Lupus (manifestations (Nervous system ( Systemic lupus may affect the…
thrombosis, vasculitis, Raynaud’s phenomenon
painful or painless mouth ulcers.
nasal or vaginal ulcers
generalised arthralgia with early morning stiffness and no swelling is very common
a non-erosive arthritis with joint tenderness and swelling may develop
deformities are unusual but may occur due to ligamentous laxity (Jacoud’s arthropathy)
Myalgia is common but inflammatory myositis occurs in only 5% of patients.
- symptoms of active disease:
Fatigue is common, and difficult to evaluate.
may be associated with: depression, fibromyalgia, secondary to systemic lupus,hypothyroidism,anaemia
fever, malaise, anorexia, weight loss
Pleurisy, often without physical signs, is common in systemic lupus.
Less common: lupus pneumonitis, pulmonary haemorrhage, pulmonary embolism, pulmonary hypertension.
important determinant of the outcome of systemic lupus.
Early nephritis is often asymptomatic.
biopsy is helpful for assessing: severity, nature, extent and reversibility, and is an important guide to treatment and prognosis.
HTN, peripheral edema, glomerulonephritis, renal failure
Pericarditis is common but often asymptomatic.
myocarditis,endocarditis (Libman-Sachs), coronary artery disease.
- Systemic lupus may affect the central and peripheral nervous systems.
- common are: headache, seizures, aseptic meningitis and cerebrovascular accidents, peripheral neuropathy, stroke
- Antiphospholipid antibodies have been implicated in cerebrovascular accidents and chorea.
Lymphopenia (most common manifestation of systemic lupus)
in 95% of SLE,
not specific for SLE,
in 5% to 15% of healthy persons,
seen in many inflammatory, infectious, and neoplastic diseases
- Anti-ds DNA
• seen in 60% of patients with SLE
• highly specific for SLE
• low titer rarely seen in other inflammatory conditions
• strongest clinical association is with nephritis
- Anti-Sm (Smith)
• seen in 10% to 30% of SLE patients
• highly specific for SLE
- Ro/SS-A & La/SS-B
Neonatal lupus syndrome
- Antihistone antibodies:
Drug-induced lupus – hydralazine (anti-hypertensives), methyldopa, procainamide (anti-arrhythmics), isoniazide.
- Patients must be educated about the nature of their disease and the need for therapy.
- Infections should be avoided and treated.
- patients with lupus should be aware that sun exposure may precipitate a disease flare.
- Milder cases with intermittent rashes, arthritis, and other
mucocutaneous features can be treated with steroid creams, NSAIDs, and hydroxychlorochine.
- persistent cases of systemic lupus may need oral corticosteroids
- includes induction therapy to control acute severe manifestations followed by maintenance therapy.
- Corticosteroids are first-line therapy.
- Initial treatment – methylprednisolone by slow i.v. on
3 successive days. Then prednisone p.o. once/day 2-3 weeks. Steroids should always be reduced slowly.
- Chronic disease should be treated with the lowest dose of corticosteroids (5-10 mg) and other drugs that control inflammation (antimalarials, low dose immunosupresants).
- Cyclophosphamide or mycophenolate mofetil (especially in African- Americans) is usually also used for induction therapy.
- In severe renal involvement cyclophosphamide is given in intremittent i.v.
- In CNS lupus or other critical crises (e.g.trombocytopenia) IgG 400 mg/kg for 5 consecutive days may be useful.
- end-stage renal disease can undergo kidney transplantation, as an alternalive to dialysis with a successful outcome, especially if their disease has been in remission.
- Improvement of severe SLE often takes 4 to 12 weeks.
- Thrombosis or embolism of cerebral or pulmonary requires short- term treatment with heparin and longer treatment with warfarin, if the diagnosis of antiphospolipid syndrome is confirmed. The target INR is usually 3.
- oral corticosteroids:
All patients should be closely monitored for atherosclerosis.
blood pressure control diabetes hyperlipidemia.
Use at the lowest possible dose to maintain remission.
- Hypertension: calcium channel blockers and angiotensin converting enzyme inhibitors are the preferred agents, because β-blockers aggravate Raynaud’s phenomenon.
Bisphosphonates are often required in postmenopausal women.
Calcium and vit. D can be used in all age groups.
Hormone replacement therapy should be used with care and is
contraindicated in women with antiphospholipid antibodies who are not anticoagulated.
ACR criteria 1997
MD 1. Malar rash “butterfly rash” 2. Discoid rash
SOAP 3. Serositis 4. Oral ulcers (painless) 5. Antinuclear antibody 6. Photosensitivity (rash in reaction to sunlight)
BRAIN 7. Blood disorder 8.Renal disorder. 9. Arthritis deforming but it is non-erosive (in contrast to RA). 10. Immunologic 11. Neurologic disorder
SLICC Criteria 2012
- Acute cutaneus lupus 2. Chronic cutaneus lupus
- Oral ulcers 4. Nonscaring alopecia
- Synovitis involving 2 or more joints, charakterized by swelling or effusion or tenderness and at least 30 minutes of morning stiffness
- Renal – 500 mg protein/24 h, or red blood cell casts
- Neurologic 9. Hemolytic anemia
- Leukopenia <4,000 / mm3) or limfopenia <1,000/mm3.
- Thrombocytopenia <100,000/mm3
- Immunologic criteria
4.Antiphospholipid antibody positivity
5.Low complement – C3, C4, CH50
6.Direct Coombs’test in the absence of hemolytic anemia
- The patient must satisfy at least 4 criteria, including at
least one clinical criterion and one immunologic criterion or
must have biopsy-proven lupus nephritis in the presence of antinuclear antibodies or anti-dsDNA antibodies
- multifactorial disease.
- genetics: common association with HLA-B8/DR3
- environment: UV radiation, cigarette smoking, infection, vitamin D deficiency
- estrogen, - viral infection.
- characterised by the development of autoantibodies, which arise from failed clearance of apoptotic cells.
- symptoms are due to deposition of immune complexes and the activation of complement.
- a syndrome that occurs in about 10% of babies born to mothers with anti-Ro or anti-La antibodies.
- most common manifestations is a rash induced by ultraviolet light a few days after birth.
- resolves spontaneously if the babies are removed from sunlight.
- A more serious, but much rarer, manifestation of this syndrome is congenital heart block, detected in uterus about 16 – 28 weeks of pregnancy
- chronic multisystemic autoimmune disease of unknown cause with many manifestations.
- more common in women and Afroamerican people.
- age of onset in reproductive yr (13-40)
- Antiphospholipid antibody syndrome (APS):
-multi-system vasculopathy manifested by: recurrent arterial and/or venous thromboses, thrombocytopenia, abortions
-+ve for anticardiolipin antibodies IgG, IgM,
-+ve for lupus anticoagulant
-+ve for antibodies to β2-glycoproteins
-presents with migraine
-The fals- positive test results for syphilis found in many patients with SLE are caused by antiphospholipid antibodies
-primaryAPLS: in the absence of other disease
-secondary APLS: in the connective tissue disease,malignancy,
drugs, and infections (HIV, TB, hepatitis C)
-catastrophic APLS: development within 1wk of small vessel thrombotic occlusion in ≥ 3 organ systems with positive antiphospholipid Ab (high mortality)
-Treatment: warfarin,heparin/low molecular weight heparin ± ASA during pregnancy,catastrophic APLA: high-dose steroids, anti-coagulation, cyclophosphamide, plasmapheresis
- Pregnancy and lupus:
systemic lupus doesn't reduces fertility, but active disease and the presence of antiphospholipid antibody syndrome may increase the risk of:
intrauterine growth retardation,
Contraceptive pills that contain oestrogen, may exacerbate lupus disease or thrombosis.