Sjogren syndrome is an autoimmune disease that mainly affects the exocrine glands. It is clinically characterized by keratoconjunctivitis sicca and xerostomia (Goransson et al., 2006).
See 200400 for association of Sjogren syndrome with achalasia in sisters. ... Sjogren syndrome is an autoimmune disease that mainly affects the exocrine glands. It is clinically characterized by keratoconjunctivitis sicca and xerostomia (Goransson et al., 2006). See 200400 for association of Sjogren syndrome with achalasia in sisters.
Lichtenfeld et al. (1976) noted familial occurrence. This probably represents the same sort of familial occurrence as is seen with systemic lupus erythematosus (SLE; 152700) and other autoimmune disorders. Moutsopoulos et al. (1979) used the term primary or ... Lichtenfeld et al. (1976) noted familial occurrence. This probably represents the same sort of familial occurrence as is seen with systemic lupus erythematosus (SLE; 152700) and other autoimmune disorders. Moutsopoulos et al. (1979) used the term primary or secondary sicca syndrome depending, respectively, on whether or not the disorder was associated with another autoimmune disease. They found a strong association with HLA-Dw3 and HLA-Dw4. Expression of HLA-DR antigen (see 142860) and intracellular adhesion molecule-1 (ICAM1; 147840) in human conjunctival epithelium is upregulated in patients with dry eyes associated with Sjogren syndrome. Tsubota et al. (1999) reported that this upregulation in Sjogren syndrome patients may be controlled by interferon-gamma (IFNG; 147570) through the activation of transcription factor NFKB (nuclear factor kappa-B; see 164011). Sade de Paiva et al. (2003) found that patients with keratitis sicca had irregular corneal surfaces when examined with computerized videokeratoscopy (CVK). The CVK regularity indices had both high sensitivity and specificity and had the potential to be used as objective diagnostic indices for dry eye, as well as a means to evaluate the severity of the disease. In a study of 16 Italian patients with Vogt-Koyanagi-Harada syndrome (an autoimmune-mediated meningoencephalitis with panuveitis) and 16 controls with diffuse non-VKH uveitis, Pivetti Pezzi et al. (2004) found that the incidence of keratoconjunctivitis sicca was higher in the patients with VKH syndrome than in the controls. Two patients satisfied the criteria for Sjogren syndrome and 2 others had scintigraphy indicative of salivary gland involvement. The authors concluded that an association between these 2 autoimmune disorders was suggested by the low incidence of VKH syndrome in Italy and might be related to HLA DR4. Goransson et al. (2006) detected clinically significant peripheral neuropathy in 17 (27%) of 62 patients with primary Sjogren syndrome. Nerve conduction studies were abnormal in 34 (55%) patients; 19 (31%) had motor neuropathy, 8 (13%) had sensory neuropathy, and 7 (11%) had sensorimotor neuropathy. The predominant neuropathic process was demyelinating.
Gottenberg et al. (2003) compared 149 patients fulfilling the American-European Consensus Group criteria for Sjogren syndrome to 222 controls and confirmed the association of Sjogren syndrome with HLA alleles DRB1*03 (see HLA-DRB1; 142857) and DQB1*02 (see HLA-DQB1; 604305). ... Gottenberg et al. (2003) compared 149 patients fulfilling the American-European Consensus Group criteria for Sjogren syndrome to 222 controls and confirmed the association of Sjogren syndrome with HLA alleles DRB1*03 (see HLA-DRB1; 142857) and DQB1*02 (see HLA-DQB1; 604305). They found, however, that the association was restricted to patients with anti-SSA (see 109092 and 600063) and/or anti-SSB (see 109090) antibodies. The absence of a difference in disease severity between patients with anti-SSA and those with anti-SSA and anti-SSB antibodies, together with a high frequency of HLA-DRB1*03 in the latter group, suggested to the authors that HLA alleles predispose to autoantibody secretion but are not associated with clinical outcome.