Sjogrens Syndrome
- autoimmune disease
- Ab's to the Ro/SSA and La/SSB antigens,(which are also found in other autoimmune diseases) cryoglobulinemia, hypocomplementemia
- propensity to develop non-Hodgkin lymphoma
- often diagnosed during Ix of neuropathies, nephropathies, interstitial pneumonitis, hematologic abnormalities, lymphoproliferative changes
- mild to moderate disease sometimes with lack of salivary involvement and more extra-salivary presentation
Clinical
- dry eyes, mouth, skin & vagina
- fatigue
- muscle, joint pain
- salivary gland inflammation and swelling
- rashes exacerbated by UV exposure
Systemic Lupus Erythematosus (SLE)
- chronic autoimmune disease of unknown cause that can affect virtually any organ of the body.
- Immunologic abnormalities, esp of antinuclear antibodies (ANA).
- variable clinical features ranging from mild joint and skin involvement to life-threatening kidney, haematologic, or CNS involvement
- fatigue, fever, myalgia and weight changes (both LOW and weight gain with fluid shifts assoc with low Alb)
- Arthritis and arthralgias in over 90% - moderately painful, usually does not cause erosion, and rarely deforming
- muco-cutaneous changes - highly variable but butterfly rash on face = common, and photosensitive rashes Painless ulcers
- vasculitis including Raynaud's
- thrombo-embolic disorders - both arterial and venous circulation
- GI, resp, CNS, blood dyscrasias
Investigations
aside from routine bloods, which may be abnormal, other supportive tests include:
- Serum protein electrophoresis esp if hypergammaglobulinemia - suggestive of systemic inflammatory process
- ANA (ideally by indirect immunofluorescence testing) - usually +ve in all SLE patients at some time. If +ve should then test for - anti-dsDNA, anti-Smith (anti-Sm), Ro/SSA, La/SSB, and U1 ribonucleoprotein (RNP)
- Anti-double-stranded DNA (anti-dsDNA)
- Antiphospholipid antibodies (lupus anticoagulant [LA], immunoglobulin [Ig] G and IgM anticardiolipin [aCL] antibodies, and IgG and IgM anti-beta2-glycoprotein [GP] 1)
- C3 and C4 or CH50 complement levels
- Erythrocyte sedimentation rate (ESR) and/or C-reactive protein (CRP) levels
- Urine protein-to-creatinine ratio