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THU0396 Igg4-Related Disease or Primary Sjogren Syndrome? Organ-Specific Involvement and Immunological Profile Are Clues in Differentiating Two Diseases with A Common Clinical Presentation
  1. P. Brito Zeron1,
  2. S. Retamozo1,
  3. H. Gheitasi1,
  4. A. Bové1,
  5. B. Kostov2,
  6. A. Sisό-Almirall2,
  7. M. Ramos-Casals1,
  8. J.H. Stone3
  9. on behalf of GEAS-SEMI Study Group
  1. 1Department of Autoimmune Diseases, ICMiD, Laboratory of Autoimmune Diseases Josep Font, Hospital Clinic, IDIBAPS
  2. 2Primary Care Research Group, IDIBAPS, Centre d'Assistència Primària ABS Les Corts, CAPSE, Barcelona, Spain
  3. 3Harvard Medical School and Department of Medicine (Division of Rheumatology, Allergy, and Immunology), Massachusetts General Hospital, Boston, MA, United States


Background IgG4-related disease and primary Sjögren syndrome (SS) share clinical presentations (sicca features, enlarged exocrine glands) and systemic organ involvement, with histopathological demonstration of IgG4+ infiltrative cells being the key differential feature.

Objectives To compare epidemiological, clinical and analytical features in patients with primary SS and IgG4-related disease.

Methods The GEAS-SS multicenter registry, founded in 2005, includes 921 consecutive Spanish patients fulfilling the 2002 primary SS classification criteria. IgG4-related disease patients fulfilling the 2012 criteria (Umehara et al) were collected by systematic review (2000-2013, 23 studies including 662 patients).

Results Primary SS patients had a similar mean age at diagnosis (54 vs 60 years, p>0.05) and were more frequently female (94% vs 40%, p<0.001) than IgG4-related disease patients. Sicca symptoms were more frequent in primary SS (oral dryness 95 vs 32%, p<0.001, ocular dryness 95 vs 24%, p<0.001). Globally, glandular enlargement was higher in IgG4-related disease patients (75% vs 35%, p>0.001), with a clear predominance of submaxillar (71% vs 15%, p<0.001) and lachrymal gland (44% vs 5%, p<0.001) involvement, while parotid gland enlargement was more frequent (34% vs 27%, p=0.04) in primary SS. Systemic involvement differed clearly, with a higher frequency of skin (15% vs 3%), joint (13% vs 0%), liver (15% vs 2%) and peripheral nerve (10% vs 1%) involvement in primary SS patients (p<0.001 for all comparisons) and a higher frequency of pancreatic (23% vs 2%), renal (18% vs 4%), retroperitoneal (13% vs 0%), biliary tract (10% vs 0%) involvement and adenopathies (44% vs 10%) in IgG4-related disease patients (all comparisons p<0.001). Primary SS patients had a higher frequency of the main immunological markers (ANA 90% vs 28%, RF 57% vs 24%, Ro/SS-A 73% vs 2.5%, La/SS-B 46% vs 0%, all comparisons p<0.001) except hypocomplementemia (12% vs 46%, p<0.001, while 91.2% of IgG4-related disease patients and only 4.7% of primary SS patients had raised serum IgG4 levels (>135 mg/dL) (p<0.001), with a mean of 789,07 mg/dL vs 42,12 mg/dL (p<0.001), and a % ratio IgG4/total IgG of 34.5% vs 2.1% (p<0.001). Mean IgG4+ cells per HPF in available histopathological samples were 104 (range 13-229) in IgG4-related disease patients and <5 in primary SS patients.

Conclusions Although IgG4-related disease and primary SS share similar clinical presentations, significant differences in epidemiological features, organ-specific involvement and immunological profiles may aid early clinical diagnosis, with raised serum IgG4 levels and tissular infiltration by IgG4+ cells being key differentiating features. A clinical score including the parameters identified could aid differentiation between IgG4-related disease and primary SS in daily paractice.

Disclosure of Interest : None declared

DOI 10.1136/annrheumdis-2014-eular.5099

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