Interstitial lung disease in primary Sjögren syndrome

Joseph G. Parambil, Jeffrey L. Myers, Rebecca M. Lindell, Eric Lawrence Matteson, Jay H Ryu

Research output: Contribution to journalArticle

214 Citations (Scopus)

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 histopatliologic 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.

Original languageEnglish (US)
Pages (from-to)1489-1495
Number of pages7
JournalChest
Volume130
Issue number5
DOIs
StatePublished - Nov 2006

Fingerprint

Interstitial Lung Diseases
Idiopathic Pulmonary Fibrosis
Amyloidosis
Biopsy
Pneumonia
Lung
Immunosuppressive Agents
Prednisone
Cough

Keywords

  • Interstitial lung disease
  • Interstitial pneumonia
  • Lung biopsy
  • Pulmonary fibrosis
  • Sjögren syndrome

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

Cite this

Interstitial lung disease in primary Sjögren syndrome. / Parambil, Joseph G.; Myers, Jeffrey L.; Lindell, Rebecca M.; Matteson, Eric Lawrence; Ryu, Jay H.

In: Chest, Vol. 130, No. 5, 11.2006, p. 1489-1495.

Research output: Contribution to journalArticle

Parambil, Joseph G. ; Myers, Jeffrey L. ; Lindell, Rebecca M. ; Matteson, Eric Lawrence ; Ryu, Jay H. / Interstitial lung disease in primary Sjögren syndrome. In: Chest. 2006 ; Vol. 130, No. 5. pp. 1489-1495.
@article{caedc07ede514ad399c17fee111d4684,
title = "Interstitial lung disease in primary Sj{\"o}gren syndrome",
abstract = "Background: Primary Sj{\"o}gren 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 histopatliologic 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.",
keywords = "Interstitial lung disease, Interstitial pneumonia, Lung biopsy, Pulmonary fibrosis, Sj{\"o}gren syndrome",
author = "Parambil, {Joseph G.} and Myers, {Jeffrey L.} and Lindell, {Rebecca M.} and Matteson, {Eric Lawrence} and Ryu, {Jay H}",
year = "2006",
month = "11",
doi = "10.1378/chest.130.5.1489",
language = "English (US)",
volume = "130",
pages = "1489--1495",
journal = "Chest",
issn = "0012-3692",
publisher = "American College of Chest Physicians",
number = "5",

}

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 Lawrence

AU - Ryu, Jay H

PY - 2006/11

Y1 - 2006/11

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 histopatliologic 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.

AB - 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 histopatliologic 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.

KW - Interstitial lung disease

KW - Interstitial pneumonia

KW - Lung biopsy

KW - Pulmonary fibrosis

KW - Sjögren syndrome

UR - http://www.scopus.com/inward/record.url?scp=33751215251&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=33751215251&partnerID=8YFLogxK

U2 - 10.1378/chest.130.5.1489

DO - 10.1378/chest.130.5.1489

M3 - Article

C2 - 17099028

AN - SCOPUS:33751215251

VL - 130

SP - 1489

EP - 1495

JO - Chest

JF - Chest

SN - 0012-3692

IS - 5

ER -