Connective Tissue Disorders Flashcards
What are symptoms of Marfan’s?
Pectus excavatum
Aortic dilatation
High arched palate
Dislocated lens in eye
Which HLA is associated with SLE?
DR2 and DR3
What causes thrombosis in SLE?
Antiphospholipid ABs
What dermatology is associated with SLE?
Photosensitive Malar rash Generalised erythema Bullous LE Chronic- Discoid rash, scarring alopecia, lupus profundus
What are neph effects of SLE?
Nephosis and nephritis
HTN
Proteinuria
Renal failure
What cardiac and resp SLE features?
PE
Pleural effusions
Pericarditis/ myocarditis
MI
What haematological SLE features?
Neutropaenia
Thrombocytopaenia
Lymphocytopaenia
Anaemia
Psych and Neuro SLE features?
Aseptic meningitis polyradiculopathy autonomic disorder CV disease Headache Neuropathy Chorea MG CN issues seizures cognitive dysfunction anxiety psychosis
What are feautres of antiphospholipid syndrome?
arterial and venous thrombosis
Recurrent foetal loss
Thrombocytopaenia
What are antibodied present in antiphospholipid syndrome?
Cardiolipin antibodies IgG IgM
Antiphospholiipid AB
Must be positiive on 2 different occasions 12 weeks apart
What is livedo reticularis?
Purple lace like rash in SLE and autoimmune conditions
What are the autoabs seen in SLE?
Anti DsDNA is specific (ANA to screen for it)
What non medical management of SLE is needed?
Education UV protection Manage in pregnancy Assess for organ damage Manage atheroschlerosis factors
What drugs are used in SLE?
Topical steroid NSAIDs Antimalarials Steroids Cytotoxics- Azathioprine, methotrexate Anticoagulants Biologicals that target B cells: Rituximab or Belimumab Stem cell transplant
What is used to treat Raynaud’s
Nifedipine
Sildenafil
Fluoxetine
What does CREST stand for in SS?
Calcinosis Raynaud's Esophageal dysmotility Schlerodactyly Telangiecstasia
What is schleroderma?
Hardened thick skin on fingers
What is sclerodactyly?
Swollen fingers and above
What autoab is present in difffuse SS?
anti scl70 and RNA Polymerase III
Which autoab is present in limited cutaneous SS?
Anti centromere AB
What is the presentation of limited cutaneous SS?
Long hx of Raynaud’s
Schlerodactyly
Pulmonary arterial hypertension
What needs to be tested annually in limited cut SS? (CREST)
Echo and pulmonary function tests
What are other skin manifesations of CREST?
Digital ulcers, hair loss, pruritis, oedema, fingertip pitting, calcinosis, skin thickening, hyper or depigmentation, joint stiffness that progresses to contractures
What are features of diffuse SS?
Fatigue, weight loss, athralgia and contractures, myocarditis, pericardial effusion, renal failure
What is the medical management for SS?
Raynauds treatment PPI for GOR for life ACEi to protect kidneys Annual Echo for PAH Skin oedema- cytotoxics or nothing Pulmonary fibrosis- cyclophosphamide
What autoabs are there is Sjogrens?
Anti Ro and anti La are diagnostic
What causes Sjogren’s?
Primary
Or secondary to SLE, RA, SS, PBC or other autoimmune conditions
What are clinical features of Sjogren’s?
Dry eyes Dry mouth Rash Vasculitis Renal tubule acidosis Interstitial lung disease Gluten sensitivity Lymphoma risk
What would you US in sjogrens?
Salivery glands
How do you manage sjogrens?
Tear and saliva replacement
Hydroxychloroquine for fatigue, myalgia and rashes
Corticosteroids or immunosupression for organ threatening symptoms
What is polymyositis?
Chronic autoimmune muscle inflammation
What is dermatomyositis?
Chronic autoimmune inflammation of skin and muscles
What is diagnostic for polymyositis and dermato?
CK (Creatine Kinase) Other causes of raised CK: Rhabdomyositis AKI MI Statins Exercise
Polymyositis and Dermato are paraneoplastic to which cancer?
Lung
Breast
Ovarian
Gastric
How does polymyositis present?
Muscle pain and fatigue
Bilateral proximal muscles
Mostly shoulder and girdle pain developing over weeks
What skin ailments present with dermatomyositis in addition to polymyositis symtoms?
Gottron lesios (scaly erythematous patches on knuckles, elbows and knees)
Photosensitive erythematous rash on back, shoulders and neck
Purple rash on face and eyelids
Preorbital oedema
Subcut calcinosis
What autoabs are presetn in polymyositis?
Anti Jo-1 abs (polymyo)
Anti Mi-2 (dermato)
ANA in dermatomyositis
How is polymyositis diagnosed?
Clinlcally Elevated CK Autoabs EMG Muscle biopsy definitive
How is polymyositis managed?
PET scan OT and physio Corticosteroids Immunosupression IVIg Biologicals -Infliximab
What is SOAP BRAIN MD for SLE?
Serositis (pleuritis, pericarditis) Oral Ulcers Arthritis Photosensitivity Blood low- thrombocytopaenia, anaemia Renal protein (nephrotic syndrome) ANA Immunological (DsDNA etc) Neurological (psych and neuro) Malar Rash Discoid Rash
