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Connective Tissue Diseases Notes

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This is an extract of our Connective Tissue Diseases document, which we sell as part of our Rheumatology Notes collection written by the top tier of University Of Leicester students.

The following is a more accessble plain text extract of the PDF sample above, taken from our Rheumatology Notes. Due to the challenges of extracting text from PDFs, it will have odd formatting:

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



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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