Demographics for typical SLE pt
- Women of childbearing age
- African, Caribbean, Hispanic descent
When might you be suspicious of SLE in a pt?
Young woman presenting with multiple clinical problems but otherwise no significant PMH
Hallmark of SLE labs are _______?
Auto-antibodies to nuclear proteins
Describe the pathophysiology of SLE
Auto-antibodies to nuclear proteins damage tissues through immune activation and deposition of immune complexes
Besides auto-antibodies, what else is present in SLE?
- C3/C4 deficiencies
- Increased B-lymphocyte stimulator (BlyS)
Clinical presentation of newly diagnosed SLE pt
Young pt with multiple problems:
- Glomerulonephritis → edema, HTN, hematuria, foamy urine, oliguria
- Hematologic disorders → anemia, thrombocytopenia
- Arthritis
- Mucucutaneous lesions → butterfly rash, livid reticularis
- Raynaud phenomenon
- Serositis → pleuritic pain, pericarditis
Clinical presentation of pt with established SLE
- Hospital admission due to flare
- Hematologic changes (d/t SLE or meds)
- Neuropsychiatric dz
- Premature CVD
What do you need to diagnose SLE?
Clinical presentation PLUS supporting labs
What labs should you get if you are suspicious for SLE?
- Elevated ANA
- CBC w/ diff → anemia, thrombocytopenia
- UA → proteinuria, urinary sediment
- Anti-dsDNA
- C3/C4 complement deficiency
Dx lupus nephritis
- Proteinuria → >0.5g/day or 3+
- Urinary sediment → >5 RBC or WBC/HFP
- Kidney bx
What should you do to treat SLE flare?
- Don’t need to get ANA levels if established dx
- R/o lupus nephritis via Cr, UA, urine sediment
- Anti-dsDNA, ESR/CRP, C3/C4
- Consult rheumatology
The mainstay of SLE treatment is _______
Glucocorticoids
Treatment of SLE includes
- Glucocorticoids!
- Hydroxychloroquine → reduces flares & improves survival
- Belimumab → binds to and inhibits BlyS
- IVIG if heart block
Indications for belimumab in SLE
- Higher disease activity
- Steroid use
- Elevated anti-dsDNA
- Low complement
Clinical manifestations of Sjogren’s syndrome
Xerophthalmia
Xerostomia
Pts with Sjogren’s also tend to develop ______
Malignant lymphoma
Labs for Sjogren’s will show _______
- Autoantibodies to Ro/SSA and La/SSB
- Antimuscarinic antibodies
Why are xerophthalmia and xerostomia concerning?
- High risk for corneal ulcer or perforation (consult ophthalmology)
- Susceptible to dental caries
What test can you use to help diagnose Sjogren’s, specifically xerophthalmia?
Schirmer’s test
What can you do if you’re suspicious for Sjogren’s but all the tests are inconclusive?
Labial biopsy
Tx Sjogren’s
Symptomatic relief
- Artificial tears, drink more water
- Propionic gels for vagina
- Corneal ulcer → eye patch, boric acid ointment
- Monoclonal antibody to CD20, add CHOP if lymphoma
- Hydroxychloroquine for arthrlagias
- Sodium bicarb for renal tubular acidosis
- Glucocorticoids only indicated for systemic vasculitis
What meds do Sjogren’s pts want to avoid?
Avoid diuretics, anti-HTN, anticholinergics, antidepressants
What happens in scleroderma?
Fibrosis of skin & internal organs
Name the 2 types of scleroderma.
Which is more common?
- CREST syndrome (limited disease) → 80% pts
- Diffuse disease
