Ten-year observation of patients with primary Sjögren’s syndrome: Initial presenting characteristics and the associated outcomes

Masako Tsukamoto, Katsuya Suzuki, Tsutomu Takeuchi

Research output: Contribution to journalArticle

Abstract

Aim/Introduction: Primary Sjögren's syndrome (pSS) is a prototypical systemic autoimmune disease that manifests with various signs and symptoms. Although some studies have examined these manifestations over the long-term course of the disease, the association between initial clinical and immunological factors and subsequent long-term manifestations has not been fully elucidated. The aim of this study is to identify initial clinical and immunological factors associated with subsequent manifestations in patients with pSS. Method: A retrospective review was performed in pSS patients who were followed up over a 10-year period in our department. Clinical and immunological data, including serum immunoglobulin (Ig) and autoantibody levels, were collected and statistically analyzed. Result: We analyzed 91 patients who were followed up in our department. The proportion of patients with extraglandular involvement decreased from 90% to 73%, while eight patients developed extraglandular organ involvement. Extraglandular involvement at 10 years more frequently occurred in patients with hyper-IgG than those without hyper-IgG at initial testing (P < 0.01). Extraglandular organ involvement at 10 years more frequently occurred in rheumatoid factor (RF)-positive patients at the time of SS diagnosis (P < 0.05). Malignancy occurred in 9% of patients. Age, lower CH50 and thrombocytopenia were significantly associated with malignancy. Extraglandular organ involvement was associated with the presence of hyper-IgG and RF positivity (P < 0.01 and P < 0.05). Conclusion: Our study identified important initial clinical and immunological factors associated with subsequent manifestations in patients with pSS over a long follow-up period. pSS patients with RF and hyper-IgG at diagnosis may have a higher risk of subsequent extraglandular involvement.

Original languageEnglish
JournalInternational Journal of Rheumatic Diseases
DOIs
Publication statusAccepted/In press - 2018 Jan 1

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Observation
Rheumatoid Factor
Immunologic Factors
Immunoglobulin G
Thrombocytopenia
Autoantibodies
Autoimmune Diseases
Signs and Symptoms
Immunoglobulins
Neoplasms
Serum

Keywords

  • extraglandular involvement
  • long-term outcome
  • malignancy
  • rheumatoid arthritis
  • Sjögren’s syndrome

ASJC Scopus subject areas

  • Rheumatology

Cite this

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title = "Ten-year observation of patients with primary Sj{\"o}gren’s syndrome: Initial presenting characteristics and the associated outcomes",
abstract = "Aim/Introduction: Primary Sj{\"o}gren's syndrome (pSS) is a prototypical systemic autoimmune disease that manifests with various signs and symptoms. Although some studies have examined these manifestations over the long-term course of the disease, the association between initial clinical and immunological factors and subsequent long-term manifestations has not been fully elucidated. The aim of this study is to identify initial clinical and immunological factors associated with subsequent manifestations in patients with pSS. Method: A retrospective review was performed in pSS patients who were followed up over a 10-year period in our department. Clinical and immunological data, including serum immunoglobulin (Ig) and autoantibody levels, were collected and statistically analyzed. Result: We analyzed 91 patients who were followed up in our department. The proportion of patients with extraglandular involvement decreased from 90{\%} to 73{\%}, while eight patients developed extraglandular organ involvement. Extraglandular involvement at 10 years more frequently occurred in patients with hyper-IgG than those without hyper-IgG at initial testing (P < 0.01). Extraglandular organ involvement at 10 years more frequently occurred in rheumatoid factor (RF)-positive patients at the time of SS diagnosis (P < 0.05). Malignancy occurred in 9{\%} of patients. Age, lower CH50 and thrombocytopenia were significantly associated with malignancy. Extraglandular organ involvement was associated with the presence of hyper-IgG and RF positivity (P < 0.01 and P < 0.05). Conclusion: Our study identified important initial clinical and immunological factors associated with subsequent manifestations in patients with pSS over a long follow-up period. pSS patients with RF and hyper-IgG at diagnosis may have a higher risk of subsequent extraglandular involvement.",
keywords = "extraglandular involvement, long-term outcome, malignancy, rheumatoid arthritis, Sj{\"o}gren’s syndrome",
author = "Masako Tsukamoto and Katsuya Suzuki and Tsutomu Takeuchi",
year = "2018",
month = "1",
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doi = "10.1111/1756-185X.13464",
language = "English",
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T2 - Initial presenting characteristics and the associated outcomes

AU - Tsukamoto, Masako

AU - Suzuki, Katsuya

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N2 - Aim/Introduction: Primary Sjögren's syndrome (pSS) is a prototypical systemic autoimmune disease that manifests with various signs and symptoms. Although some studies have examined these manifestations over the long-term course of the disease, the association between initial clinical and immunological factors and subsequent long-term manifestations has not been fully elucidated. The aim of this study is to identify initial clinical and immunological factors associated with subsequent manifestations in patients with pSS. Method: A retrospective review was performed in pSS patients who were followed up over a 10-year period in our department. Clinical and immunological data, including serum immunoglobulin (Ig) and autoantibody levels, were collected and statistically analyzed. Result: We analyzed 91 patients who were followed up in our department. The proportion of patients with extraglandular involvement decreased from 90% to 73%, while eight patients developed extraglandular organ involvement. Extraglandular involvement at 10 years more frequently occurred in patients with hyper-IgG than those without hyper-IgG at initial testing (P < 0.01). Extraglandular organ involvement at 10 years more frequently occurred in rheumatoid factor (RF)-positive patients at the time of SS diagnosis (P < 0.05). Malignancy occurred in 9% of patients. Age, lower CH50 and thrombocytopenia were significantly associated with malignancy. Extraglandular organ involvement was associated with the presence of hyper-IgG and RF positivity (P < 0.01 and P < 0.05). Conclusion: Our study identified important initial clinical and immunological factors associated with subsequent manifestations in patients with pSS over a long follow-up period. pSS patients with RF and hyper-IgG at diagnosis may have a higher risk of subsequent extraglandular involvement.

AB - Aim/Introduction: Primary Sjögren's syndrome (pSS) is a prototypical systemic autoimmune disease that manifests with various signs and symptoms. Although some studies have examined these manifestations over the long-term course of the disease, the association between initial clinical and immunological factors and subsequent long-term manifestations has not been fully elucidated. The aim of this study is to identify initial clinical and immunological factors associated with subsequent manifestations in patients with pSS. Method: A retrospective review was performed in pSS patients who were followed up over a 10-year period in our department. Clinical and immunological data, including serum immunoglobulin (Ig) and autoantibody levels, were collected and statistically analyzed. Result: We analyzed 91 patients who were followed up in our department. The proportion of patients with extraglandular involvement decreased from 90% to 73%, while eight patients developed extraglandular organ involvement. Extraglandular involvement at 10 years more frequently occurred in patients with hyper-IgG than those without hyper-IgG at initial testing (P < 0.01). Extraglandular organ involvement at 10 years more frequently occurred in rheumatoid factor (RF)-positive patients at the time of SS diagnosis (P < 0.05). Malignancy occurred in 9% of patients. Age, lower CH50 and thrombocytopenia were significantly associated with malignancy. Extraglandular organ involvement was associated with the presence of hyper-IgG and RF positivity (P < 0.01 and P < 0.05). Conclusion: Our study identified important initial clinical and immunological factors associated with subsequent manifestations in patients with pSS over a long follow-up period. pSS patients with RF and hyper-IgG at diagnosis may have a higher risk of subsequent extraglandular involvement.

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