Published ahead of print on December 3, 2004, doi:10.1164/rccm.200403-417OC
Am. J. Respir. Crit. Care Med., Volume 171, Number 6, March 2005, 632-638
A more recent version of this article appeared on March 15, 2005
Submitted on March 28, 2004
Accepted on November 24, 2004
Pulmonary Manifestations of Primary Sjogren's Syndrome: A Clinical, Radiologic, and Pathologic Study
Isao Ito1, Sonoko Nagai1*, Masanori Kitaichi2, Andrew G Nicholson3, Takeshi Johkoh4, Satoshi Noma5, Dong Soon Kim6, Tomohiro Handa1, Takateru Izumi7, and Michiaki Mishima1
1 Department of Respiratory Medicine, Kyoto University Hospital, Kyoto, Japan,
2 Laboratory of Anatomic Pathology, Kyoto University Hospital, Kyoto, Japan,
3 Department of Histopathology, Royal Brompton Hospital, London, United Kingdom,
4 Department of Medical Physics and Radiology, Osaka University Graduate School of Medicine, Osaka, Japan,
5 Department of Radiology, Tenri Hospital, Nara, Japan,
6 Division of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan, College of Medicine, Seoul, Korea, Republic of,
7 Central Clinic in Kyoto, Kyoto, Japan
* To whom correspondence should be addressed. E-mail: nagai{at}kuhp.kyoto-u.ac.jp.
Rationale: Clinicopathologic pulmonary manifestations associated with primary Sjogren's syndrome (pSjS) have yet to be reviewed in a large series since the recognition of nonspecific interstitial pneumonia (NSIP) as a distinct histologic pattern.
Objectives: To determine clinical presentations, high-resolution computed tomographic (HRCT) and histologic findings of the lung disease associated with pSjS in the light of NSIP, and to analyze prognosis of the disease.
Methods: Based upon 33 cases (31 surgical lung biopsies and 2 autopsies) collected consecutively from multiple centers, we have retrospectively evaluated clinical, radiologic and pathologic manifestations of the disease. Prognostic factors were identified by univariate and multivariate analysis.
Measurements and Main Results: We found that NSIP was the most frequently seen histologic pattern (20 of 33 cases, 61%, 19 fibrosing and 1 cellular). Bronchiolar diseases, amyloid and malignant lymphoma were seen less frequently. HRCT-pathologic correlation resulted in a 94% positive predictive value of CT-NSIP pattern for pathological diagnosis of NSIP, whilst the diagnostic value of HRCT was low (15%) with a HRCT pattern other than NSIP, data that may influence the decision to biopsy. The 5-year survival was 84% overall and 83% in patients with NSIP. Multivariate analysis on all patients showed that low PaO2 (p=0.02) and presence of microscopic honeycombing (p=0.04) were independently associated with survival. NSIP patients showed lower vital capacity (mean±SD: 68.5±16.6%pred) than patients without NSIP (92.5±18.6), p<0.001.
Conclusions: Among a diversity of pulmonary lesions in pSjS, NSIP was the commonest histologic pattern and had a favorable prognosis.
Key words: nonspecific interstitial pneumonia, bronchiolitis, honeycombing, surgical lung biopsy, survival
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