Flashcards in Autoimmune Rheumatologic Disorders Deck (31):
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
- 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
- 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 _______
Treatment of SLE includes
- 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
Pts with Sjogren's also tend to develop ______
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?
What can you do if you're suspicious for Sjogren's but all the tests are inconclusive?
- 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
Presentation of limited scleroderma disease
CREST - limited to face, neck, distal extremities
- Calcinosis cutis (calcification of skin)
- Raynaud phenomenon
- Esophageal dysmotility
- Sclerodactyly (sausage fingers)
Presentation of diffuse scleroderma dz
- Widespread fibrosis in skin and internal organs
- Shorter period b/w Raynaud's & onset of other sx's (weeks to months)
- Polyarthralgia, weight loss, malaise
- Early "edematous" phase w/ hyperpigmentation, carpal tunnel, muscle weakness, fatigue, decreased joint motility; Clinically silent organ involvement
Most rapidly evolving pulmonary & renal damage in scleroderma pts occurs when?
Initial 4 years
______ and _______ are present in almost all scleroderma pts
_______ antibodies are found in 50% CREST pts
Tx severe Raynaud syndrome in scleroderma pt
CCB (e.g. nifedipine) or losartan