Flashcards in Rheumatology - Vasculitis Deck (44)
What are the investigations for Wegener's?
- ESR and CRP: typically raised in proportion to disease activity
- renal function: requires close monitoring
- urinalysis: proteinuria, microscopic haematuria and casts
- cANCA: positive in majority of cases
- plain chest X-ray, CT chest, and CT/MRI of the nasal sinuses: check for respiratory tract involvement
- biopsy of affected tissue (nasal, lung or renal): shows necrotising vasculitis with granuloma formation
How is Wegener's managed?
Corticosteroids and cyclophosphamide - first line therapy.
Rituximab (anti-CD20 monoclonal antibody which depletes B cells) may be as effective as cyclophosphamide in inducing remission.
Corticosteroid and cyclophosphamide sparing therapy (e.g. azathioprine, methotrexate) may be used for maintenance therapy.
What is microscopic polyangiitis? What renal disease does it typically cause?
Microscopic polyangiitis is a necrotising vasculitis that predominantly affects the kidneys causing rapidly progressive glomerulonephritis.
Approximately 50% of patients have lung involvement presenting as haemoptysis, pleurisy, or asthma. Frank pulmonary haemorrhage is rare but potentially fatal.
Other features include arthralgia, vasculitis or purpuric rashes, hypertension, mononeuritis multiplex and peripheral neuropathy.
What is leucocytoclastic vasculitis? What is the aetiology of these disease?
This is also caused hypersensitivity vasculitis, and is a group of immune complex mediated vasculitides characterised by acute inflammation of small blood vessels; the multiple lesions tend to be of the same age.
The main clinical feature is palpable purpura which coalesce to form plaques or ecchymoses, especially on the lower limbs. There can also be joint, renal or GIT involvement.
It may be precipitated by exogenous antigens such as drugs (penicillin, sulphonamides, thiazides), foods or infectious organisms. It can also occur as a complication of systemic illnesses, such as connective tissue disorders or malignancies.
What are the 2 clinical syndromes of leucocytoclastic vasculitis?
1) Henloch - Schonlein purpura - most common in young children
2) Serum sickness
What are the clinical features of HSP?
HSP is most common in young children and is a systemic vasculitis characterised by deposition of IgA containing immune complexes. It affects small vessels and is a clinical syndrome of leucocytoclastic vasculitis.
Clinically, haemorrhagic urticaria is seen on the extensor surfaces or the arms, legs and buttocks, with fever, arthralgias and GIT and renal involvement (often similar to an IgA nephropathy).
It can sometimes be post streptococcal in origin. It is associated with antecedent upper respiratory tract infection.
What is serum sickness?
Serum sickness is a syndrome seen experimentally, where rabbits, after serial injections of bovine serum albumin, develop generlised deposition of antigen-antibody complexes in the heart, joints and kidney.
Serum sickness is now rare in humans, but in the past it was caused by therapeutic administration of various antitoxins (foreign serum containing specialised antibodies prepared by immunisation of animals such as horses).
What is Churg-Strauss syndrome?
Chrug-Strauss syndrome is a necrotising vasculitis considered by some to be a variant of PAN. It is characterised by prominent involvement of pulmonary vasculature, marked peripheral eosinophilia and clinical manifestations of asthma.
pANCA positivity is seen in most patients.
Treatment is with corticosteroids and cyclophosphamide.
What is cryoglobulinaemic vasculitis?
This is an immune complex mediated vasculitis usually arising in patients with mixed cryoglobulinaema types I and II. There is a strong associated with hepatitis C infection. Females are affected more commonly than males.
- chronic renal failure, hypertension and leg ulcers: in long standing disease
- liver failure and B cell lymphoma are rare associations
What is Behcets disease?
Behcets is a condition of unknown aetiology characterised by disordered regulation of the inflammatory response with vasculitis of veins and arteries of all sizes, hypercoagulability and neutrophil disorders.
It occurs with greater prevalence in the Middle East and Central Asia. Males are more commonly affected with peak age of onset in the 20s.
What is the clinical presentation of Behcets?
Diagnosis remains predominantly clinical.
- oral aphthous ulcers (occurring on >3 occasions during a 12 month period with >2 of the following "hallmark" features)
- ano-genital ulcers
- erythema nodosum
- relapsing anterior and posterior uveitis
- retinal vasculitis
- arthalgia or non deforming mono or polyarthritis
The International Study Group for Behcets Disease sets the diagnostic criteria.
What differential diagnosis should be considered in Behcets?
- herpes simplex virus: recurrent oro-genital ulceration
- sarcoidosis: erythema nodosum, uveitis and pulmonary involvement
- IBD: oral/ peri-anal ulcers, GI involvement
- seronegative arthritis: arthritis, uveitis
What is Buerger's disease?
Buerger's (or thrombophlebitis obliterans) is an acute inflammation involving small to medium sized arteries of the extremities, extending to adjacent veins and nerves.
It occurs with greater frequency in young men and in the Jewish population. It results in painful ischaemic disease often resulting in gangrene. It is clearly exacerbated by heavy cigarette smoking.