Rheum E2 -SLE, APS, & Dx tests

Who does SLE more commonly affect?

F > M, peak 15-45 yo, AA, Hispanic, Asian >>>> whites, more severe in males, genetic component

What triggers SLE?

infxn, stress, hormonal, sunlight, fluorescent light, drugs (BCPs, Sulfonamides)

What causes Systemic Lupus erythematosus?

B-cell, T-cell, and monocyte OVERproduction and dysfunction driven by interferons; complement deficiencies, & immune complex deposition

How does the subtype Acute cutaneous lupus (ACLE) present?

malar (“butterfly”) rash that SPARES the naso-labial fold

How does the Subacute cutaneous lupus (SCLE) present?

annular, circular or papulosquamous lesion, NON-scarring, very photosensitive (affects sun exposed areas), MC on caucasian females

Subacute cutaneous lupus (SCLE) typically has what antibodies?

Ribonucleoproteins SSA (Ro) & SSB (La)

How does Chronic Cutaneous Discoid lupus present?

localized to skin, Scarring -VERY disfiguring!!, mainly on face(cheeks), scalp, & UE

How is Chronic Cutaneous Discoid lupus dx?

skin biopsy

What is a lupus-band test?

lupus skin biopsy → reveals immune complexes deposited at the dermo-epidermal junction

What is the 1st line Tx for cutaneous LE?

*in order

Sunblock, Topical steroids, Hydroxychloroquine

What is the 2nd line Tx for cutaneous LE?

IL steroids, Leflunomide

How do mothers give their fetuses lupus?

Vertical transmission- SSA (Ro+) & SSB (La+) IgG Abs travel across the placenta

When do the maternal LE antibodies disappear from the infant's circulation?

8 months

What are the effects of Neonatal lupus?

heart block with bradycardia and/or myocarditis, Thrombocytopenia/Leukopenia, self-resolving rash

What drugs WILL trigger drug-induced lupus erythematosus?

Hydralazine, Minocin & Procainamide, Anti-TNF inhibitors (Remicade, Enbrel, Humira)

What drugs MIGHT trigger drug-induced lupus erythematosus?

Quinidine, Phenytoin, Sulfonamides, D-Penicillamine, Isoniazid

How does drug-induced lupus present?

mainly rash, fever, serositis, no renal or lung disease

90% of patients with drug-induced lupus will have what antibodies?

Anti-histone

What is the tx for drug-induced lupus?

self-limited → REMOVE the offending agent

What is the pathogenesis of SLE?

excessive CD4 (T-helper) cells, B-cell proliferation, increased interferon activity, Diffuse I:C deposition

What is the pneumonic for the dx criteria of SLE?

MD SOAP BRAIN

"MD" in MD SOAP BRAIN for SLE

Malar rash

Discoid rash

"SOAP" in MD SOAP BRAIN for SLE

Serositis — pleuritis, pericarditis

Oral ulcers (painless)

Arthritis (non-erosive)- Jaccoud's arthritis

Photosensitivity

"BRAIN" in MD SOAP BRAIN for SLE

Blood- leukopenia, thrombocytopenia, hemolytic anemia

Renal- proteinuria, RBC casts

+ ANA

Immunologic- + Anti-dsDNA, smith, ACL Abs

Neuro- seizures, psychosis

What type of non-erosive arthritis is associated with SLE?

Jaccoud's arthritis

What 2 new criteria were added for SLE diagnosis?

Fever & Mucocutaneous

What is an absolute/required major criteria for SLE?

+ ANA

How many criteria are required for SLE diagnosis?

4 out of 11

What organ function is commonly involved in SLE?

Renal- glomerulonephritis or nephrotic syndrome

What are the new guidelines for lupus nephritis?

Biopsy all & patients w/ proteinuria should be on hydroxychloroquine & ACE inhibitor

Which antibodies have a strong pathological associated with lupus nephritis?

Anti-ds DNA

What stage of Lupus nephritis:

Minimal change- mild, good prognosis

Stage I

What stage of Lupus nephritis:

Mesangial- interstitium

Stage II

What stage of Lupus nephritis:

Focal proliferative- Spotty glomeruli

Stage III

What stage of Lupus nephritis:

Diffuse proliferative- aggressive

Stage IV

What stage of Lupus nephritis:

Membranous- (basement membrane) heavy proteinuria (nephrotic) & thrombosis

Stage V

What stage of Lupus nephritis:

Advanced sclerosis- end stage

Stage VI

What is Jaccoud's arthritis?

deforming non-erosive arthropathy characterized by ulnar deviation of the 2nd to 5th fingers with MCP subluxation

What is an LE cell?

PMN that has phagocytosed another cell's damaged nucleus that has combined with a specific antibody

Anti-nucleolar antibodies:

SLE & PSS

Anti-DNA antibodies:

SLE

What is the 1st line tx for mild sx of SLE?

NSAIDs and/or Hydroxychloroquine

What is the 1st line tx for moderate sx of SLE?

Steroids (prednisone) + Methotrexate (Rheumatrex)

What is the 1st line tx for severe sx of SLE?

IV Cyclophosphamide (Cytoxan) or Mycophenalate mofetil (Cellcept) or Rituximab

What are 2nd line tx for lupus nephritis?

Belimumab, Voclosporin, Anifrolumab

Is pregnancy contraindicated in patients with Lupus?

No

When should patients with Lupus get pregnant?

When SLE/APL is inactive or stabilized and avoid fetal toxic drugs

Anti-Phospholipid (APL or APA) syndrome is an autoimmune HYPERcoagulable state resulting in what 4 symptoms?

-Fetal wastage-recurrent miscarriages

-Arterial & venous thrombosis

-Thrombocytopenia

-Livedo reticularis

What is the most pathogenic APL antibody?

Lupus anticoagulant (LAC)

What are the 3 most common APL labs?

Lupus anticoagulant (LAC), Anti-cardiolipid (ACL), Anti-beta 2 glycoprotein I

What Ig is the worst Anti-cardiolipin?

IgG

What is the dx criteria for APL syndrome?

Lupus anticoagulant positivity on ≥ 2 occasions at least 12 weeks apart

What is the tx for + APL Abs and no thrombosis?

Baby ASA (81 mg)

What is the tx for + APL Abs and positive thrombosis?

ASA + Coumadin*

*keep INR btwn 2-3

Do you continue anticoagulation even when APL Abs become negative?

Yes

What is an exception to anticoagulation therapy in patients with APL Abs?

Platelets < 50,000

When would you give a DOAC for APL instead of Coumadin?

ONLY if Coumadin is contraindicated — allergy, SAEs, noncompliance

*DOACs are inferior