOSURE: The following authors have nothing to disclose: Pranathi Sundaragiri, Saraschandra Vallabhajosyula, Jennifer Kanaan
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