Clinical Presention And Evaluation Of Vasculitis Flashcards
Vasculitis broad details
Is considered a syndrome since it encompasses a wide variety of different issues and multiples organs can be affected.
- all issues deal with damage to vessels (usually arteries/capillaries)
- can occur in every organ system, but can also be limited to only one organ
- can be caused by an ischemic injury
- is rare and often presents with delayed diagnosis
- can be primary or secondary to other diseases
- widespread presentation (minimal -> life threatening)
Polyangiitis
Vasculitis in small vessels
Possible causes of large vessel vasculitis
Takayasu arteritis
Giant cell arteritis
Possible causes of Medium vessel vasculitis
Polyarteritis Nodosa
Burgers syndrome
Kawasaki disease
Possible secondary causes of vasculitis syndromes
Drug-induced
Hepatitis B/C associated
Cancer associated
SLE associated
Sarcoidosis vasculitis
RA associated
What are the three anti neutrophilic cytoplasmic antibody (ANCA) associated vasculitis causes?
Microscopic polyangiitis
Granulomatosis w/ polyangiitis
Eosinophillic granulomatosis w/ polyangiitis
Specific Clinical feature of vasculitis in post-capillary venules
Palpable purpura
Specific clinical features of vasculitis in glomerular capillaries
Haematuria
RBCs in urine
Proteinuria
Declined renal function
Specific clinical features of vasculitis in pulmonary capillaries
Lung hemorrhage w/ severe dyspnea
Widespread alveolar shadowing on chest radiography
Specific clinical features of vasculitis in extra cranial branches of the carotid artery
Temporal artery = temporal severe headache
Ophthalmic artery = partial blindness
Facial artery and its branches = jaw claudication
What type of questions to ask when faced w/ potential cases of vasculitis
1) is there a differential differential that could mimic vasculitis?
2) is there a secondary underlying cause
3) what is the extent of vasculitis
4) how do I confirm the diagnosis
5) what is the specific type of vasculitis
Cryoglobulinemic vasculitis
Small vessel vasculitis (polyangiitis) associated w/ Hep C
- serum proteins precipitate out in the cold (cold agglutinins) usually around 36C
While most associated with Hep C, the following can also be causes:
- SLE
- RA
- HIV
- Cardiac/HTN drugs
Wegeners syndrome (granulomatosis w/ polyangiitis)
Presents with
- granulomas in the lungs
- coughing up streaks of blood
- dark urine w/ blood
- creatinine increases in CMP
- splinter hemorrhages
Need cANCA and PR3 tests
ANCA associated vasculitis w/ microscopic polyangiitis (MPA)
Presents with
- high blood in urine
- proteinuria
- high platelets
- high creatinine
- stupid high ESR and CRP levels
- blue extremities (Reynolds looking)
- small palpable rash (possible but not always)
- myalgia and fever
Need pANCA/anti-MPO blood tests to confirm
Chung-Strauss syndrome
Eosinophillic granulomatosis w/ polyangitis (EGPA)
Presents w/
- high eosinophil levels (>10%)*
- x rays appear really spotty and patchy on the lungs
- asthma w/ no past history
- wheezing in both lungs
- muscle wasting and deformity I of specifically the left hand
- fever, malaise
- anorexia
- pain/tingling in the hands
- mononeuritis multiplex (75%) of the time
(Asymmetric peripheral neuropathy appearing idiopathic)
Requires p-ANCA blood tests and high eosinophil levels to confirm
Most common causes of mononeuritis multiplexes
1) leprosy
2) Diabetes
3) Vasculitis
4) sarcoidosis
5) amyloidosis
6) cancer
7) HIV infections
Treatment of vasculitis
Varies based on disease stage, but all stages requires steroids
Early systemic w/ no vital organ damage = methotrexate and steroids
Generalized vasculitis w/ vital organ damage = cyclophosphamide w/ steroids
Severe renal or pulmonary vasculitis =
Cyclophosphamide/ azathioprine and steroids w/ plasma exchange if needed
Refractory vasculitis (vital organ damage w/ no response to therapy) = Rituximab
Buerger Disease (thromboangiitis Obliterans)
Presents w/
- intermittent claudication of cyanotic extremities (worsens when warming up the affected regions)
- cyanotic extremities (worsens when cold)
- necrosis may be present
- ONLY found in heavy smokers
Tx: Stop smoking
- if you dont extremities become necrotic via dry gangrene
Giant cell (temporal) arteritis
Presents with
- severe temporal headaches
- mild fevers
- age >50yrs old (almost never seen in younger patients)
- proximal upper muscle weakness
- blurry vision/ very short intermittent blindness
- intermittent jaw claudication
- tenderness over temporal arteries
- stupid high CRP/ESR levels
- biopsy confirms (treat before this step though)
Note: if girdle weakness w/ flu-like syndrome consider polymyalgia rheumatica (PMR) on top of giant cell arteritis
Tx: high steroid doses (even before biopsy)
Must get this correct or the patient will either go blind permanently or die
Takayasu arteritis and Kawasaki disease
Presents w/:
- fever, malaise and weight loss
- massive HTN
- high proteinuria and hemouria
- elevated ESR and CRP rates
Takayasu will usually present w/ pulseless or super weakened pulses
Kawasaki is usually young patients
IgA vasculitis (Henoch-Schonlein)
Presents only in children and has the following
- very palpable purpura in the extremities
- arthralgia
- GI and kidney arthralgia
- often follows an infections
Possible causes for small cell vasculitis
Microscopic polyangitis (MPA)
Granulomatosis polyangitis (GPA) - also know as Wegners
Cryoglobinemic vasculitis
Churg Strauss vasculitis
Henloch-schonlein purpura
