title: Unique characteristics of systemic sclerosis sine scleroderma-associated interstitial lung disease.
abstract: OBJECTIVE
  To describe the characteristics of systemic sclerosis sine scleroderma (ssSSc)-associated interstitial lung disease (ILD) presenting as idiopathic interstitial pneumonia (IIP).
  
  
  METHODS
  Retrospective review of six patients with ssSSc-associated ILD diagnosed after referral for evaluation of IIP.
  
  
  RESULTS
  All patients were white, their mean age was 56 years (range, 37 to 86), and gender was evenly divided. Sclerodactyly, skin thickening, and digital edema were absent in all patients. All patients had scattered telangiectasia, and four patients had Raynaud phenomenon with abnormal nailfold capillaroscopy findings. All described gastroesophageal reflux, and three patients had esophageal dysmotility by esophagography. All had restrictive pulmonary physiology and a reduced diffusion capacity. High-resolution CT revealed nonspecific interstitial pneumonia (NSIP) or usual interstitial pneumonia (UIP) radiographic patterns. Of the three patients who underwent surgical lung biopsy, two patients had NSIP and one patient had UIP pathologic patterns. Five patients had asymptomatic pericardial effusions and elevated pulmonary artery pressures by echocardiography. All patients had nucleolar-staining anti-nuclear antibodies (ANAs), and one patient was anti-Scl-70 positive. All five anti-Scl-70-negative patients were anti-Th/To positive, and the anti-Scl-70-positive patient was anti-Th/To negative.
  
  
  CONCLUSIONS
  In the presentation of an IIP, the presence of a nucleolar-staining ANA, telangiectasia, Raynaud phenomenon with abnormal capillaroscopy findings, gastroesophageal reflux, or pericardial disease suggests underlying systemic sclerosis. These findings should aid clinicians in the evaluation and treatment of patients with otherwise undefined ILD.
authors.affiliation: Division of Rheumatology, National Jewish Medical and Research Center, 1400 Jackson St, Denver, CO 80206, USA. fischera@njc.org
authors.forename: Aryeh
authors.initials: A
authors.lastname: Fischer
authors.forename: Richard T
authors.initials: RT
authors.lastname: Meehan
authors.forename: Carol A
authors.initials: CA
authors.lastname: Feghali-Bostwick
authors.forename: Sterling G
authors.initials: SG
authors.lastname: West
authors.forename: Kevin K
authors.initials: KK
authors.lastname: Brown
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000918:Antibody Specificity / Q000276:immunology; D001323:Autoantibodies / Q000097:blood; D001706:Biopsy; D005260:Female; D006801:Humans; D008168:Lung / Q000473:pathology; D017563:Lung Diseases, Interstitial / Q000175:diagnosis* / Q000276:immunology; D008297:Male; D020678:Microscopic Angioscopy; D008875:Middle Aged; D012189:Retrospective Studies; D012595:Scleroderma, Systemic / Q000175:diagnosis* / Q000276:immunology; D036542:Tomography, Spiral Computed
chemical_list: D001323:Autoantibodies