476906
Systemic lupus erythematosus
- A non-organ-specific autoimmune disease in which autoantibodies are
produced against a variety of autoantigens (eg ANA ). Immunopathology
results in polyclonal B -cell secretion of pathogenic autoantibodies causing
formation of immune complexes depositing in sites such as the kidneys
- Epidemiology
- F:M : 9:1. Prevalence: ~0.2%. Common in: Pregnancy;
African-Americans; Asians— and if HLA B 8 , DR 2 or DR 3
+ve. ~10% of relatives of SLE patients are affected. It is a
remitting and relapsing illness, with peak age at diagnosis
being 30– 40yrs
- F:M : 9:1. Prevalence: ~0.2%. Common in: Pregnancy;
African-Americans; Asians— and if HLA B 8 , DR 2 or DR 3
+ve. ~10% of relatives of SLE patients are affected. It is a
remitting and relapsing illness, with peak age at diagnosis
being 30– 40yrs
- Symptoms & Signs
- Splenomegaly, lymphadenopathy, alopecia, recurrent
abortion, retinal exudates, fibrosing alveolitis, myalgia,
anorexia, myositis, migraine
- Splenomegaly, lymphadenopathy, alopecia, recurrent
abortion, retinal exudates, fibrosing alveolitis, myalgia,
anorexia, myositis, migraine
- Investigations
- Raised ESR (think of SLE whenever someone has a
multisystem disorder and ESR ^ but CRP «). Immunogenetics
>95% are ANA +ve. High titer of antibodies directed against
double-stranded DNA is nearly exclusive to SLE . Its absence
does not exclude it. 11% have false +ve syphilis serology from
IgG anticardiolipin antibodies. Antibodies to Ro ( SS - A ), La (
SS - B ), and U 1 ribonuclear protein help define overlap
syndromes (eg with Sjögren’s)
- Monitoring activity
- 3 best tests: (1) ESR (2) Complement: C 3 |,
C 4 |; C 3 d^ (denotes degradation products
of C 3 , hence it moves in the opposite way)
(3) Double-stranded (antiDS ) DNA antibody
titers. Others: Urinalysis, electrolytes, FBC
- 3 best tests: (1) ESR (2) Complement: C 3 |,
C 4 |; C 3 d^ (denotes degradation products
of C 3 , hence it moves in the opposite way)
(3) Double-stranded (antiDS ) DNA antibody
titers. Others: Urinalysis, electrolytes, FBC
- Raised ESR (think of SLE whenever someone has a
multisystem disorder and ESR ^ but CRP «). Immunogenetics
>95% are ANA +ve. High titer of antibodies directed against
double-stranded DNA is nearly exclusive to SLE . Its absence
does not exclude it. 11% have false +ve syphilis serology from
IgG anticardiolipin antibodies. Antibodies to Ro ( SS - A ), La (
SS - B ), and U 1 ribonuclear protein help define overlap
syndromes (eg with Sjögren’s)
- Drug induced lupus
- This can be caused by
isoniazid, hydralazine (in slow
acetylators), procainamide,
chlorpromazine, minocycline,
TNF inhibitors. Lung and skin
signs prevail over renal and
CNS signs. It remits if the drug
is stopped. Sulfonamides and
birth control pills may
exacerbate idiopathic SLE
- This can be caused by
isoniazid, hydralazine (in slow
acetylators), procainamide,
chlorpromazine, minocycline,
TNF inhibitors. Lung and skin
signs prevail over renal and
CNS signs. It remits if the drug
is stopped. Sulfonamides and
birth control pills may
exacerbate idiopathic SLE
- Antiphospholipid syndrome
- Either primary or secondary to SLE (20-30%)
- Antiphospholipid antibodies,
anticardiolipin antibody & lupus
anticoagulants are present
- Features of CLOT: Coagulation defect, Livedo
reticularis, Obstetric recurrent miscarriage,
Thrombocytopenia (low platelets).
Prothrombotic tendency,
- Treatment: Low dose
aspirin or warfarin if
recurrent thromboses
- Either primary or secondary to SLE (20-30%)
- Treatment
- Refer to a rheumatologist. NSAID s . Sun-block creams. Hydroxychloroquine if
joint or skin symptoms are uncontrolled by NSAID s. SE : Irreversible
retinopathy— annual ophthalmic referral is recommended. High-dose prednisone
is kept for severe episodes, may be combined with other immunosuppressive
agents (eg cyclophosphamide), or ‘steroid-sparing agents’, eg azathioprine,
methotrexate, or mycophenolate. Low-dose steroids may be of value in chronic
disease. Cyclophosphamide is indicated for some nephritides. Azathioprine can
be a ‘steroid-sparer’ SE : Lymphoma. Renal transplantation may be needed;
nephritis recurs in ~50%, on biopsy, but is a rare cause of graft failure.
- Refer to a rheumatologist. NSAID s . Sun-block creams. Hydroxychloroquine if
joint or skin symptoms are uncontrolled by NSAID s. SE : Irreversible
retinopathy— annual ophthalmic referral is recommended. High-dose prednisone
is kept for severe episodes, may be combined with other immunosuppressive
agents (eg cyclophosphamide), or ‘steroid-sparing agents’, eg azathioprine,
methotrexate, or mycophenolate. Low-dose steroids may be of value in chronic
disease. Cyclophosphamide is indicated for some nephritides. Azathioprine can
be a ‘steroid-sparer’ SE : Lymphoma. Renal transplantation may be needed;
nephritis recurs in ~50%, on biopsy, but is a rare cause of graft failure.
- Revised criteria for diagnosing SLE
- If 4 out of the 11 are present serially or simultaneously
- 1. Malar rash (butterfly rash): Fixed erythema,
flat or raised, over the malar eminences, tending
to spare the nasolabial folds
- 2. Discoid rash: Erythematous raised patches with
adherent keratotic scaling and follicular plugging ±
atrophic scarring. Think of it as a 3-stage rash affecting
ears, cheeks, scalp, forehead, and chest: Erythema ¬
pigmented hyperkeratotic edematous papules ¬ atrophic
depressed lesions
- 3. Photosensitivity on exposed skin representing unusual reaction to light
- 4. Oral ulcers: Oral or nasopharyngeal ulceration
- 5. Arthritis: Non-erosive arthritis involving 2 or more peripheral joints, characterized by tenderness,
swelling, or effusion. Joint involvement is seen in 90% of patients. Deforming arthropathy may occur
due to capsular laxity (Jaccoud’s arthropathy). Aseptic bone necrosis also occurs
- 6. Serositis: (a) Pleuritis (b) Pericarditis.
- 7. Renal disorders: (a) Persistent proteinuria >0.5g/d (or >3+ on dipstix) OR (b) Cellular casts— may
be red cell, granular, or mixed
- 8. CNS disorders: (a) Seizures, in the absence of causative drugs or known metabolic imbalance, eg
uremia, ketoacidosis, OR (b) Psychosis in the absence of causative drugs/metabolic derangements,
as above
- 9. Hematological disorders: (a) Hemolytic anemia with reticulocytosis OR (b) Leukopenia, ie WBC
<4Þ´10 9 / L on ³2 occasions OR (c) Lymphopenia, ie <1.500Þ´10 9 / L on ³2 occasions OR (d)
Thrombocytopenia, ie platelets <100Þ´10 9 / L in the absence of a drug effect
- 10. Immunological disorders: (a) AntiDNA antibody to native DNA in abnormal titer OR (b) Anti-Sm
antibody to Sm nuclear antigen OR (c) Antiphospholipid antibody +ve based on: (1) an abnormal
serum level of IgG or IgM anticardiolipin antibodies, (2) positive result for lupus anticoagulant using a
standard method, or (3) false positive serological test for syphilis +ve for >6 months and confirmed
by Treponema pallidum immobilization or fluorescent treponemal antibody absorption tests
- 11. Antinuclear antibody: Positive in 95%
- 11. Antinuclear antibody: Positive in 95%
- 10. Immunological disorders: (a) AntiDNA antibody to native DNA in abnormal titer OR (b) Anti-Sm
antibody to Sm nuclear antigen OR (c) Antiphospholipid antibody +ve based on: (1) an abnormal
serum level of IgG or IgM anticardiolipin antibodies, (2) positive result for lupus anticoagulant using a
standard method, or (3) false positive serological test for syphilis +ve for >6 months and confirmed
by Treponema pallidum immobilization or fluorescent treponemal antibody absorption tests
- 9. Hematological disorders: (a) Hemolytic anemia with reticulocytosis OR (b) Leukopenia, ie WBC
<4Þ´10 9 / L on ³2 occasions OR (c) Lymphopenia, ie <1.500Þ´10 9 / L on ³2 occasions OR (d)
Thrombocytopenia, ie platelets <100Þ´10 9 / L in the absence of a drug effect
- 8. CNS disorders: (a) Seizures, in the absence of causative drugs or known metabolic imbalance, eg
uremia, ketoacidosis, OR (b) Psychosis in the absence of causative drugs/metabolic derangements,
as above
- 7. Renal disorders: (a) Persistent proteinuria >0.5g/d (or >3+ on dipstix) OR (b) Cellular casts— may
be red cell, granular, or mixed
- 6. Serositis: (a) Pleuritis (b) Pericarditis.
- 5. Arthritis: Non-erosive arthritis involving 2 or more peripheral joints, characterized by tenderness,
swelling, or effusion. Joint involvement is seen in 90% of patients. Deforming arthropathy may occur
due to capsular laxity (Jaccoud’s arthropathy). Aseptic bone necrosis also occurs
- 4. Oral ulcers: Oral or nasopharyngeal ulceration
- 3. Photosensitivity on exposed skin representing unusual reaction to light
- 2. Discoid rash: Erythematous raised patches with
adherent keratotic scaling and follicular plugging ±
atrophic scarring. Think of it as a 3-stage rash affecting
ears, cheeks, scalp, forehead, and chest: Erythema ¬
pigmented hyperkeratotic edematous papules ¬ atrophic
depressed lesions
- 1. Malar rash (butterfly rash): Fixed erythema,
flat or raised, over the malar eminences, tending
to spare the nasolabial folds
- If 4 out of the 11 are present serially or simultaneously
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