Multi-system Autoimmune Disease Flashcards
Spondyloarthropathy includes?
Psoriatic arthritis
Enteropathic arthritis
Ankylosis spondiltis
Reactive arthritis
All serum negative no rheumatoid factor
STIR sequence?
Cuts out fat and highlights inflammation
Peripheral manifestation of spondyloarthropathy?
Uveitis
Tendinitis
Psoriasis
What is the average delay in diagnosing ankylosis going spondylitis?
8 years
Prevalence of ankylosing spondilitis?
0.2-1%
Ratio of ankylosing spondilitis in men and women?
3:1, highest in Caucasians
Genetic link for ankylosing spondilitis?
Over 90% with it have HLA B27
And there’s a 16 x increased risk If family history of HLA B27
Inflammatory back pain questions?
Age of onset less than 40
Insidious onset
Improvement with exercise
No improvement at rest
Pain at night
How would you manage spondyloarthropathy?
Physiotherapy/ hydrotherapy NSAIDs Glucocorticoid injections in joints Synthetic DMARDs- for peripheral Biological DMARDs Surgery
Signs of vasculitis?
Stroke, MI, hypertension, bloody stool. Abdominal pain, arthritis, palpable pupura, fever, headache, weight loss, gloerular nephritis, bloody cough, nose bleeds, reduced visual acuity, muscle pain
What are little red marks on hands called? Sign of vasculitis?
Petechiae
What is the most common vasculitis in over 50s?
Giant cell arthritis, risk of permanent sight loss, inflammation of external carotid arteries (temporal)
Differential diagnosis of GCA?
Over 50 Acute Headache Jaw or scalp tenderness Claudication of tongue or jaw (pain when chewing) New visual symptoms Raised CPR/ESR
How would you confirm GCA?
US- halo sign,
Temporal artery biopsy (giant cells)
What is treatment for GCA?
High dose steroids
If you have large vessel vasculitis what would symptoms be?
Fever weight loss fatigue, because inflammation cuts of blood supply
Weak reduced pulses
Limb claudication
Do an angiogram or PET-CT
Investigations for vasculitis?
Raised ESR/CRP Urine dip: protein, blood or cast cells Chest abdomen imaging, Vessel wall imaging Tissue biopsy PET CT Auto antibodies, anti neutrophil cytoplasmic antibodies ANCA, anti glomerular basement membrane GBM Infection screen, HIV, hepatitis B/C, TB , varicella
How would you treat vasculitis?
Steroids high dose Manage side effects of steroids Immunosuppression Biological agents Synthetic DMARDs
CTD symptoms?
Raynaud’s phenomenon, ulcers of nails
Scarring alopecia
Incidence of systemic sclerosis?
1 to 20/ million
What is pathophysiology of systemic sclerosis?
Microvasculopathy, immune activation (anti nuclear antibodies), upregulation of collagen production causing fibrosis of tissues
What are the limited signs of systemic sclerosis?
Calcinosis- calcium deposition in fingers and toes
Raynauds
Oesophageal dysmotility
Sclerodactylyl- thickening of skin
Telangiectasia - smaller capillaries broken
What are the diffuse signs of systemic sclerosis?
Multi-organ- interstitial Lung disease, myocardial disease, pulmonary hypertension, renal crisis, GI disease
SLE manifestations?
Malar rash
Jaccoud’s arthropathy- deformity of hands
Idiopathic inflammatory myositis signs?
Proximal, symmetrical Painless muscle weakness
What is incidence of idiopathic inflammatory myositis?
2to 8 per million
What are the clinical bio markers you would look for in idiopathic inflammatory myositis?
Elevated muscle enzymes CK, LDH, AST/ ALt
What are the investigations for IIM
MRI, muscle biopsy, EMG
What percentage does IIM overlap with other autoimmune diseases?
15-20%
Dermatomyositis signs?
Gottrons papules Nailfolds inflammed Joint inflammation Muscle weakness myalgia Dysphagia Lung fibrosis Associated with malignancy Periorbital helitrope rash
