Week 2 - G - Medium & Small Vessel Vasculitis - Kawasaki/polyarteritis nodosa (&Scarlet fever) and G.P.A/E.G.P.A/M.P.A/H.S.P Flashcards
What are the two main types of medium vessel vasculitis known as?
Polyarteritis nodosa (PAN) and Kawasaki Disease
Where in the medium sized vessels does the necrotizing inflammatory lesions happen in polyaerteritis nodosa? What liver condition is often associated with polyarteritis nodosa?
In polyarteritis nodosa, the necrotizing inflammatory lesions occur at vessel bifurcations
Hepatitis B or hepatitis C is often associated with polyarteritits nodosa
What is polyarteritis nodosa? * ie what kind of vasculitis * what does it cause
Polyarteritis nodosa is a necrotizing vasculitis that causes aneurysms and thrombosis in medium sized arteries leading to infarction in affected organs with severe systemic symptoms
Symptoms of PAN depend mainly on location and severity of the arteritis and extent of secondary ischemia. Only one organ or organ system may be affected. What organs are typically affected by the vascular inflammation in polyarteritis nodosa?
The kidneys, skin, joints, muscles, peripheral nerves, and gastrointestinal (GI) tract are most commonly affected but any organ can be.
However, the lungs are usually spared.
What are the presenting symptoms of polyarteritis nodosa?
Symptoms depend on the organ affected by the inflamaion of the arteries *
Kidneys - hypertension, haematuria, proteinuria
* Skin - purpurua, livedo reticularis, ulcers
* Joints - arthralgia
* Muscles - myalgia
* Peripheral nerves - mononeuritis multiplex (sensory and motor disturbances in greater than or equal to 2 peripheral nerves)
* GI - malabsorption, pain
How is polyarteritis nodosa diagnosed?
Clinical findings
Biopsy of affected tissue
Arteriography if no clinically involved tissue is available for biopsy
What is the treatment of polarteritis nodosa?
Treatment- control BP
Steroids for mild cases and immunosuppressants if severe eg cyclophosphamide
What patient does Kawasaki disease typically affect?
Kawasaki typically occurs in children under the age of 5 and is a medium vessel vasculitis - inflammation weakens blood vessls leading to aneursyms (outpouching of the vessel)
What are the characterisitc features of Kawasaki disease? (Clue- Warm CREAM)
Child (typically Asian) presents with fever lasting 5+ days + at least 4 of *
* Conjuncitvitis (non-exudative) *
* Rash - dequamative polymprophic rash on trunk
* Erythema palms and soles + swelling
* Adenopathy - cervical lymphadenopathy
* Mucous membrane - dry and strawberry tongue
Restate the presenting features of Kawasaki disease? What is the worrying complication of Kawasaki disease?
Child (typically Asian /=5days and at least 4 of
* Conjunctivitis (non-exudative)
* Rash - desquamative rash on trunk
* Erythema on palms and soles
* Adenopathy - cervical
* Mucosal membrane - red and strwaberry tongue
Coronary artery aneurysms may develop and rupture or cause myocardial infarction
What is the treatment of Kawaski disease?
Treatment is
High dose aspirin and high dose IV immune globulin (IVIG).
Coronary thrombosis may require fibrinolysis or percutaneous interventions. - DO AN ECHO
What is another cause of strawberry tongue? What infection causes it?
Scarlett fever and toxic shock syndrome are other causes of strawberry tongue
Both conditions can be causes by group A b-haemolytic strep (aka streptococcus pyogenes)
What typically precedes scarlet fever? What is the toxin released by streptococcus pyogenes that causes this fever?
Scarlet fever, a predominantly childhood disease, usually follows a pharyngeal streptococcal infection
Scarlet fever is caused by group A streptococcal strains that produce an erythrogenic toxin - causes lysing of RBCs and tissue cells
How does scarlet fever present?
Sandpaper-like red rash develops on the skin (chest, axillae, behind the ears) usually into 2nd day of fever which will desquamate
Child has tender cervical lymph nodes and strawberry tongue
What is the treatment of Scarlet fever?
Treatment is the same as for other GABHS infections - penicillin V (Phenoxymethylpenicillin)
SMALL VESSEL VASCULITIS What can the small vessel vasculitis be divided into?
ANCA positive and ANCA negative small vessel vasculitis
ANCA positive = ANCA associated vasculitis (AAV)
What are the four ANCA positive vasculitis? * Which two have granuloma formation? (A granuloma is a tiny cluster of white blood cells and other tissue)
Graulomatous ANCA positive vasculitis
- * Granulomatosis with polyangiitis (GPA) (aka Wegner’s syndrome)
- * Eosinophilic granulomatosis with polyangiitis (EGPA (aka Chrug-Strauss syndrome)
Non granulomatous ANCA positive vasculitis
- * Microscopic polyangitiis (MPA)
- * Renal limited vasculitis (RLV) - subset of MPA
Which two vasculitis are associated with glomerulonephritis? How do you differentiate between the two?
Both granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) are associated with glomerulonephritis
Can differentiate between the two as MPA does not have granuloma formation
What are the common shared symotoms of the different vasculitis? (not included RLV)
* Fever and weight loss
* A raised, non blanching purpuric rash
* Arthralgia/arthritis
* Mononeuritis multiplex
* Glomerulonephritis
* Lung opacities on x-ray
What are the presenting features of GPA? Best way to think is the symptoms that related to each organ/system it affects
ENT symptoms- nosebleeds, recurrent sinusisits, nasal crusting and over time loss of nose cartilage
Respiratory symptoms - haemoptysis
Renal - haematuria / proteinuria due to glomerulonephritis
What is the nasal deformity that occurs due to GPA?
Due to the loss of nasal cartilage due to ischaemia caused by GPA, there is the characteristic ‘saddle nose’ deformity that can be seen in these patients
What may be seen on CXR in Wegener’s?
Cavitating lung lesions may be seen on CXR
If proteinuria or haematuria is found on urinalysis, what needs to be carried out?
If these are present, consider carrying out a renal biopsy for the patient to understand the extent of the renal involvement
What antibodies are tested for in GPA? What other vasculitis are these antibodies seen in?
Test for c-ANCA direct against PR3 * (cytoplasmic - anti-neutrophil cytoplasmic antibodies specific for serine proteinase 3)
c-ANCA PR3 antibodies are also seen in microscopic polyangiitis
What is EGPA characterised by?
This condiiton is characterised by late onset asthma, rhinitis and a raised peripheral blood eosinophil count
As well as vasculitis affecting the lung, nerves heart and skin
Neurological symptoms such as mono-neuritis multiplex are common
Which antibodies are found in EGPA? What other vasculitis are these commonly found in?
in EGPA P-ANCA directed against MPO
* (perinuclear-anti-neurtrophil cytoplasmic antiboies directed against myeloperoxidase)
p-ANCA MPO are commonly found in microscopic polyangiitis
How does MPA present? What is the most important complication which occurs in up to 90% of patients?
MPA presents the similar to Wegener’s without the upper airway disease (no recurrent sinusitis or nasal deformity)
Will have Respiratory symptoms - haemoptysis
Renal - haematuria / proteinuria due to glomerulonephritis
Most important complication is the rapidly progressive glomerulonephritis
What are the main differences between MPA and GPA? What antibodies are seen in bloods for MPA?
MPA - does not have granulomatous formation and does not affect upper airways
MPA - most commonly p-ANCA directed against MPO also c-ANCA directed against PR3 positive sometimes
It is important to note that ANCA is negative in a small proportion of all these conditions so it cannot be relied upon solely to confirm the diagnosis What other investigations / tests are useful for confirming the diagnosis in a small vessel vasculitis?
ESR, PV and CRP and raised Anaemia of chronic disease is common.
U+E looking for renal involvement
Urinalysis (looking for renal vasculitis)
CXR
Biopsy of an affected area e.g. skin or kidney is often helpful in confirming the diagnosis
What are most cases of ANCA associated vasculitis treated with?
Most cases of ANCA associated vasculitis require treatment with IV steroids and cyclophosphamide due to their aggressive disease course
What is the acute immunoglobulin mediate ANCA negative small vessel vasculitis known as? It typically affects children Which immunoglobulin is it mediated by?
This is Henoch-Schonlein purpura (HSP)- it is an acute IgA mediated small vessel vasculitis
Henoch-Schönlein purpura (HSP) is an acute immunoglobulin A (IgA)–mediated disorder characterized by a generalised vasculitis involving the small vessels of the skin, the gastrointestinal (GI) tract, the kidneys, the joints, and, rarely, the lungs and the central nervous system (CNS). How does HSP typically present? and in what age group?
HSP typically presents as
Child with a purpuric rash on the buttocks and lower limbs, with abdo pain and vomiting and joint pain
There may be glomerulonephritis It typically occurs in children aged 2-11 years
What typically precedes HSP?
HSP is typically preceded by a respiratory tract infection of GI infection predating hte condition by about 2 weeks
What is the most common bacterial infection to predate Henoch Schonlein purpura?
Group A beta haemolytic step infection - strep pyogenes infection
What is the treatment for henoch schonlein purpura?
Usually self limiting with symptoms resolving in roughly 8 week - provide analgesia for symptomatic relief (paracetamol) (dont take NSAIDs as can harm the kidneys)
Corticosteroids may be given if severe or in adults
