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Interstitial lung disease in primary Sjögren syndrome.
Chest. 2006 Nov; 130(5):1489-95.Chest

Abstract

BACKGROUND

Primary Sjögren syndrome (pSS) has been associated with various histologic patterns of interstitial lung disease (ILD).

METHODS

We retrospectively identified 18 patients with pSS and suspected ILD who underwent lung biopsies (14 surgical biopsies and 9 bronchoscopic biopsies) at our institution during a 13-year period from 1992 through 2004. Histopathologic findings were analyzed and correlated with radiologic features and outcome.

RESULTS

Median age was 62 years (range, 34 to 78 years), and 15 patients (83%) were women. Most patients presented with dyspnea and cough. Chest radiographs demonstrated bilateral infiltrates, and high-resolution CT revealed abnormalities of various types including ground-glass, consolidation, reticular, and nodular opacities. The major histopathologic patterns included nonspecific interstitial pneumonia (NSIP) [five patients], organizing pneumonia (OP) [four patients], usual interstitial pneumonia (UIP) [three patients], lymphocytic interstitial pneumonia (three patients), primary pulmonary lymphoma (two patients), and diffuse interstitial amyloidosis (one patient). In four patients (three with OP and one with amyloidosis), the diagnosis was established on transbronchial biopsy results. Treatment commonly included prednisone with or without another immunosuppressive agent. During the follow-up period (median, 38 months), most patients improved or remained stable except three patients with UIP, one patient with NSIP, and one patient with amyloidosis. Seven patients (39%) died, including three deaths from acute exacerbation of interstitial pneumonia.

CONCLUSIONS

A variety of histologic patterns can be seen in patients with pSS-associated ILD. Those with UIP tended to have progression of lung disease. Death from acute exacerbation of interstitial pneumonia may occur in patients with pSS-associated ILD.

Authors+Show Affiliations

Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN 55905, USA.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

17099028

Citation

Parambil, Joseph G., et al. "Interstitial Lung Disease in Primary Sjögren Syndrome." Chest, vol. 130, no. 5, 2006, pp. 1489-95.
Parambil JG, Myers JL, Lindell RM, et al. Interstitial lung disease in primary Sjögren syndrome. Chest. 2006;130(5):1489-95.
Parambil, J. G., Myers, J. L., Lindell, R. M., Matteson, E. L., & Ryu, J. H. (2006). Interstitial lung disease in primary Sjögren syndrome. Chest, 130(5), 1489-95.
Parambil JG, et al. Interstitial Lung Disease in Primary Sjögren Syndrome. Chest. 2006;130(5):1489-95. PubMed PMID: 17099028.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Interstitial lung disease in primary Sjögren syndrome. AU - Parambil,Joseph G, AU - Myers,Jeffrey L, AU - Lindell,Rebecca M, AU - Matteson,Eric L, AU - Ryu,Jay H, PY - 2006/11/14/pubmed PY - 2006/12/15/medline PY - 2006/11/14/entrez SP - 1489 EP - 95 JF - Chest JO - Chest VL - 130 IS - 5 N2 - BACKGROUND: Primary Sjögren syndrome (pSS) has been associated with various histologic patterns of interstitial lung disease (ILD). METHODS: We retrospectively identified 18 patients with pSS and suspected ILD who underwent lung biopsies (14 surgical biopsies and 9 bronchoscopic biopsies) at our institution during a 13-year period from 1992 through 2004. Histopathologic findings were analyzed and correlated with radiologic features and outcome. RESULTS: Median age was 62 years (range, 34 to 78 years), and 15 patients (83%) were women. Most patients presented with dyspnea and cough. Chest radiographs demonstrated bilateral infiltrates, and high-resolution CT revealed abnormalities of various types including ground-glass, consolidation, reticular, and nodular opacities. The major histopathologic patterns included nonspecific interstitial pneumonia (NSIP) [five patients], organizing pneumonia (OP) [four patients], usual interstitial pneumonia (UIP) [three patients], lymphocytic interstitial pneumonia (three patients), primary pulmonary lymphoma (two patients), and diffuse interstitial amyloidosis (one patient). In four patients (three with OP and one with amyloidosis), the diagnosis was established on transbronchial biopsy results. Treatment commonly included prednisone with or without another immunosuppressive agent. During the follow-up period (median, 38 months), most patients improved or remained stable except three patients with UIP, one patient with NSIP, and one patient with amyloidosis. Seven patients (39%) died, including three deaths from acute exacerbation of interstitial pneumonia. CONCLUSIONS: A variety of histologic patterns can be seen in patients with pSS-associated ILD. Those with UIP tended to have progression of lung disease. Death from acute exacerbation of interstitial pneumonia may occur in patients with pSS-associated ILD. SN - 0012-3692 UR - https://www.unboundmedicine.com/medline/citation/17099028/Interstitial_lung_disease_in_primary_Sjögren_syndrome_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0012-3692(15)37327-X DB - PRIME DP - Unbound Medicine ER -