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Chapter: Medicine Study Notes : Musculo-Skeletal

Systemic Lupus Erythematosus - Connective Tissue 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.

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%): ­BP, pericarditis, Libman-Sacks endocarditis 

o  Blood: Normocytic anaemia (75%), Coombs +ive haemolysis, ¯WCC, ­INR, ¯platelets, ­ESR, 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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