Rheum 2 Flashcards
What is the pathophysiology behind systemic vasculitis?
- inflammation of the vessel wall
- inflammation and necrosis of blood vessel walls with subsequent impaired blood flow
i) Vessel wall destruction - aneurysm, rupture and stenosis
ii) endothelial injury - thrombosis +ischaemia/infarction of dependent tissues
What are some examples of large vessel vasculitis?
GIant-cell arteritis/polymyalgia rheumatica
What are some examples of medium-vessel vasculitis?
classical polyarteritis nodose (PAN)
kawasaki’s
What are some examples of small-vessel vasculitis?
ANCA-associated - microscopic polyangitism granulomatosis
ANCA-negative - essential cryoglobulinaemiam cutaneous leucocytoclastic vasculitis
What are all systemic vasculitis associated with?
Anaemia
Raised ESR
Subacute infective endocarditis, RA, SLE, scleroderma, polymyositis
What are some risk factors for polymyalgia rheumatica?
Affect those over 50
Females
SLE
Polymyositis
How does polymyalgia rheumatic present?
sudden onset of severe pain and stiffness of the shoulder, neck, hips and lumbar spine
worse in the morning - 30 mins to several hours
mild polyarthritis of peripheral joints
fatigue, fever, weight loss and depression
What investigations should you run in polymyalgia rheumatica?
CRP - raised
ESR - >40
ALP - raised
CK - normal (differentiate from myositis)
How would you treat polymyalgia rheumatica?
Oral Pred
What crystals do you find in crystal arthropathies?
monosodium urate crystals - needle-shaped urate crystals, negatively bifringent under polarised light
calcium pyrophosphate crystals - small rhomboid brick-shaped pyrophosphate crystals, positively bifringent under polarised light
What is the pathophysiology behind crystal arthropathies?
Neutrophils ingest the crystals and initiate a pro-inflammatory reaction
What are risk factors for Gout?
Male
High alcohol (beer > spirits > wine)
Purine rich food (red meat, liver, seafood)
High fructose intake
High saturated fat
Low dose aspirin
*Ischaemic heart disease
*Diabetes
*Renal - defective URAT1 transporter, high insulin - lower urate excretion
*increased production of uric acid - increased purine turnover, leukaemia, carcinoma, psoriasis
How would gout present?
sudden onset, agonising pain, swelling and redness of the first MTP
- normally one joint but can be polyarthritic
- attack precipitated by excess food, alcohol, dehydration, diuretic therapy, cold, traum or sepsis
Tophaceous gout - persistently high levels of uric acid - tophi in skin, joints, ear, fingers or achilles
In gout, what do tophi do to bone?
Release enzymes and cause erosions to bone forming circular punch-like holes
How would investigate Gout?
Arthrocentesis with synovial fluid analysis - strongly negative bifringent crystals under polarised light
Uric acid level
X-ray of affected joint
How would you manage gout?
Acute - NSAIDs, colchicine, corticosteroid
Recurrnt - Allopurinol, NSAIDs
What is pseudogout?
deposition of calcium pyrophosphate crystals on joint surface
What are some causes/risk factors for pseudogout?
old age diabetes osteoarthritis joint trauma metabolic disease - hyperparathyroidism, haemochromatosis
How would pseudogout present?
acute synovitis that resembles gout but more common in elderly women
usually KNEE or WRIST
very painful, acute hot swollen wrist or knee
hot joint and fever - can be mistaken for septic arthritis (steroid effect can be devastating)
How would you investigate pseudogout?
arthrocentesis with synovial fluid analysis - positive intracellular birefringent rhomboid-shaped crystals, fluids often bloody
X-ray
Serum calcium and parathyroid hormone - exclude hyperparathyroidism
Iron studies - exclude haemochromatosis
How would you treat pseudogout?
intra-articular corticosteroids - dexamethasone
NSAIDs
Colchicine
Systemic corticosteroids - prednisolone
What is Paget’s disease of bone? What are causes /RFs?
focal disorder of bone remodelling
incidence increases with age - rare under 40
females
latent viral infection may be cause
fam hx
How does Paget’s disease of bone present?
Pelvis, lumbar spine, femur, thoracic spine, skull and tibia Maj. Asymp Bone pain Joint pain Bowed tibia Nerve compression - deafness, paraparesis high-output cardiac failure osteosarcoma
How would you investigate Paget’s disease of bone?
X-ray
Bone scan
Serum alk phos
Bone specific alk phos
Calcium
Procollagen 1 N-terminal peptide (P1NP) - marker of bone formation - initially elevated
C-terminal propeptide of type 1 collagen (CTX) - marker of bone resorption - initially elevated
How would you treat Paget’s disease of bone?
Bisphos - zolendronic acid
Physio
What is osteomalacia? rickets?
Normal amount of bone but its mineral content is LOW - defective mineralisation
Rickets - defective mineralisation during bone growth at the epiphyseal growth plate
Both clinical manifestations of profound vitamin D deficiency