Kinder CIS- Lupus Flashcards
Criterion for SLE
4 of the 11:
Malar rash Discoid rash Photosensitivity Oral ulcers- Nonerosive arthritis pleuritis or pericaritis Renal Disorder Neurologic Disorder Hematologic Disorder Immunologic Disorder Positive Antinuclear Antibody
Malar rash of SLE
Fixed erythema, flat or raised, over the malar eminences, tending to spare the nasolabial folds
Discoid rash of SLE
- erythrematous raised patches with adherent keratotic scaling and follicular plugging, atrophic scarring may occur in older lesions
Photosensitivity of SLE
- skin rash as a result of unusual reation to sunlight, by patient history or physician observation
Oral ulcers of SLE
oral or nasopharyngeal ulceration, usually painless, observed by physician
Nonerosive arthritis of SLE
- involving 2 or more peripheral joints, characterized by tenderness, swelling, or effusion
pleuritis or pericaritis of SLE
- pleuritis- convincing history or pleuritic pain or rubbing heard by a physican of evidence of pleural effusion - OR-
pericarditis- documented by electrocardiogram or rub or evidence of pericardial effusion
- pleuritis- convincing history or pleuritic pain or rubbing heard by a physican of evidence of pleural effusion - OR-
Renal Disorder of SLE
Persistent proteinuria > 0.5 grams per day or > than 3+ if quantitation not performed –OR—
Cellular casts – may be red cell, hemoglobin, granular, tubular, or mixed
Neurologic Disorder of SLE
Seizures – in the absence of offending drugs or known metabolic derangements; e.g., uremia, ketoacidosis, or electrolyte imbalance –OR—
Psychosis – in the absence of offending drugs or know metabolic derangements, e.g., uremia, ketoacidosis, or electrolyte imbalance
Hematologic Disorder of SLE
Hemolytic anemia – with reticulocytosis –OR—
Leukopenia – less than 4,000/mm3 on ≥ 2 occasions –OR—
Lymphopenia – less than 1,500/mm3 on ≥ 2 occasions –OR—
Thrombocytopenia – less than 100,000/mm3 in the absence of offending drugs
Immunologic Disorder of SLE
Anti-DNA: antibody to native DNA in abnormal titer –OR—
Anti-Sm: presence of antibody to Sm nuclear antigen –OR—
Positive finding of antiphospholipid antibodies
Positive Antinuclear Antibody
of SLE
An abnormal titer of antinuclear antibody by immunofluorescence or an equivalent assay at any point in time and in the absence of drugs
Systemic Lupus Erythematosus etiology/ characteristics
Female predominance 10-15:1
Unknown etiology
- Genetic, hormonal, and environmental influences play a role in the disease
- Smoking is a risk factor for SLE and UV light can trigger exacerbations
SLE is characterized by the production of autoantibodies to:
- Nuclear antigens - ANA
- Cytoplasmic antigens
- Cell surface antigens
- Soluble antigens – IgG and phospholipids
Lupus autoantibodies and timeline
First to appear 3.4 years prior to diagnosis ANA anti-Ro/SSA anti-La/SSB Antiphospholipid
Second to appear 2.2 years prior to diagnosis
anti-dsDNA
Third to appear 1 year prior to diagnosis
anti-Sm
anti-RNP
constitutional and mucocutaneous symptoms of lupus
Constitutional
Fever, fatigue, and weight loss
Mucocutaneous 80-90% of patients
Alopecia
Nasal and oral ulcers – painless
Acute cutaneous lupus erythematosus (ACLE)
Subacute cutaneous lupus erythematosus (SCLE)
Chronic cutaneous lupus erythematosus (CCLE)
Acute Cutaneous Lupus Erythematosus (ACLE)
Localized
- Classic malar rash
— Sparing of nasolabial folds
— Differential diagnosis: acne rosacea, seborrheic dermatitis, and flushing syndromes
Rosacea – telangiectasias, pustules, heat and alcohol make it worse
Seborrehic dermatitis – scaly, erythematous plaques on the eyebrows and lateral sides of nose including the nasolabial folds
Generalized
- Maculopapular erythematosus lesions involving any area of the body in a photosensitive distribution
- Dorsa of hands and the extensor surfaces of the fingers are commonly involved
- Erythema is typically found between the interphalangeal joints and spares the knuckles, unlike Gottron papules
Subacute Cutaneous Lupus Erythematosus
Papulosquamous or annular Non scarring Most photosensitive Torso, Limbs, and spares face Associated with anti-SSA/Ro antibody Can be induced by medicines - Hydrochlorothiazide - terbinafine
Chronic Cutaneous Lupus Erythematosus
Discoid Lupus
- Raised, erythematous plaques with adherent scale
- Scalp, face, and neck
- Erythematous ring around lesion – active component
- Can lead to scarring, skin atrophy, permanent alopecia, and disfigurement
Lupus panniculitis
- Deep, firm nodule that can lead to cutaneous atrophy and rarely ulceration
Musculoskeletal stuff in SLE
Arthritis and arthralgias in up to 95% of SLE patients
Commonly involve wrists, and small joints of hands
Boney erosions rarely occur
Swan neck deformity and ligamental laxity often noted
- Usually reducible
Lupus Nephritis
Significant cause of morbidity and mortality
90% of SLE patients have pathologic evidence of nephritis on biopsy
Clinically significant lupus nephritis occurs in 50% of patients with SLE
Usually develops in the first 3 years of the disease
Immune complex glomerulonephritis is most common, but SLE patients may have tubulointerstitial disease or vascular disease
Clinical presentation – highly variable
- Asymptomatic hematuria
- Nephrotic Syndrome
- Rapidly progressive glomerulonephritis with loss of renal function
Lupus nephritis screening
Screening
History
- Polyuria, nocturia, or foamy urine
Examination
- Blood pressure and Lower extremity edema
Urinalysis
- Hematuria, pyuria, dysmorphic blood cells, and red cell casts
- Proteinuria
- Screen patients every 3 months with active disease
Biopsy
- Patients with clinical or laboratory findings suggestive of nephritis should have a renal biopsy
- Confirms Diagnosis
- Evaluates disease activity
- Determines appropriate treatment
Classes of Lupus Nephritis
Class I and Class II nephritis
Excellent renal prognosis
Does not require specific treatment
Class III and Class IV nephritis
Long-term prognosis is extremely poor without immunosuppression
Class V Better prognosis than class III or IV Proliferative changes portend a worse prognosis
Cardiovascular stuff with Lupus
May involve pericardium, valves, myocardium, and coronary arteries
Raynaud’s affects 30% of SLE patients
- Vasospasm of the digital arteries and arterioles after exposure to cold or stress
Pericarditis
- May be asymptomatic with small pericardial effusions
- On occasion can be life threatening from hemodynamic complications
Valves
- Mitral and Aortic Valve leaflets with valve thickening
- Libman-Sacks endocarditis – nonbacterial vegetations
Myocarditis and conduction defects are rare
SLE is associated with accelerated atherosclerosis and is a risk factor for coronary artery disease
Pulmonary stuff with SLE
Pleural effusions, typically small develop in 50% of patients
Pleuritic chest pain
Lupus pneumonitis
- Extremely rare
- Acute respiratory illness with fever, cough, and pulmonary infiltrates
- High Mortality
Chronic interstitial lung Disease
- Rare
Diffuse Alveolar Hemorrhage
- Rare and frequently fatal
- Acute pulmonary infiltrates, falling hematocrit, and hemorrhagic bronchoalveolar lavage
- Lupus nephritis frequently associated
