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Dr. Ajith Kumar J MD
Dept. of Emergency Medicne
KIMS, Kollam
India
editor
SLE
It is the most common connective tissue disease.
90% of affected individual are women.
5 year survival rate is 90%.
PATHOPHYSILOGY
Cause of SLE is incompletely understood.
SLE has high concordance in monozygotic twins & assosciated with multiple poymorphism in HLA locus on chromosome 6.
There are different polymorphism which perdisposes to SLE like ITGAM gene, IRF5, STAT4 & BLK gene.
Basically the disease is due to production of autoanitbody.
CLINICAL FEATURE
Non specific symptoms: Fever, weight loss, mild lymphadenopathy reflects active inflammatory disease.
Arthritis & fibromyalgia: A variety of problems occurs including migratory arthralgia & early morning stiffness, tenosynovitis & small joint synovitis.
Revised American Rheumatism Assosciation criteria for SLE | |
Malar rash | Fixed erythema, flat or raised , sparing nasolabial fold. |
Discoid rash | Erythematous raised patches with adherent keratotic scarring & follicular plugging |
Photosensitivity | Rash due to unsual reaction to sunlight. |
Oral ulcers | Oral or nasopharyngeal ulceration may be painless. |
Arthritis | Non erosive, involving two or more peripheral joints |
Serositis | Pleuritis, pericarditis |
Renal disorder | Persistent proteinuria >0.5g/d or cellular cast |
Neurological disorder | Seizures or psychosis in the absence of provoking drugs or metabolic dearrangement. |
Heamatological disorder | Hemolytic anemia or leucopenia (<4x109/l) or thrombocytopenia(<100 X 109/l) or lymphopenia in the absence of offending drug. |
Immunological disorder | Anti DsDNA antibodies in abnormal titre or anti Sm or Positive phospholipid antibodies. |
ANA antibody disorder | Abnormal titre of ANA by immunofluresence. |
Presence of 4 these features either serially or simultaneously is daignostic. | |
INVESTIGATION
Patients with active disease is almost always positive for ANA
Anti Ds DNA are characteristic of severe active SLE.
Patients with active disease tends to have low C3,C4.
A raised ESR, lecopenia & lymphopenia are typical of active SLE along with anemia, hemolytic anemia, thrombocytopenia.
CRP are often normal in active SLE.
MANAGEMENT
Conservative management of non life threatening disease
NSAID'S
It is particularly useful for athralgia or arthritis.
People with SLE at high risk for NSAID induced asceptic meningitis, elevated serum transaminase, hypertension, and renal dysfunction.
Increased risk of MI.
Hydroxychloroquine:200-400mg/day
Reduces dermatitis, arthritis and fatigue.
Adverse effects: Rentinal damage, agranulocytosis, aplastic anemia, ataxia, cardiomyopathy, dizziness, myopathy, otoxicity, peripharal neuropathy, pigmentation of skin, seziures, thrombocytopenia.
Short course of steroids may be required for rash, synovitis, pleurisy & pericarditis.
Hypertension& hyperlipidemia should be controlled.
Pulse therpay in acute setting
Methylprednisolone :1 gm IV OD X 3days.
Followed by prednisoline 0.5mg- 1mg/kg per day PO; it is then tapered down to 5 -10mg/day depending on clinical response over 4-6 weeks.
Lowest dose of corticosteroid or immunosuppresive agent has to be used.
Adverse effects :Infection, hypertension, hyperglycemia, hypokalemia, acne, allergic reaction, anxiety, asceptic necrosis of the bone, cushingoid changes, insomnia, menstural irregularities, mood swings, osteoporosis, psychosis.
Mycophenolate mofetil (MMF) or cyclophospamide are added to glucocorticoids in patients with lupus nephritis.
Azothiprine is probably less effective .
Persons with antiphospholipid syndrome who had previous thrombosis require life long warfarin.
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emmedonline
Dr. Ajith Kumar J MD
Dept. of Emergency Medicne
KIMS, Kollam
India
editor