Journal List > J Korean Rheum Assoc > v.14(1) > 1003598

Seo, Kim, Kwok, Ju, Kim, Yoon, Kim, and Park: Extraglandular Manifestations and Autoantibodies of Korean Patients with Primary Sjogren's Syndrome

Abstract

Objective:

To investigate the extraglandular manifestations and serological features of Korean patients with primary Sjogren's syndrome (pSS).

Methods:

Clinical and laboratory data of 125 patients with pSS attending rheumatology clinic of Kangnam St. Mary's hospital, with a mean followup period of 64.8 months, were assessed retrospectively.

Results:

Arthralgia was the most common extraglandular manifestation of pSS. Other extraglandular manifestations, such as myalgia, peripheral neuropathy, Raynaud's phenomenon, and hypothyroidism were frequently presented, but lymphoma was rare manifestation in Korean patients with pSS. Some extraglandular manifestations of pSS were associated with hypocomple- mentemia and antibodies against Ro, and La, and antinuclear antibody.

Conclusion:

The clinical manifestations and extraglandular manifestations of Korean patients with pSS patients were similar to other studies except low prevalence of lymphoma. Positivity of anti-Ro/SS-A, anti-La/SS-B antibodies and hypocomplementemia were closely associated with extraglandular manifestations in patients with pSS.

REFERENCES

1). Fox RI., Howell FV., Bone RC., Michelson P. Primary Sjogren's syndrome: proposed criteria for classification. Arthritis Rheum. 1984. 14:77–105.
2). Kruize AA., Hene RJ., Vander HA., Boudeutsch C., de Wilde PCM., van Bijsterveld OP, et al. Long term follow up of patients with Sjogren's syndrome. Arthritis Rheum. 1996. 39:297–303.
3). Fox RI., Howell FV., Bone RC., Michelson P. Primary Sjogren's syndrome: clinical immunopathologic feature. Semin Arthritis Rheum. 1984. 14:77–105.
4). Alexander EL. Neurologic disease in Sjogren's syndrome: mononuclear inflammatory vasculopathy affecting central/peripheral nervous system and muscle. Rheum Dis Clin North Am. 1993. 19:869–908.
crossref
5). Amussen K., Andersen V., Bendixen G., Schiodt M. A new model for classification of disease modification in primary Sjogren's syndrome: evaluation in a retrospective long term study. J Int Med. 1996. 239:475–82.
6). Fox RI. Sjogren's syndrome. Lancet. 2005. 366:321–31.
7). Morgan WS., Castleman B. A clinicopathologic study of Mickuliz's disease. Am J Pathol. 1953. 29:471–504.
8). Fox RI., Saito I. Criteria for diagnosis of Sjogren's syndrome. Rheum Dis Clin North Am. 1994. 20:391–407.
crossref
9). Bloch KJ., Buchanan WW., Wohl MJ., Bunim JJ. Sjogren's syndrome: a clinical, pathological and serological study of 62 cases. Medicine (Baltimore). 1956. 44:187–231.
10). Vitali C., Bombardieri S., Moutsopoulos HM., Balestrieri G., Bencivelli W., Bernstein RM, et al. Preliminary criteria for the classification of Sjogren's syndrome. Results of a prospective concerted action supported by the European Community. Arthritis Rheum. 1993. 36:340–7.
11). Fox RI., Robinson C., Curd J., Michelson P., Bone R., Howell FV. First international symposium on Sjogren's syndrome: suggested criteria for classification. Scand J Rheumatol. 1986. 562:28–30.
12). Vitali C., Moutsopoulos HM., Bombardieri S. The European Community Study Group on diagnostic criteria for Sjogren's syndrome: sensitivity and specificity of tests for ocular and oral involvement in Sjogren's syndrome. Ann Rheum Dis. 1994. 53:637–47.
crossref
13). Fox RI. Sjogren's syndrome: progress and controversies. Med Clin North Am. 1997. 17:441–4.
14). Vitali C., Bombadien S., Jonsson R. Classification criteria for Sjogren's syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis. 2002. 61:554–8.
crossref
15). Vitali C. Classification criteria for Sjogren's syndrome. Ann Rheum Dis. 2003. 62:94–5.
crossref
16). Zhao Y., Zheng WJ., Zhou W., Zeng XF., Zhang FC., Dong Y, et al. The evaluation of the international classification criteria (2002) for primary Sjogren's syndrome in Chinese patients. APLAR J Rheumatol. 2005. 8:184–7.
crossref
17). 이충원: 이원기: 이지수: 이찬회: 송창호: 서창희등. Sjogren 증후군의임상적고찰. 대한류마티스학회지. 1997. 4:139–46.
18). Alamanos Y., Tsifetaki N., Voulgari PV., Venetsanopoulou AI., Siozos C., Drosos AA. Epidemiology of primary Sjogren's syndrome in north-west Greece, 1982-2003. Rheumatoloty. 2006. 45:187–91.
crossref
19). Skopouli FN., Dafni U., Ioannidis JP., Moutsopoulos HM. Clinical evolution, and morbidity and mortality of primary Sjogren's syndrome. Semin Arthritis Rheum. 2000. 29:296–304.
20). Talalx N. Sjogren's syndrome: historical overview and clinical spectrum of disease. Rheum Dis Clin North Am. 1992. 18:507–15.
21). Provost TT., Watson R. Cutaneous manifestations of Sjogren's syndrome. Rheum Dis Clin North Am. 1992. 18:609–16.
22). Lindvall B., Bengtsson A., Ernerudh J., Eriksson P. Subclinical myositis is common in primary Sjogren's syndrome and is not related to muscle pain. J Rheumatol. 2002. 29:717–25.
23). 최승원: 정현철: 김종철: 강병성: 김영민. 급성횡단척수염이동반된일차성쇼그렌증후군1예. 대한류마티스학회지. 2006. 13:86–90.
24). Andronopoulos AP., Lagos G., Drosos AA., Moutsopoulos HM. Neurological involvement in primary Sjogren's syndrome: a preliminary report. J Autoimmun. 1989. 2:485–8.
25). D'Arbonneau F., Ansart S., Le Berre R., Dueymes M., Youinou P., Pennec YL. Thyroid dysfunction in primary Sjogren's syndrome: a long-term followup study. Arthritis Rheum. 2003. 49:804–9.
26). Hansen BU., Ericsson UB., Henricsson V., Larsson A., Manthorpe R., Warfvinge G. Autoimmune thyroiditis and primary Sjogren's syndrome: clinical and laboratory evidence of the coexistence of the two diseases. Clin Exp Rheumatol. 1991. 9:137–41.
27). Perez B., Kraus A., Lopez G., Cifuentes M., Alarcon-Segovia D. Autoimmune thyroid disease in primary Sjogren's syndrome. Am J Med. 1995. 99:480–4.
28). Ramos-Casals M., Garcia-Carrasco M., Cervera R., Gaya J., Halperin I., Ubieto I, et al. Thyroid disease in primary Sjogren's syndrome. Study in a series of 160 patients. Medicine. 2000. 79:103–8.
29). Kohriyama K., Katayama Y., Tsurusako Y. Relationship between primary Sjogren's syndrome and autoimmune thyroid disease. Nippon Rinsho. 1999. 57:1878–81.
30). 김윤아: 이신석: 정해권. 하시모토갑상선염에동반된쇼그렌증후군1예. 대한류마티스학회지. 2002. 9:73–7.
31). Skopouli FN., Barbatis C., Moutscopulos HM. Liver involvement in primary Sjogren's syndrome. Br J Rheumatol. 1994. 33:745–8.
32). Tzioufas AG., Boumba DS., Skopouli FN., Moutsopoulos HM. Mixed monoclonal cryoglobulinemia and monoclonal rheumatoid factor cross-reactive idiotypes as predictive factor for the development of lymphoma in primary Sjogren's syndrome. Arthritis Rheum. 1996. 39:767–72.
33). Fox RI., Pearson G., vaughan JH. Detection of Epstein-Barr virus associated antigens and DNA in salivary gland biopsies from patients with Sjogren's syndrome. J Immunol. 1986. 137:3162–8.
34). Nardi N., Brito-Zeron P., Ramos-Casals M., Aguilo S., Cervera R., Ingelmo M, et al. Circulating auto-antibodies against nuclear and non-nuclear antigens in primary Sjogren's syndrome. Clin Rheumatol. 2006. 25:341–6.
35). Shah F., Rapini RP., Arnett FC., Warner NB., Smith CA. Association of labial salivary gland histopathology with clinical and serologic features of connective tissue diseases. Arthritis Rheum. 1990. 33:1682–7.
crossref
36). Moutsopoulos HM., Zerva LV. Anti-Ro (SS-A)/La (SSB) antibodies and Sjogren's syndrome. Clin Rheumatol. 1990. 9(Suppl 1):123–30.
37). Ramos-Casals M., Brito-Zeron P., Yague J., Akasbi M., Bartista R., Ruano M, et al. Hypocomplementaemia as an immunological marker of morbidity and mortality in patient with primary Sjogren's syndrome. Rheu- matoloty. 2005. 44:89–94.

Table 1.
Patients characteristics and clinical manifestations of primary Sjogren's syndrome
Number of patients (%) Mean ± SD (range)
Age (year) 48.60 ±11.54
(17-83)
Sex (male : female) 4 : 121
(3.2 : 96.8)
Followup (year) 5.34 ±4.52
(0.7-25.7)
Ocular symptom 122/125 (97.6)
Positive response of Schimer's I test 114/125 (91.2)
Dry mouth symptom 123/125 (98.4)
Parotid gland swelling 8/125 (6.4)
Positive response of salivary gland scan 109/116 (93.96)
Histopathology of minor salivary gland 27/34 (75)

SD: standard deviation

Table 2.
Extraglandular manifestations in primary Sjogren's syndrome
Number of patients (%)
Arthralgia/Arthritis 82/125 (65.6)
Myalgia 46/125 (36.8)
Raynaud's phenomenon 30/125 (24.0)
Peripheral neuralgia 29/125 (23.2)
Dry skin (itching sense) 24/125 (17.6)
Hypothyroidism 16/125 (12.8)
Alopecia 15/125 (12.0)
Central nervous system diseas 8/125 (6.4)
Liver involvement 7/125 (5.6)
Skin purpura 7/125 (5.6)
Lung nodule 6/125 (4.8)
Interstitial lung disease 6/125 (4.8)
Vasculitis 5/125 (4.0)
Hyperthyroidism 4/125 (2.7)
Renal tubular acidosis 2/125 (1.6)
Lymphoma 2/125 (1.6)
Table 3.
Autoantibodies in primary Sjogren's syndrome
Autoantibody Number of patients (%)
Antinuclear antibody 101/125 (80.8)
Rheumatoid factor 99/125 (79.2)
Anti-Ro/SS-A antibody 90/125 (72.0)
Anti-La/SS-B antibody 38/125 (30.4)
Anti-CCP antibody 5/125 (4.0)
Table 4.
Correlation with clinical manifestation
ANA RF Anti-Ro/SS-A Anti-La/SS-B Hypo C3 Hypo C4
Arthralgia NS p< 0.05∗ NS NS NS NS
Peripheral neurralgia NS NS NS p< 0.05∗ NS NS
Raynaud's phenomenon NS NS NS NS NS NS
Arthritis NS NS p=0.091∗ p=0.071∗ NS NS
Hypothyroidism NS NS NS NS NS p< 0.05∗
Liver involvement NS NS p< 0.05∗ p< 0.05∗ NS p< 0.05∗
Skin purpura NS NS p< 0.05∗ p< 0.05∗ NS NS
Interstitial lung disease NS NS NS NS NS p< 0.05∗
Leukopenia NS NS p< 0.05∗ p< 0.05∗ p< 0.05∗ NS
Thrombocytopenia p<0.05∗ NS NS NS NS p< 0.05∗

ANA: antinuclear antibody, RF: rheumatoid factor, C3: third component of complement, C4: fourth component of complement, NS: not significant. ∗p values by Chi-squared test or Fisher's exact test

TOOLS
Similar articles