Pulmonary Arterial Hypertension and Severe Pulmonary Fibrosis in Systemic Sclerosis Patients with a Nucleolar Antibody

VIRGINIA D. STEEN, MARY LUCAS, NOREEN FERTIG, and THOMAS A. MEDSGER Jr

ABSTRACT.

Objective. Pulmonary arterial hypertension (PAH) and severe pulmonary fibrosis (SPF) are the most common causes of death in scleroderma. Our study focuses on lung disease in patients with a nucleolar antibody in comparison to other scleroderma-specific autoantibodies.

Methods. Patients initially seen between 1972 and 1995 (and followed through 2004) with PAH [systolic pulmonary artery pressure (sPAP) > 50 mm Hg] or SPF [forced vital capacity (FVC%) < 55% predicted) were grouped by the presence of anticentromere antibody (ACA), an isolated antinucleolar antibody (ANoA), or an antitopoisomerase antibody-I (TOPO).

Results. Twenty percent of ACA, 23% of TOPO, and 32% of ANoA patients had severe lung disease (p < 0.005). In ANoA patients with PAH without severe fibrosis, the FVC was lower (71% predicted) than in ACA patients, suggesting they had some interstitial fibrosis. However, they had a higher FVC%/diffusing capacity for carbon monoxide (DLCO)% ratio than the ACA patients (2.4 vs 1.8). Pulmonary hypertension in TOPO patients was associated with a lower FVC%/DLCO% ratio and lower levels of PAP than either the PAH in ACA or ANoA patients.

Conclusion. Scleroderma-specific autoantibodies are associated with characteristic subgroups of lung disease. The ANoA patients have a unique mixture of PAH and SPF subgroups of lung disease. Scleroderma-specific autoantibodies and the FVC%/DLCO% ratio are helpful in determining whether a patient has PAH alone, PAH along with pulmonary fibrosis, or secondary PAH from chronic hypoxia with SPF. (First Release Oct 15 2007; J Rheumatol 2007;34:2230-5)

Key Indexing Terms:

SCLERODERMA
SYSTEMIC SCLEROSIS
PULMONARY FIBROSIS
PULMONARY HYPERTENSION
NUCLEOLAR ANTIBODIES
AUTOANTIBODIES

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From the Department of Medicine, Division of Rheumatology, Allergy and Immunology, Georgetown University, Washington, DC; and the Department of Medicine, Division of Rheumatology and Clinical Immunology, University of Pittsburgh, Pittsburgh, Pennsylvania, USA.

V.D. Steen, Professor of Medicine, Department of Medicine, Division of Rheumatology, Allergy and Immunology, Georgetown University; M. Lucas, RN, MSN, Research Associate; N. Fertig, BS, Research Assistant; T.A. Medsger Jr, MD, Professor of Medicine, Department of Medicine, Division of Rheumatology and Clinical Immunology, University of Pittsburgh.

Address reprint requests to Dr. V. Steen, Department of Medicine, Georgetown University, 3800 Reservoir Road, LL Kober Cogan, Washington, DC 20007.

Accepted for publication July 20, 2007.

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