Primary Sjögren Syndrome in Han Chinese

Abstract

The epidemiological characteristics of Sjögren syndrome (SS) are significantly varied in different countries. We conducted the present study to survey the epidemiological characteristics of primary SS in China. We recruited 483 primary SS patients from 16 Chinese medical centers nationwide from January 2009 to November 2011 and assessed salivary and lacrimal gland dysfunction, organ involvement, and autoimmunity in these patients. The cohort included 456 women and 27 men (ratio, 17:1; mean age at onset, 42 ± 11 years; median age at diagnosis, 49 years; range, 41–56 years). Male patients showed a lower frequency of xerophthalmia (37.0% vs 60.7%) and a higher frequency of arthritis (40.7% vs 16.4%). Young-onset patients showed a higher frequency of low C3 levels (57.7% vs 36.3%) and pancytopenia (22.2% vs 8.8%). Patients with systemic involvement had a higher frequency of immunoglobulin A (IgA) (39.4% vs 22.5%) and immunoglobulin M (IgM) (12.4% vs 37.9%). Patients with pulmonary involvement had a higher parotid enlargement (21.4% vs 10.2%), purpura (12.1% vs 5.7%) and higher anti-La/SS-B (61.7% vs 41.8%), immunoglobulin G (IgG) (80.7% vs 64.6%) and IgA (48.9% vs 30.6%) levels. Patients with anti-Ro/SSA antibodies had more frequent exocrine gland symptoms and some extraglandular symptoms and immunological alterations. Compared with previous studies performed in other countries, SS patients in China showed particular clinical manifestation, systemic involvement, and immunological alterations.

Primary Sjögren's syndrome in Asia: Yin and Yang?

“…Interstitial lung disease, in particular, was found to be present in 22%, 20% and 18.6% of patients as per three different studies from China . Pulmonary hypertension was seen in 12.5% (44/352 patients) and 10.6% (29/274 patients), respectively, in two studies . Pericardial effusion and atrioventricular block were described by Zhao et al .…”

“…In another recent study from China, 143 (29.6%) had lung involvement, defined by presence of persistent cough, dyspnea along with lung infiltrates on imaging or abnormal pulmonary function tests . Interstitial lung disease, in particular, was found to be present in 22%, 20% and 18.6% of patients as per three different studies from China . Pulmonary hypertension was seen in 12.5% (44/352 patients) and 10.6% (29/274 patients), respectively, in two studies .…”

“…Based on computed tomography scan findings, lung involvement was detected in 221/523 (42.3%) patients in a recent study . In another recent study from China, 143 (29.6%) had lung involvement, defined by presence of persistent cough, dyspnea along with lung infiltrates on imaging or abnormal pulmonary function tests . Interstitial lung disease, in particular, was found to be present in 22%, 20% and 18.6% of patients as per three different studies from China .…”

“…Similarly, a retrospective study from China by Li et al . reported dry eyes and dry mouth at first presentation in 50.2% and 31.4%, respectively; while another recent study reported sicca symptoms in 73.3% of patients at initial presentation . Underreporting of sicca symptoms may also be related to patients’ perceptions, understanding and socio‐economic status.…”

“…In these studies, only patients with sicca symptoms were evaluated for pSS as the concept of occult pSS had not evolved by then. From late 2000 onward, case series reported from China and India seemed to suggest that the disease in Asia has a varied spectrum and it is quite different from that in the West . This attains significance as Asia accounts for nearly 60% of the world population and possibly a proportionate global burden of the disease.…”