title: A case of primary Sjögren's syndrome complicated with inflammatory myopathy and interstitial lung disease.
abstract: We experienced a case of a 63-year-old woman with primary Sjögren's syndrome (pSS) complicated with inflammatory myopathy and interstitial lung disease (ILD). She had suffered from morning stiffness and dry mouth for 1 year without being medically examined. A chest CT scan demonstrated ground glass opacity and a reticular shadow in the lower lung field. A diagnosis of SS was made based on positive findings from Schirmer's test, sialography of the parotid gland, a labial salivary gland biopsy and the presence of anti-SS-A antibody. Musculoskeletal symptoms were absent; however, the elevation of creatine kinase (CK) as well as magnetic resonance imaging (MRI)-proven inflammatory change of bilateral muscles of the thigh was evident. Histological examination of the thigh revealed diameter variation, degeneration of muscle fibers and inflammatory cell infiltration in the perivascular area, corresponding to the inflammatory myopathy of pSS. Oral prednisolone 30 mg/day was introduced, and serum CK rapidly decreased within 2 weeks. ILD also responded well to prednisolone without relapse. These clinical outcomes are consistent with extraglandular organ involvement of pSS.
authors.affiliation: Unit of Translational Medicine, Department of Immunology and Rheumatology, Graduate School of Biomedical Sciences, Nagasaki University, 1-7-1 Sakamoto, Nagasaki 852-8501, Japan. tkoga@nagasaki-u.ac.jp
authors.forename: Tomohiro
authors.initials: T
authors.lastname: Koga
authors.forename: Yukiko
authors.initials: Y
authors.lastname: Kouhisa
authors.forename: Hideki
authors.initials: H
authors.lastname: Nakamura
authors.forename: Akinari
authors.initials: A
authors.lastname: Mizokami
authors.forename: Masakatsu
authors.initials: M
authors.lastname: Motomura
authors.forename: Atsushi
authors.initials: A
authors.lastname: Kawakami
authors.forename: Katsumi
authors.initials: K
authors.lastname: Eguchi
mesh_terms: D005260:Female; D006801:Humans; D017563:Lung Diseases, Interstitial / Q000150:complications* / Q000473:pathology; D008875:Middle Aged; D009220:Myositis / Q000150:complications* / Q000473:pathology; D012470:Salivary Glands, Minor / Q000473:pathology; D012859:Sjogren's Syndrome / Q000150:complications* / Q000473:pathology
references.citation: J Intern Med. 1996 Jun;239(6):475-82
references.pmid: 8656140
references.citation: J Rheumatol. 2002 Apr;29(4):717-25
references.pmid: 11950012
references.citation: Lancet. 2005 Jul 23-29;366(9482):321-31
references.pmid: 16039337
references.citation: Ann Rheum Dis. 2002 Jun;61(6):554-8
references.pmid: 12006334
references.citation: Autoimmunity. 2005 Aug;38(5):383-92
references.pmid: 16227154
references.citation: J Rheumatol. 1994 Apr;21(4):649-53
references.pmid: 8035388
references.citation: Ann Rheum Dis. 1985 Jul;44(7):455-61
references.pmid: 4026406
references.citation: Am J Med. 1986 Jan;80(1):23-31
references.pmid: 2417478
references.citation: Medicine (Baltimore). 1965 May;44:187-231
references.pmid: 14315274
references.citation: Mod Rheumatol. 2003 Mar;13(1):57-61
references.pmid: 24387117
references.citation: N Engl J Med. 1975 Feb 20;292(8):403-7
references.pmid: 1089199