General
The classic lupus patient
Female
15-45
non caucasian (AA, Asian, Hispanic)
The 1997 ACR Criteria mnemonic
SOAP-BRAIN-MD
Pathology of SLE skin rash
interface dermatitis
Biopsy showing immunoglobulins deposited at dermal epidermal junction on immunofluorescence
Differentials for change of behavior in a SLE patient
NPSLE, prednisone induced psychosis, infection, mental illness, medication effect, metabolic disturbance
Indications for a renal biopsy
increasing serum creatinine w/o compelling alt cause
confirmed proteinuria of >1 g/day
proteinuria >.5 g/day + hematuria or cellular casts
Klinefelter's Disease men have 14 fold risk of SLE
2017 EULAR SLE Classification
ANA >1:80 and >10 points
Treatment of SLE skin rash
Photoprotection, control SLE activity, hydroxychloroquine, chloroquine, thalidomide (severe mouth ulcers), Belimumab (recalcitrant discoid or subacute rash)
Lung involvement in Lupus
pleuritis, acute lupus pneumonitis, chronic ILD, Pulmonary htn, shrinking lung syndrome, cryptogenic organizing pneumonia, infection
Serologies useful in Lupus Nephritis
dsDNA, complement component levels, (C4 can always be low in someone with a C4 deficiency)
patients w/ crescents more likely to be p-ANCA positive
Risks for developing Lupus
Environmental 1/3: tobacco, viral infection, CMV, silica exposure, UV light, pesticides, gut microbiome, demyelineating drugs
Genetic 2/3: affected family members, first degree relatives and twins
HLA DR2 and HLA DR3
complement component deficiencies
Screening test for ANA
indirect immunofluorescence test
Other: ELISA, Multiplex immunoassays
Mild, Moderate, Severe Treatment approach with an SLE patient with increasing symptoms
Mild: NSAIDs, Hydroxychloroquine, low dose prednisone, methotrexate
Moderate: MMF or AZA, Belimumab, Prednisone
Severe: High dose prednisone, cytotoxic medications: induction with CYC or MMF f/b maintainence (AZA MMF, Tacrolimus/Cyclosporine), biologics, +- plasmapharesis, IVIG, stem cell transplant
Heart involvement in lupus
pericarditis
myocarditis
vasculitis
secondary atherosclerotic CAD and MI
secondary hypertensive dx
medication effects
valvular dx
libman sacchs verrucae
The first line therapy for patients with severe lupus nephritis
IV methylprednisone daily for 3 days, then prednisone daily (tapered) +
MMF 2-3 g/day for 6 months OR CYC
Adjunctive Therapies: HCQ, ACE Inhib, statin therapy, stop smoking, counsel against pregnancy
Four most common causes of death in SLE patients
infection, active SLE (esp lupus nephritis with renal failure, CNS lupus, vasculitis, pneumonitis), CVD, Malignancy (need annual pap)
Nuclear staining patterns and an ANA negative SLE
Peripheral/Rim - SLE
Speckled - SLE and other; not specific
Nucleolar - Scleroderma
SSA/Ro 52kD which is a cytoplasmic antigen not nuclear.
Lupus Manifestations that warrant high dose steroid therapy
SLE Nephritis, CNS lupus with severe manifestations, AI Thrombocytopenia (<30k), AIHA, Acute pneumonitis, DAH, Severe vasculitis with visceral organ involvement, serious complications from pleuritis, pericarditis, peritonitis, MAS
GI involvement in lupus
esophageal dysmotility, pancreatitis, serositis, mesenteric vasculitis, hepatitis, intestinal pseudoobstruction, protein losing enteropathy (pt w/ low albumin but no proteinuria
Maintenance therapy for lupus nephritis
After induction therapy, can use AZA or MMF.
*Avoid AZA for patients on Allopurinol or Warfarin. Can use CYC for patients who can’t tolerate either. Prednisone tapered over time.
Serologies and manifestations of
SCLE
DILE
Discoid Lupus
SCLE - skin, can be ANA negative SSA or SSB+
DILE - systemic but X CNS X Renal; Histone antibodies
Discoid Lupus - skin, typically negative ANA
Labs in SLE Flare
*how to differentiate from infection
hypocomplementemia, elevated DsDNA antibodies, low WBC
fever and elevated CRP - r/o infection
WBC "normalizes" and fever - r/o infection
left shift - r/o infection
complements rise in infection
Hematologic Manifestations of SLE
AIHA (Coombs positive!)
Leukopenia
Thrombocytopenia
APLAS
Anemia of Chronic dx
TTP (fever, AMS, AKI, MAHA, Thrombocytopenia)
Macrophage Activation Syndrome (R/o EBV and CMV as a trigger)
Joint Manifestations in SLE
Pain, tenderness, tenosynovitis, joint deformities
- non erosive arthropathy (MCP joint subluxation, ulnar deviation, swaan neck deformity d/t lax joint capsules, tendons, ligaments)
- erosive symmetric polyarthritis (RF and CCP positive)