Clinical and serologic study of sjögren's syndrome in patients with progressive systemic sclerosis

Osial, Thaddeus A.; Whiteside, Theresa L.; Buckingham, Robert B.; Singh, Gurmukh; Barnes, Eleanor; Pierce, John M.; Rodnan, Gerald P.

doi:10.1002/art.1780260408

Cited by 63 publications

(41 citation statements)

References 24 publications

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“…The reported prevalence of Sjögren's syndrome in SSc patients varies from 5 to 90%, depending on the criteria used, while with salivary gland biopsy 29% of SSc patients were described to have Sjögren's syndrome [35]. In the present study, Sjögren's syndrome was the most common CTD that overlapped with SSc, and the prevalence of this overlap syndrome was 12.6%.…”

Section: Discussionmentioning

confidence: 46%

Clinical features of 405 Japanese patients with systemic sclerosis

et al. 2011

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We aimed to clarify the clinical features of Japanese patients with systemic sclerosis (SSc), especially with reference to organ involvement and autoantibodies. A cohort of 405 patients with SSc who attended our institution from 1973 to 2008 was identified retrospectively. Data on clinical features, including autoantibodies, organ involvement, and overlap of other connective tissue diseases, were obtained by following the medical records until 2009. The percentage of male patients during or after 1990 was greater than that before 1990 (3.9 vs. 10.6%, respectively). Limited cutaneous SSc (lSSc) was twice as frequent as diffuse cutaneous SSc (dSSc). About half of the patients had lung involvement (50.4%), while only 3.2% had scleroderma renal crisis. Male gender was associated with lung involvement, and dSSc was associated with most organ involvements except for pulmonary arterial hypertension (PAH). Anti-Scl-70 antibody was associated with lung or heart involvement, while anti-U1-RNP antibody was only associated with PAH. Conversely, patients with anti-centromere antibody had less organ involvement. SSc-Sjögren overlap syndrome was related to lSSc, further overlapping systemic lupus erythematosus (SLE), and less lung or heart involvement. In conclusion, these results not only confirmed previous reports but revealed several other findings, such as the increased proportion of male patients in recent years and the relationships between clinical features.

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Section: Discussionmentioning

confidence: 46%

Clinical features of 405 Japanese patients with systemic sclerosis

et al. 2011

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“…Cutaneous xerosis is a frequent complaint in scleroderma patients, associated with the skin thickening and the atrophy of the sebaceous and sweat glands in the affected areas. Ocular and salivary dryness may also occur, as a result of localised glandular fibrosis or in association with Sjögren's syndrome [17][18][19], present in 8% of the patients in the current study. Scleroderma of the uterine cervix is very rare in SSc [20].…”

Section: Discussionmentioning

confidence: 99%

Gynaecologic History in Systemic Sclerosis

Sampaio-Barros

Sâmara

Neto

2000

Clinical Rheumatology

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The aim of the study was to analyse the gynaecologic history of 150 Brazilian patients with systemic sclerosis (SSc) by comparing the outcome of the pregnancies before and after disease onset and in the two clinical variants of SSc, as well as to assess the effects of the pregnancy on the progress of the disease. A retrospective analysis was carried out of 150 female SSc patients, more than 18 years old, who attended the outpatient clinic of the Unit of Rheumatology of the State University of Campinas. The patients were questioned about the number of pregnancies, deliveries (full-term infants, premature births and twins) and fetal deaths (spontaneous abortions and perinatal deaths). These data were subdivided into pregnancies before and after SSc onset. In those gestations started after disease onset the patients were questioned about the evolution of SSc during the pregnancy. The patients were also asked about dyspareunia and the age at menopause. Thirty-two patients (21 %) had never been pregnant, and only five of them were considered infertile. One hundred and eighteen patients (79%) had a total of 406 pregnancies, with an average of 3.4 per patient; there were 364 pregnancies before and 42 after SSc onset. There were 58 fetal deaths (14% of the pregnancies), 50 of these occurring before and eight after disease onset; 55 were spontaneous abortions and the other three were perinatal deaths. The fertility rate was higher in the limited SSc (3.6) than in the diffuse SSc patients (3.1), although the percentage of fetal deaths and the evolution of SSc during the pregnancy were similar in the two clinical variants. In the pregnancies that occurred after the onset of SSc, the clinical course remained stable in 72% of the cases, worsened in 14% and improved in 14%. Dyspareunia was mentioned by 49 patients (37% of those with an active sexual life). Menopause was reported by 72 patients, predominantly with limited SSc (61 patients). The fertility rate in the postmenopausal SSc patients was 3.9, similar to that observed in general postmenopausal population in Brazil. The analysis of the gynaecologic history in this series of SSc patients showed no increased risk in infertility or spontaneous abortions. The fertility rate in the two SSc clinical variants was higher than that observed in the local global population. Most of the patients who became pregnant after the onset of SSc showed no signs of worsening during the course of the disease.

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“…" Sicca symptoms also occur with increased frequency in patients with PSS (15). However, the characteristic feature of the salivary gland biopsy material from PSS patients is extensive fibrosis (50), which is similar to the histologic findings in other organs affected by PSS, and is in contrast to the persisting lymphoid infiltrates in subjects diagnosed as having SS (10). Recently, Matthews and coworkers (51) have demonstrated that there is a normal level of IgA in the saliva of PSS patients.…”

Section: Breadth Of the Spectrum Of Sjogren's Syndromementioning

confidence: 91%

Sjögren's syndrome. Proposed criteria for classification

Fox¹,

Robinson²,

Curd³

et al. 1986

Arthritis & Rheumatism

617

230

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The term “Sjögren's syndrome” (SS) is frequently used to describe the occurrence of keratocon‐junctivis sicca and xerostomia in association with an autoimmune disorder. However, well‐defined criteria for the classification of SS have not been established, and this diagnosis is being applied to a wide spectrum of conditions, ranging from clear “autoimmune” disease in some patients, to sicca complaints without evidence of a systemic immune process in elderly patients. Here, we review the clinical and laboratory features of patients referred for evaluation of sicca symptoms. In particular, we emphasize the need for care in choosing the site for minor salivary gland biopsy, and we describe the histologic features that aid in the evaluation of these biopsy specimens. In an attempt to identify a population of patients whose conditions might have a common etiopathogenesis and, thus, a common treatment program, we propose the following criteria for a diagnosis of SS: 1) objective evidence of keratoconjunctivis sicca, as documented by rose bengal or fluorescein dye staining; 2) objective evidence of diminished salivary gland flow; 3) minor salivary gland biopsy, obtained through normal mucosa, with the specimen containing at least 4 evaluable salivary gland lobules, and having an average of at least 2 foci/4 mm2; 4) evidence of a systemic autoimmune process, as manifested by the presence of autoantibodies, such as rheumatoid factor and/or anti‐nuclear antibody. The diagnosis of “definite SS” would be made when all 4 criteria are met; the diagnosis of “possible SS” would be made when 3 criteria are present. Specific exclusions for this diagnosis are preexisting lymphoma, graft‐versus‐host disease, sarcoidosis, and acquired immunodeficiency disease. These proposed criteria are more restrictive than are those currently used for diagnosing SS. Future multicenter trials will be required to determine whether these criteria are generally useful. Finally, we emphasize the rheumatologist's role in selecting safe, inexpensive methods of objectively monitoring sicca complaints (i.e., kerato‐conjunctivitis sicca and parotid salivary flow) and in evaluating the results of minor salivary gland biopsy.

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Clinical and serologic study of sjögren's syndrome in patients with progressive systemic sclerosis

Cited by 63 publications

References 24 publications

Clinical features of 405 Japanese patients with systemic sclerosis

Clinical features of 405 Japanese patients with systemic sclerosis

Gynaecologic History in Systemic Sclerosis

Sjögren's syndrome. Proposed criteria for classification

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