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 #SLE #Systemic #Lupus #Erythematosus #Diagnosis #Rheumatology
