SLE (Systemic Lupus Erythematosus)



Suspect:

Clinical evidence of (fatigue, rash, photosensitivity, inflammatory arthritis, weight loss, and fever) and laboratory findings (ANA, anti-Smith Ab, low complements and a high erythrocyte sedimentation rate + Normal CRP)



Epidemiology:

• 90% Female

• Peaks 3rd Decade



Skin Disease Classification:

• Acute cutaneous lupus erythematosus (ACLE)

	• Erythematous, macular, patchy eruption - Butterfly rash

	• Heals without scarring

• Subacute cutaneous lupus erythematosus (SCLE):

	• Photosensitive rash

	• Erythematous annular/polyclinic or patchy papulosquamous lesions

	• Anti-Ro/SSA autoantibodies

• Discoid lupus erythematosus (DLE):

	• Scarring, atrophy, and permanent alopecia



Differential Diagnosis:

• ANCA-associated vasculitis, rheumatoid arthritis, adult-onset Still disease, dermatomyositis, Sjogren syndrome, and mixed connective tissue disease

• Non-autoimmune rheumatologic diseases (i.e. fibromyalgia)

• Infections

• Malignancy (e.g. hematologic)

• Endocrine disorders (e.g. thyroid disease)

• Kikuchi disease

• Type-I Interferonopathies

• Castleman's disease

• Prolidase deficiency

• Angioimmunoblastic T-cell lymphoma

• Evans' syndrome in the context of primary immune deficiencies

• Autoimmune lymphoproliferative syndrome



Clinical Features:

Lungs:

• Pleural effusions

• Acute lupus pneumonitis

• Diffuse alveolar hemorrhage

• Shrinking lung syndrome

Arthritis:

• Joint pain

• Swan neck/Ulnar deviation

• Jaccoud arthropathy

• 90% joints affected

• Non-erosive

Serositis:

• Pleural/pericardial/abd

GI:

• Noninfectious hepatitis

• Mesenteric vasculitis, inflammation of the small and large bowel, pancreatitis, protein-losing enteropathy, and diffuse peritonitis

Lupus nephritis can present with:

• Minimal laboratory abnormalities (non-nephrotic proteinuria, hematuria)

• Frank nephritis (hypertension, lower extremity edema, active urine sediment, and elevated serum creatinine)

• And/or nephrosis (nephrotic-range proteinuria, dependent edema, and thrombosis)

Neuropsychiatric systemic lupus erythematosus (NPSLE):

• May involve PNS and CNS

• Headache, mild cognitive dysfunction, and mood disorder

• Seizures and psychosis

• AB: Antineuronal, Anti-NMDA receptor, Antiribosomal P, APLA, PAC

• Aseptic meningitis

• Cranial neuritis

• Encephalitis

• Mononeuritis Multiplex

• Peripheral neuropathy

• Transverse Myelitis

• Psychosis

• Seizures

• Strokes

Skin:

• Nonscarring alopecia

• Photosensitivity

• Malar rash - spares nasolabial folds

• Painless oral or nasopharyngeal ulcerations occur in 5% of patients

Cardiac:

• Asymptomatic pericarditis

• Myocarditis

• Libman-Sacks endocarditis

• Valvular disorders

• CAD

APLA/LAC:

• Renal artery or vein thrombosis

• Miscarriage

• Livedo reticularis

• Cytopenias

• Cardiac valve vegetations

APLA/LAC:

• Mesenteric thrombosis

• Pelvic vein thrombosis

Hematologic:

• AIHA 10%

• Leukopenia, lymphopenia, low PLT

• Anemia of inflammation

Cutaneous Vasculitis:

• Distal extremities



Drug Induced Lupus:

• Symptoms limited to: Arthritis, Fever, Serositis



Labs:

• ANA titers > 1:80

• CBC, TSH and urinalysis (Cytopenias)

• Anti-Smith, Ro/La, anti-Jo-1, SCL-70, RNP antibodies, Complement levels (C3 and C4)

• ds-DNA: Kidney disease common

• Antihistone: Drug induced SLE

• Kidney biopsy if suspicion for nephritis

• Anti dsDNA correlates disease activity

• C3 C4 accompanies flares

• CRP normal, ESR active flare

• Thrombosis highest risk with + LAC, anti-β2-glycoprotein I, and anticardiolipin antibodies

• (-) ANA + (+) anti-Ro/SSA can rule in SLE



Malignancy Associated with Lupus:

• Chronic B-cell activation and/or medications (azathioprine or cyclophosphamide)

• Hodgkin lymphoma and leukemia

• Lung cancer

• Cervical cancer - immunosuppression and human papillomavirus



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