[Overlap of Sjögren-lupus erythematosus syndrome].
Patients with anti-SSA(Ro)-positive Sjögren's syndrome (SS)/lupus erythematosus (LE) overlap are a immunogenetically, serologically and clinically homogeneous group; what they have in common is an increased frequency of the HLA-DR3 phenotype, demonstrating SSA(Ro) antibody activity and the typical annular, polycyclic, erythematous lesions of subacute cutaneous LE. The sicca symptoms of SS may develop long after the cutaneous lesions of LE have been present, or vice versa, as well as vasculitic, purpuric, and Sweet's syndrome-like lesions. The elderly, predominantly female patients are at enhanced risk for pulmonary and neurological disease, but glomerulonephritis occurs infrequently. We present an 81-year-old, SSA(Ro) antibody-positive and HLA-DR3-positive woman with SS/LE overlap syndrome, who had cutaneous LE for 30 years, developing sicca symptoms, vasculitic and Sweet's syndrome-like skin lesions only years after the LE symptoms. Although there is still some discussion as to whether phenotypical SS/LE overlap represents a distinct disease or is secondary SS in SLE or systemic SS with cutaneous involvement, the SS/LE overlap syndrome seems well enough delineated to be considered as a disease entity with separate implications for therapy and prognosis. Its position in the spectrum of anti-SSA(Ro) antibody- and HLA-DR3-positive diseases and its relationship to SS and to anti-SSA(Ro) antibody-positive LE are discussed.
Trüeb
R M
RM
Dermatologische Klinik, Universitätsspital, Zürich.
Borelli
S
S
Schmid-Grendelmeier
P
P
Panizzon
R G
RG
Burg
G
G
D000368:Aged; D000369:Aged, 80 and over; D000974:Antibodies, Antinuclear / Q000097:blood; D001706:Biopsy; D003937:Diagnosis, Differential; D005260:Female; D015803:HLA-DR3 Antigen / Q000235:genetics; D006801:Humans; D008178:Lupus Erythematosus, Cutaneous / Q000175:diagnosis* / Q000235:genetics / Q000276:immunology; D010641:Phenotype; D012470:Salivary Glands, Minor / Q000473:pathology; D012859:Sjogren's Syndrome / Q000175:diagnosis* / Q000235:genetics / Q000276:immunology; D012867:Skin / Q000473:pathology; D016463:Sweet Syndrome / Q000175:diagnosis / Q000235:genetics / Q000276:immunology; D014657:Vasculitis / Q000175:diagnosis / Q000235:genetics / Q000276:immunology
D000974:Antibodies, Antinuclear; D015803:HLA-DR3 Antigen; C035356:SS-A antibodies
Sjögren-Lupus erythematodes-Uberlappungssyndrom.