Systemic Lupus Erythematous

INTRODUCTION:
  • Autoimmune disease that affects multisystems
  • Women > Men - 9:1 ratio
  • African Americans > Whites
  • SLE is Type III hypersensitivity reaction
  • Onset usually between ages of 15 and 45 years, but can occur in childhood or later in life
TYPES:
  • Histologic Classifications:
  • Class I Minimal mesangialglomerulonephritis:Histologically normal on light microscopy but with mesangial deposits on electron microscopy.
  • Class II Mesangial proliferative lupus nephritis: responds completely to treatment with corticosteroids.
  • Class III Focal proliferative nephritis :Responds to treatment with high doses of corticosteroids.
  • Class IV Diffuse proliferative nephritis:Treated with corticosteroids and immunosuppressant drugs.(most common)
  • Class V Membranous nephritis :Extreme edema and protein loss.
  • Class VI Glomerulosclerosis
CLINICAL MANIFESTATIONS :
  • Most common rash is photosensitive, raised erythematousmalar rash. 55-85% develop at some point in disease
  • Discoid Lupus Erythematosus (DLE): 15-30% circular, scaly hyperpimented lesions with erythematous rim, atrophic center—can be disfiguring
  • Mouth/vaginal/nasal ulcers
  • Alopecia: may be diffuse or patchy. Occurs 50%
  • Polyarthritis, mild to disabling, occurs most frequently in hands, wrists, knees. Occurs 90%
  • Joint deformities occur in only 10%
  • Arthritis of SLE tends to be transitory
  • Onion skin spleen is seen in SLE
  • Spontaneous abortions and prematurity in patient with SLE planning for pregnancy
  • If single joint has persistent pain, consider osteonecrosis (prevalence increased in SLE over general population, especially if on steroids.)
  • Myositis with elevated CK and weakness rarely occurs
  • Serositis
Pulmonary:
  1. Pleuritis with or without effusion
  2. If case is mild, tx: NSAIDS
  3. If case is severe, tx: steroids
  4. Life-threatening manifestations: interstitial inflammation which can lead to fibrosis and intra-alveolar hemorrhage.
  5. Also pneumothorax and pulmonary HTN can occur
Cardiac:
  1. Pericarditis: most common cardiac manifestation and usually responds to NSAIDs.
  2. Myocarditis (rare) and fibrinous endocarditis (Libman-Sacks) may occur. 
  3. Steroids plus treatment for CHF/arrhythmia or embolic events. 
  4. MI due to atherosclerosis 
  5. Maternal lupus erythmatosus has been associated with a high incidence of congenital heart block in offspring
Neuro:
  1. Cranial or peripheral neuropathy occurs in 10-15%, it is probably secondary to vasculitis in small arteries supplying nerves.
  2. Diffuse CNS dysfunction: memory and reasoning difficulty
  3. Headache: if excruciating, often indicate acute flare
  4. Seizures of any type
  5. Psychosis: must distinguish from steroid-induced psychosis (occurs in 1st weeks of tx at doses ≥40mg prednisone and resolves after several days of reducing or stopping tx) TIA, Stroke: mostly increased among patients that are APLA positive
  6. 50-fold increase in risk of vascular events in women under 45 compared to healthy women
  7. Treatment for clotting event is long-term anticoagulation
Heme:
  1. Autoimmune Hematolytic Anemia
  2. Leukopenia: almost always consists of lymphopenia, not granulocytopenia
  3. Thrombocytopenia 
Renal:
Lupus Nephritis:
  • Wire loop lesions represent local Positive thickening of glomerular capillary walls
Immunoglobulins:
  1. Anti-dsDNA IgG: very specific, may correlate with disease activity
  2. Anti-Sm: specific, but only present in 25% of cases, does not correlate with activity
  3. APLA: not specific. Used to identify patients at increased risk for clots, thrombocytopenia and fetal loss
  4. Anti Ro:subacute cutaneous lupus erythematosu
  5. Image result for SLE

DIAGNOSIS:
  • American College of Rheumatology (ACR) criteria:
  • (SOAP BRAIN MD)
  • Serositis
  • Oral ulcers
  • Arthritis
  • Photosensitivity
  • Blood disorders
  • Renal involvement
  • Antinuclear antibodies
  • Immunologic phenomena (eg, dsDNA; anti-Smith [Sm] antibodies)
  • Neurologic disorder
  • Malar rash
  • Discoid rash
Laboratory studies :
  • CBC with differential
  • Serum creatinine
  • Urinalysis with microscopy
  • Normal CRP with elevated ESR
  • Complement levels
  • Liver function tests
  • Creatine kinase assay
  • Spot protein/spot creatinine ratio
  • Autoantibody tests
Lupus band test
  • Imaging studies: 
  • Joint radiography
  • Chest radiography and chest CT scanning
  • Echocardiography
  • Brain MRI/MRA
  • Cardiac MRI 
  • Procedures 
  • Arthrocentesis
  • Lumbar puncture
  • Renal biopsy
TREATMENT:
  • Conservative Management:
  • NSAIDs: to control pain, swelling, and fever
  • Antimalarials: Generally to treat fatigue joint pain, skin rashes, and inflammation of the lungs
  • Commonly used: Hydroxycholorquine
  • Corticosteroids (Mainstay of SLE treatment)
  • To rapidly suppress inflammation
  • Commonly used: prednisone, hydrocortisone, methylprednisolone, and dexamethasone
  • Immunosuppressives:
  • Primarily for CNS/renal involvement
  • Mycophenolatemofetil
  • Azathioprine
  • MTX: for treatment of dermatitis and arthritis, not life-threatening disease
  • Cyclosporine: used in steroid-resistant SLE, risk of nephrotoxicity
  • Cyclophosphamide: patients with nephritis
Exam Question
  • Commonest cutaneous eruption in Systemic Lupus Erythmatosus is Erythema of light exposed area
  • Criteria for diagnosis of systemic lupus erythematosus includes Photosensitivity, Malar rash ,Discoid rash
  • 22% of patients with disseminated discoid lupus erythematosus develops SLE
  • Anti Ro autoantibodies is most suggestive of subacute cutaneous lupus erythematosus
  • High titers of Anti dsDNA are considered to be highly specific for Systemic lupus erythematosus 
  • Neonatal lupus erythematosus (NLE) is characterized by Cutaneous lesions, Heart block & Thrombocytopenia
  • Libman-Sacks endocarditis may develop with systemic lupus erythematosus 
  • Woman with systemic lupus erythematosus (SLE) and chronic renal failure develops anemia because of IgG directed against red blood cells
  • There may be an increase in spontaneous abortions and prematurity in patient with SLE planning for pregnancy
  • Diffuse proliferative is the most common histological type of nephritis seen in systemic lupus erythematosus (SLE)
  • Children born to mothers with systemic lupus erythematosis are likely to have Complete heart block
  • Characteristic feature of SLE is Polyserositis
  • Autoimmune Hematolytic Anemia & Anti-ds DNA are seen in SLE
  • In SLE, characteristic kidney lesion is Wire loop lesions
  • Indications of steroids in SLE are Endocarditis
  • SLE is Type III hypersensitivity reaction
  • Onion skin spleen is seen in SLE
  • Normal CRP with elevated ESR seen in SLE
  • Skin biopsy of SLE patient using RTC labeled human IgG antiserum shows deposition of irregular particles at dermoepidermal junction. This means there is presence of Immune complex deposits
  • SLE is can cause both cicatricial as well as noncicatrical alopecia 
  • Band test is done in SLE
  • According to WHO, membranous glomerulo­nephritis seen in SLE, is Class V 
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