SELECTED ARTICLES FROM THE RECENT LITERATURE 2006

12/28/06

Pulmonary involvement in systemic vasculitides

Summary
Background – Pulmonary involvement occurs with variable frequency in different types of systemic vasculitis (SV).

Findings – The occurrence and characteristics of pulmonary involvement in SV were reviewed by Manganelli et al of the Univ of Parma in Italy. Lung disease is a very common, important feature of SV associated with positive anti-neutrophil cytoplasm antibody (ANCA) tests such as Wegener’s Granulomatosis (WG), Churg-Strauss Syndrome (CSS), and microscopic polyangiitis (MPA).

In WG, involvement of upper and/or lower respiratory tract occurs in almost all cases with solitary or multiple nodules in the lungs, sometimes necrotic as the most common chest X-ray picture. In CSS, almost all patients have pre-existent/current asthma. Patchy, sometimes transient, pulmonary infiltrates are the most common radiographic abnormality. In MPA, diffuse alveolar capillaritis is the most common pulmonary manifestation, sometimes manifest as frank hemoptysis but also presenting as bilateral alveolar infiltrates and progressive anemia without apparent hemoptysis. In giant cell arteritis, the most common pulmonary manifestation is a persistent cough.

In contrast, pulmonary involvement is very unusual in classic polyarteritis nodosa (PN). Kawasaki disease, Henoch-Schonlein Purpura, and cryoglobulinemic vasculitis.

Reference
Clin Exp Rheumatol 2006;24:S48-59

Editor's Comments
It was generally thought for many years that pulmonary involvement was rare in SV. Such an impression was likely because the only SV condition recognized at that time in the past was PN in which pulmonary involvement was very unusual, even rare. This infrequent pulmonary involvement in PN may be because the vascular involvement in PN is usually in the relatively high pressure areas of the systemic arterial circulation. We now know that SV may involve small vessels, even capillaries, including those in the lung.

 

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