Arthritis & Rheumatism, Volume 60,
October 2009 Abstract Supplement

The 2009 ACR/ARHP Annual Scientific Meeting
Philadelphia October 16-21, 2009.


A Nucleolar-Pattern ANA in Patients with Idiopathic Interstitial Pneumonia Suggests Underlying Connective Tissue Disease

Fischer,  Aryeh, Swigris,  Jeffrey J., du Bois,  Roland M., Gillis,  Joann Z., Cohen,  Marc D., Meehan,  Richard T., Brown,  Kevin K.

Background:

Interstitial lung disease (ILD) comprises a diverse group of disorders characterized histologically by varying degrees of inflammation and fibrosis. Although ILD may have no identifiable etiology, (i.e. idiopathic interstitial pneumonia [IIP]), ILD is a common finding in patients with known connective tissue disease (CTD) or can be the forme fruste presentation of CTD. Recent data suggest that, CTD-related ILD has a more favorable prognosis than IIP, arguing for the careful evaluation of patients presenting with an idiopathic ILD in an attempt to identify underlying CTD.

Purpose:

To examine the significance of a nucleolar-pattern ANA in patients presenting with idiopathic ILD, and specifically, to describe the clinical characteristics of 15 patients presenting with IIP and a nucleolar-pattern ANA over a 12 month period at a tertiary ILD referral center.

Method:

Retrospective cohort of 21 patients presenting to a tertiary referral center for evaluation of IIP and found to have a nucleolar-pattern ANA. All patients underwent comprehensive ILD evaluation that included consultation with a board certified rheumatologist. The diagnoses of systemic sclerosis (SSc) and undifferentiated connective tissue disease (UCTD) were rendered by the treating physicians and confirmed on retrospective review based on current or proposed criteria.

Results:

Based on collaborative pulmonary and rheumatology evaluations, 17 (81%) were diagnosed with SSc and 4 (19%) with UCTD. As a result of the connective tissue disease (CTD) classification, most patients (81%) were treated with medication regimens containing a cytotoxic agent.

 N=21
Median age (range)66 (35–86)
Female gender10 (48%)
Smoking history12 (57%)
Duration of dyspnea in months (range)12 (1–60)
Median ANA titer (range)1:640 (80–5120)
Anti-Scl-703 (14%)
Other antibodies2 anti-Ro, 1 with combined PM-Scl, RF, CCP, dsDNA
Arthritis7 (33%)
Sclerodactyly5 (24%)
Telangiectasia12 (57%)
Raynaud's13 (62%)
Digital edema8 (38%)
Esophageal dysmotility (N=18)14 (67%)
FVC%59 (30–105)
DLCO%43 (15–82)
ILD pattern by HRCT12 NSIP (57%), 7 UIP (33%), 2 NSIP/UIP (10%)
Surgical lung biopsy (N=6)2 NSIP, 2 UIP, 1 NSIP+OP, 1 OP

Conclusion:

Cross-specialty collaboration with rheumatology is helpful to detect occult forms of CTD-related ILD. The presence of a nucleolar-pattern ANA in patients with IIP suggests underlying CTD – and SSc in particular – and should prompt rheumatologic evaluation. Clinicians involved in the evaluation of patients with ILD should ensure that ANA testing includes pattern determination in addition to titer.

More precise ILD classification will allow for studies that determine optimal therapy and prognosis based on specific ILD type.

To cite this abstract, please use the following information:
Fischer, Aryeh, Swigris, Jeffrey J., du Bois, Roland M., Gillis, Joann Z., Cohen, Marc D., Meehan, Richard T., et al; A Nucleolar-Pattern ANA in Patients with Idiopathic Interstitial Pneumonia Suggests Underlying Connective Tissue Disease [abstract]. Arthritis Rheum 2009;60 Suppl 10 :787
DOI: 10.1002/art.25867

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