Connective Tissue Diseases
·
= Collagen vascular diseases
·
Affect many organ systems,
associated with systemic fever and malaise, run a chronic course, respond to
steroids, associated with anaemia of chronic disease and a raised ESR
Systemic Lupus
Erythematosus
·
Non-organ specific autoimmune
vasculitis with positive ANAs
·
Epidemiology:
o Peak age of diagnosis: 30 - 40
o Female:male = 9:1
o Prevalence: 0.2%
o Commoner in pregnancy, Afro-Caribbeans, Asians
o Genetic predisposition: HLA B8, DR3, DR2
·
Presentation (OHCM p 672):
o Gradual or sudden onset
o General: Fever (77%), splenomegaly, lymphadenopathy, extreme fatigue
o Musculo-skeletal symptoms (95%): joint/muscle pain, non-erosive small joint polyarthritis, bone
necrosis, rare joint deformity due to capsular laxity
o Skin (81%): photosensitive butterfly rash (hyperkeratosis, follicular plugging), scarring alopecia,
o Raynaud‟s, purpura, oral ulcers, discoid lupus (3 stage rash: erythema ® pigmented hyperkeratotic oedematous papules ® atrophic depressed lesions), nailfold vasculitis
o Renal (<75%): proteinuria, casts, oedema, uraemia, glomerulonephritis.
o CNS (<18%): Depression, psychosis, fits, cranial nerve lesions,
retinal exudates
o Pulmonary (<48%): Pleurisy (+/- effusion), fibrosing alveolitis, BOOP
o CVS (38%): ÂBP, pericarditis, Libman-Sacks endocarditis
o Blood: Normocytic anaemia (75%), Coombs +ive haemolysis, ¯WCC, ÂINR, ¯platelets,
ÂESR, normal CRP
o Mortality is due to renal failure
·
Pathology: Autoantibodies ®
fibrinoid change ® fibrosis
·
Investigations:
o FBC
o ESR > 20, CRP often low
o 80% are ANA +ive: High dsDNA ANA almost exclusive to SLE (+ive in 40 –
60%)
o 40% are RF +ive
o 30% are anti-Sm positive
o Lupus anti-coagulopathy test
o Antibodies to Ro (SS-A), La (SS-B) and anti-RNP (ribonuclear protein)
help define overlap syndromes
o VDRL false positive in 30%
o Organ/skin biopsy
o Pain disproportionate to radiological damage on X-ray
·
Monitoring:
o BP
o Urinalysis
o FBC, U&E, Complement (C3, C4 – better than ESR)
o dsDNA ANA titres
·
Treatment:
o Sun block creams
o Analgesics/NSAIDs: joint pain, swelling, fever
o Hydroxychloroquine for skin and joint pain. For disease not controlled by NSAIDs SE: retinopathy – check eyes annually
o Prednisolone: higher dose for exacerbations, lower dose for chronic disease – mainstay of treatment
o Cyclophosphamide: either daily or monthly pulse (fewer side effects):
helps renal function more than steroids
o Azathioprine: steroid sparing.
SE: lymphoma
o Cyclosporin or methotrexate
·
Drug Lupus:
o Caused by isoniazid, hydralazine, procainamide, chlorpromazine,
anticonvulsants
o Lung and skin effects greater than renal and CNS
o ENA anti-histone more likely to be positive
o Remits if drug stopped
o Sulfonamindes and the Pill may exacerbate idiopathic SLE
· Discoid lupus = skin involvement only.
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