Rheumatology (3) Flashcards
Demographics (epidemiology) of Giant Cell Arthritis
- Common in elderly (rare <55)
- Associated with Polymyalgia Rheumatica in 50%
Features of Temporal Arteritis
- Systemic signs: fever, malaise, fatigue
- Headache
- Temporal artery and scalp tenderness
- Jaw claudication
- Amaurosis fugax
- Prominent temporal arteries ± pulsation
Management and Ix of Temporal arteritis
If suspected Do ESR and start prednisolone 40-60mg/d PO
- ↑↑ESR and CRP
- ↑ALP
- ↓Hb (normo normo), ↑Plats
- Temporal artery biopsy: but skip lesion occur
Continuing Rx
- Taper steroids gradually, guided by symptoms and ESR
- PPI and alendronate cover (~2yr course usually)
Presentation of Polymyalgia Rheumatica
- >50yrs old
- Severe pain and stiffness in shoulders, neck and hips
- Sudden / subacute onset
- Symmetrical
- Worse in the morning: stops pt. getting out of bed
- No weakness (different from myopathy or myositis)
- ± mild polyarthritis, tenosynovitis and carpal tunnel syndrome
- Systemic signs: fatigue, fever, wt. loss
- 15% develop Giant Cell Arteritis
Ix of Polymyalgia Rheumatica
- ↑↑ESR and CRP (+ ↑plasma viscosity)
- ↑ALP
- Normal CK
Management of Polymyalgia Rheumatica
- Prednisolone 15mg PO: taper according to symptoms and ESR
PPI and alendronate cover (~2yr course usually)
Another name for Takayasu’s Arteritis
Pulseless Disease
Epidemiology of Takayasu’s arteritis
- Female Japan/Asian
- Sex: F>M
- Age: 20-40yrs
Features of Takayasu’s Arteritis
- Constitutional symptoms: fever, fatigue, wt. loss
- Weak pulses in upper extremities
- Visual disturbance
- HTN
Management of Takayasu’s arteritis
Steroids
Epidemiology of Polyarteritis Nodosa
- rare in UK
- M>F=2:1
- young adults
What’s Polyarteritis Nodosa
- vasculitis affecting medium-sized arteries with necrotizing inflammation leading to aneurysm formation
- more common in middle-aged men and is associated with hepatitis B infection
Features of Polyarteritis Nodosa
- fever, malaise, arthralgia
- weight loss
- hypertension
- mononeuritis multiplex, sensorimotor polyneuropathy
- testicular pain
- livedo reticularis
- haematuria, renal failure
- perinuclear-antineutrophil cytoplasmic antibodies (ANCA) are found in around 20% of patients with ‘classic’ PAN
- hepatitis B serology positive in 30% of patients
Management of Polyarteritis Nodosa
Prednisolone + cyclophosphamide
What’s Wegener’s Granulomatosis?
- Necrotizing granulomatous inflammation and small-vessel vasculitis
- URTI, LRTI and Kidneys problems
Features of Wegener’s Granulomatosis
URTI
- Chronic sinusitis
- Epistaxis
- Saddle-nose deformity
LRTI
- Cough
- Haemoptysis
- Pleuritis
Renal
- Rapidly Progressing Gromeluronephritis
- Haematuria and proteinuria
Other
- Palpable purpura
- Ocular: conjunctivitis, keratitis, uveitis
Ix for Wegener’s Granulomatosis
- cANCA
- Dipstick: haematuria and proteinuria
- CXR: bilateral nodular and cavity infiltrates
Another name for Wegener’s Granulomatosis
Granulomatosis with polyangitis
Management of Wegener’s Granulomatosis
- steroids
- cyclophosphamide (90% response)
- plasma exchange
- median survival = 8-9 years
Granulomatosis with polyangiitis vs Churg-Strauss
Another name for Churg-Strauss syndrome
Eosinophilic granulomatosis with polyangiitis
What’s Churg-Strauss Syndrome?
ANCA associated small-medium vessel vasculitis
Features of Churg - Strauss syndrome
- late onset asthma
- blood eosinophilia (e.g. > 10%)
- paranasal sinusitis
- mononeuritis multiplex
- pANCA positive in 60%
Leukotriene receptor antagonists may precipitate the disease
What’s Henoch-Schonlein Purpura
- IgA mediated small vessel vasculitis
- degree of overlap with IgA nephropathy (Berger’s disease)
- usually seen in children following an infection
Features of Henoch-Schonlein purpura
- Children 3-8yrs
- Post-URTI
- Palpable purpura on buttocks
- Colicky abdo pain
- Arthralgia
- Haematuria
Management of Henoch-Schonlein purpura
- analgesia for arthralgia
- treatment of nephropathy is generally supportive
- inconsistent evidence for the use of steroids and immunosuppressants (?)
Prognosis in Henoch Schonlein Purpura
- usually excellent, HSP is a self-limiting condition, especially in children without renal involvement
- around 1/3rd of patients have a relapse
What’s Goodpasture Syndrome?
- rare condition associated with both pulmonary haemorrhage and rapidly progressive glomerulonephritis
- caused by anti-glomerular basement membrane (anti-GBM) antibodies against type IV collagen
Epidemiology of Goodpasture syndrome
- more common in men (sex ratio 2:1)
- bimodal age distribution (peaks in 20-30 and 60-70 age bracket)
- associated with HLA DR2
Features of Goodpasture syndrome
- pulmonary haemorrhage
- followed by rapidly progressive glomerulonephritis
Ix in Goodpasture Syndrome
- renal biopsy: linear IgG deposits along the basement membrane
- raised transfer factor secondary to pulmonary haemorrhages
- X-ray: bilateral lower zone infiltrates (due to pulmonary haemorrhage)
Management of Goodpasture syndrome
- plasma exchange (plasmapheresis)
- steroids
- cyclophosphamide
