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Pulmonary manifestations of primary Sjogren's syndrome: a clinical, radiologic, and pathologic study.
Am J Respir Crit Care Med. 2005 Mar 15; 171(6):632-8.AJ

Abstract

RATIONALE

Clinicopathologic pulmonary manifestations associated with primary Sjogren's syndrome 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 primary Sjogren's syndrome in the light of NSIP, and to analyze prognosis of the disease.

METHODS

On the basis of 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 and amyloid and malignant lymphoma were seen less frequently. HRCT-pathologic correlation resulted in a 94% positive predictive value of CT-NSIP pattern for pathologic diagnosis of NSIP, whereas the diagnostic value of HRCT was low (15%) with an HRCT pattern other than NSIP, data that may influence the decision to biopsy. The 5-year survival rate was 84% overall and 83% in patients with NSIP. Multivariate analysis on all patients showed that low Pa(O(2)) (p = 0.02) and presence of microscopic honeycombing (p = 0.04) were independently associated with survival. Patients with NSIP showed lower vital capacity (mean +/- SD: 68.5 +/- 16.6%pred) than patients without NSIP (92.5 +/- 18.6%pred; p < 0.001).

CONCLUSION

Among a diversity of pulmonary lesions in primary Sjogren's syndrome, NSIP was the commonest histologic pattern and had a favorable prognosis.

Authors+Show Affiliations

Department of Respiratory Medicine, Kyoto University Hospital, 54 Shogoin-kawaharacho, Sakyo, Kyoto, Japan.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo 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

15579729

Citation

Ito, Isao, et al. "Pulmonary Manifestations of Primary Sjogren's Syndrome: a Clinical, Radiologic, and Pathologic Study." American Journal of Respiratory and Critical Care Medicine, vol. 171, no. 6, 2005, pp. 632-8.
Ito I, Nagai S, Kitaichi M, et al. Pulmonary manifestations of primary Sjogren's syndrome: a clinical, radiologic, and pathologic study. Am J Respir Crit Care Med. 2005;171(6):632-8.
Ito, I., Nagai, S., Kitaichi, M., Nicholson, A. G., Johkoh, T., Noma, S., Kim, D. S., Handa, T., Izumi, T., & Mishima, M. (2005). Pulmonary manifestations of primary Sjogren's syndrome: a clinical, radiologic, and pathologic study. American Journal of Respiratory and Critical Care Medicine, 171(6), 632-8.
Ito I, et al. Pulmonary Manifestations of Primary Sjogren's Syndrome: a Clinical, Radiologic, and Pathologic Study. Am J Respir Crit Care Med. 2005 Mar 15;171(6):632-8. PubMed PMID: 15579729.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Pulmonary manifestations of primary Sjogren's syndrome: a clinical, radiologic, and pathologic study. AU - Ito,Isao, AU - Nagai,Sonoko, AU - Kitaichi,Masanori, AU - Nicholson,Andrew G, AU - Johkoh,Takeshi, AU - Noma,Satoshi, AU - Kim,Dong Soon, AU - Handa,Tomohiro, AU - Izumi,Takateru, AU - Mishima,Michiaki, Y1 - 2004/12/03/ PY - 2004/12/8/pubmed PY - 2005/5/13/medline PY - 2004/12/8/entrez SP - 632 EP - 8 JF - American journal of respiratory and critical care medicine JO - Am J Respir Crit Care Med VL - 171 IS - 6 N2 - RATIONALE: Clinicopathologic pulmonary manifestations associated with primary Sjogren's syndrome 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 primary Sjogren's syndrome in the light of NSIP, and to analyze prognosis of the disease. METHODS: On the basis of 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 and amyloid and malignant lymphoma were seen less frequently. HRCT-pathologic correlation resulted in a 94% positive predictive value of CT-NSIP pattern for pathologic diagnosis of NSIP, whereas the diagnostic value of HRCT was low (15%) with an HRCT pattern other than NSIP, data that may influence the decision to biopsy. The 5-year survival rate was 84% overall and 83% in patients with NSIP. Multivariate analysis on all patients showed that low Pa(O(2)) (p = 0.02) and presence of microscopic honeycombing (p = 0.04) were independently associated with survival. Patients with NSIP showed lower vital capacity (mean +/- SD: 68.5 +/- 16.6%pred) than patients without NSIP (92.5 +/- 18.6%pred; p < 0.001). CONCLUSION: Among a diversity of pulmonary lesions in primary Sjogren's syndrome, NSIP was the commonest histologic pattern and had a favorable prognosis. SN - 1073-449X UR - https://www.unboundmedicine.com/medline/citation/15579729/Pulmonary_manifestations_of_primary_Sjogren's_syndrome:_a_clinical_radiologic_and_pathologic_study_ DB - PRIME DP - Unbound Medicine ER -