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Connective Tissue Diseases Notes

This is a sample of our (approximately) 6 page long Connective Tissue Diseases notes, which we sell as part of the Rheumatology Notes collection, a MBChB (hons) (1st) package written at University Of Leicester in 2014 that contains (approximately) 20 pages of notes across 6 different documents.

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Connective Tissue Diseases Revision

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Connective Tissue Diseases Systemic Lupus Erythematosus (SLE) A multisystem autoimmune disease in which autoantibodies are produced against a variety of auto antigens Epidemiology Females>males Women of childbearing age Common in Asians and Afro-Caribbean Associated with HLA B8, DR 2+ DR 3 Pathology Abnormalities in
- Cellular and humoral immunity
- Complement system (abnormal clearance of immune complexes)
- Apoptosis Polyclonal B secretion of pathogenic autoantibodies results in formation of immune complexes, which deposit in sites such as the kidney. There is also vasculitis, coagulopathy and tissue inflammation Clinical features Musculoskeletal
- Myalgia, arthritis, arthralgia Neurological

- Headaches, seizures, anxiety/depression Respiratory
- PE, pleurisy, pulmonary hypertension Cardiovascular
- Pericarditis, myocarditis, arrhythmias, hibman-sachs (non-infective) endocarditis Gastrointestinal
- Nausea, abdo. Pain Renal
- Proteinuria, haematuria, nephrotic syndrome Haematological
- Raised ESR, anaemia, thrombocytopaenia Skin
- Vasculitis, malar rash, alopaecia, purpura, photosensitivity, reynauds Constitutional
- Fever, malaise, fatigue, weight loss Classification criteria Need >4 of the following
- Malar rash
- Discoid rash
- Oral ulcers
- Non-erosive arthritis

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Haematological disorders Immunological disorder (anti ds-DNA - 90% have, Anti SM, antiphospholipid) ANA (abnormal titre +ve in 95%) Renal disorders CNS disorders

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Serositis

Investigations Autoantibodies
- Anti-dsDNA
- Anti-Sm
- Anti-phospholipid
- ANA Also may have positive RF Evidence of clinical features e.g anaemia, renal disease, endocarditis Drug-induced Lupus
- A range of drugs can induce SLE
- Known as the 'slow acetylators' e.g isoniazid, methyldopa, chlorpromazine, sulfasalazine
- Mainly affects lungs and skin (rarely CNS or renal disease)
- Remits if drug is removed Management NSAIDS (but increased risk of CVS and renal disease) Acute flare
- High dose prednisolone + IV cyclophosphamide Maintenance
- Low dose steroids + azathioprine Topical sunscreens BP control vital in renal disease

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