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Respiratory involvement in IgG4-related Mikulicz's disease.
Mod Rheumatol 2012; 22(1):31-9MR

Abstract

'Immunoglobulin G4 (IgG4)-related disease' is a new clinical concept of multi-organ diseases, with Mikulicz's disease (MD) being a clinical phenotype of IgG4-related disease. To clarify the clinical characteristics of respiratory involvement associated with IgG4-related MD, we retrospectively assessed 25 patients with MD, 11 (44%) of whom had allergic symptoms, and 7 (28%) of whom complained of respiratory problems. Thirteen patients (52%) presented with pulmonary and/or mediastinal lesions (P-MD) on chest computed tomography (CT), and 11 (44%) had lesions limited to the lacrimal and/or salivary glands (L-MD). Mean serum total protein, IgG, and IgG4 concentrations were significantly higher and CH50 was significantly lower in the P-MD than in the L-MD group. Immune complex was present only in the P-MD group. Chest CT images showed bronchial wall thickening, consolidation, nodule(s), interlobular thickening, ground glass opacity, pleural thickening/effusion, and mediastinal lymphadenopathy. Five of seven patients who underwent histological examination of the lungs had abundant IgG4-positive plasma cell infiltrates (IgG4/IgG-positive plasma cells >40%), but the other two did not. These findings suggest that respiratory lesions are not rare in patients with IgG4-related MD, and that they present with various manifestations. IgG4-related MD should be differentiated from similar diseases, such as sarcoidosis, bronchial asthma, Sjögren's syndrome, and malignant lymphoma.

Authors+Show Affiliations

Department of Internal Medicine (1), University of Toyama, 2630 Sugitani, Toyama, Toyama 930-0194, Japan. shoko-tym@umin.ac.jpNo 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 availableNo affiliation info availableNo affiliation info available

Pub Type(s)

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

Language

eng

PubMed ID

21811890

Citation

Matsui, Shoko, et al. "Respiratory Involvement in IgG4-related Mikulicz's Disease." Modern Rheumatology, vol. 22, no. 1, 2012, pp. 31-9.
Matsui S, Taki H, Shinoda K, et al. Respiratory involvement in IgG4-related Mikulicz's disease. Mod Rheumatol. 2012;22(1):31-9.
Matsui, S., Taki, H., Shinoda, K., Suzuki, K., Hayashi, R., Tobe, K., ... Ishizawa, S. (2012). Respiratory involvement in IgG4-related Mikulicz's disease. Modern Rheumatology, 22(1), pp. 31-9. doi:10.1007/s10165-011-0504-x.
Matsui S, et al. Respiratory Involvement in IgG4-related Mikulicz's Disease. Mod Rheumatol. 2012;22(1):31-9. PubMed PMID: 21811890.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Respiratory involvement in IgG4-related Mikulicz's disease. AU - Matsui,Shoko, AU - Taki,Hirofumi, AU - Shinoda,Koichiro, AU - Suzuki,Kensuke, AU - Hayashi,Ryuji, AU - Tobe,Kazuyuki, AU - Tokimitsu,Yoshiharu, AU - Ishida,Masayuki, AU - Fushiki,Hiroaki, AU - Seto,Hikaru, AU - Fukuoka,Junya, AU - Ishizawa,Shin, Y1 - 2011/08/03/ PY - 2011/06/15/received PY - 2011/07/12/accepted PY - 2011/8/4/entrez PY - 2011/8/4/pubmed PY - 2012/6/6/medline SP - 31 EP - 9 JF - Modern rheumatology JO - Mod Rheumatol VL - 22 IS - 1 N2 - 'Immunoglobulin G4 (IgG4)-related disease' is a new clinical concept of multi-organ diseases, with Mikulicz's disease (MD) being a clinical phenotype of IgG4-related disease. To clarify the clinical characteristics of respiratory involvement associated with IgG4-related MD, we retrospectively assessed 25 patients with MD, 11 (44%) of whom had allergic symptoms, and 7 (28%) of whom complained of respiratory problems. Thirteen patients (52%) presented with pulmonary and/or mediastinal lesions (P-MD) on chest computed tomography (CT), and 11 (44%) had lesions limited to the lacrimal and/or salivary glands (L-MD). Mean serum total protein, IgG, and IgG4 concentrations were significantly higher and CH50 was significantly lower in the P-MD than in the L-MD group. Immune complex was present only in the P-MD group. Chest CT images showed bronchial wall thickening, consolidation, nodule(s), interlobular thickening, ground glass opacity, pleural thickening/effusion, and mediastinal lymphadenopathy. Five of seven patients who underwent histological examination of the lungs had abundant IgG4-positive plasma cell infiltrates (IgG4/IgG-positive plasma cells >40%), but the other two did not. These findings suggest that respiratory lesions are not rare in patients with IgG4-related MD, and that they present with various manifestations. IgG4-related MD should be differentiated from similar diseases, such as sarcoidosis, bronchial asthma, Sjögren's syndrome, and malignant lymphoma. SN - 1439-7609 UR - https://www.unboundmedicine.com/medline/citation/21811890/Respiratory_involvement_in_IgG4_related_Mikulicz's_disease_ L2 - https://dx.doi.org/10.1007/s10165-011-0504-x DB - PRIME DP - Unbound Medicine ER -