VASCULITIS Flashcards
What is the vasculitis most common to Turkey
Behcets
What is the vasculitits most common to japan
Kawasakis syndrome
What vasculitis is most common with Hep C
Cryoglobulinemia
What vasculitis is most common with Hep B
Polyarteritis Nodosa
What separates Giant Cell and Takayasu
If older than 50 = giant cell
If younger than 50= takayasu
50 % of Giant cell pts present with what other vasculitis
Polymyalgia Rheumatica : Aching and stiffness of the shoulders, neck and hip-girdle area
When should Bx be done for Giant Cell arteritis
Needs to be done within 2 weeks of steroids before biopsy results will be altered
Consider second biopsy if clinical suspicion is high after initial negative biopsy
If a pt with Giant cell arteritis develops transient vision loss
What should be done
Patients with suspected GCA who have experienced transient visual loss for a few hours should be admitted and given high-dose intravenous methylprednisolone (eg, 1000 mg/day) for 3–5 days.
Then transitioned to oral prednisone 40-60mg daily.
If a pt has giant cell without vision loss
What is the Tx?
Prednisone 1mg/kg max;60mg/ day
X 1 year
When can steroid taper start in pts with giant cell
After the first month of treatment, almost all patients will have a normal ESR
- At this point, the prednisone can begin to be tapered by 10% every week or two
- When patients reach 10 mg, decrements of 1 mg every 2 or so weeks may reduce the chance of flare
Consideration of addition of low dose aspirin 81mg may reduce chance of associated visual loss or stroke
How old is the Dx for Polymyalgia Rheumatica
50 and older
A 50 year old pt report morning MUSCLE stiffness and aching that last longer than 30 minutes (not in the joint)
Think
Polymyalgia Rheumatica
Stiffness involves at least two of the following three areas:
- Neck or torso
- Shoulders or proximal regions of the arms
- Hips or proximal aspects of the thighs
What is the treatment appraoch to polymaylagia Rheumatica
PMR alone is usually treated with 10–20 mg/day of prednisone.
-If the patient does not improve within the first week, doubt should be cast on the diagnosis of PMR.
Prolonged corticosteroid taper. Most patients require treatment for 1-2 years.
What are the important late complications of giant cell arteritis
Thoracic aortic aneurysms and aortic dissection are important late complications
Define Takayasu’s Arteritis
Granulomatous Vasculitis of the Aorta and its major branches
Preferentially affects young women
Distinguishing characteristics related to aorta/branch involvement
A 20 year old woman presents with absent pulse or bruit, Claudication, or HTN +/- fever of unknown origin
Think what vasculitis
Takayasu’s
A 20 year old woman that complains of Claudication while drying her hair
.. think
Takayasus
Look for abNML pulses or unequal BP
Bruits, or HTN
Murmurs: AR
Or Angina from stenosis of the ostia of the coronary arteries
What is the tx approach to Takayasu’s
High dose prednisone 1mg/kg/day x 1 month -> Taper to 10mg/day over 4-6 months
-Very effective but relapse is common
Addition of Methotrexate or Mycophenolate Mofetil to prednisone may provide added benefit
May require Surgical intervention for heart conditions
What is the vasculitis associated with HLA B51
Behcets
What types of vessels does Behcets affect?
Inflammatory vasculitis that affects large, medium, and small blood vessels.
Affects both arteries and veins
Arterial inflammation leads to occlusion, aneurysm, or rupture
What is the hallmark sign of Behcets
painful aphthous ulcers in the mouth and genitalia
- Tongue, gums, oral cavity
- Genital lesions –similar, but don’t occur in all pts
What is the major difference in the lesions of Behcets and sarcoidosis or IBD ?
Erythema nodosum–like lesions that tend to ulcerate
What are the 4 major S/s of Behcets
Painful apthous ulcers
Erythema Nodusom like lesions that then ulcerate
Anterior or posterior Uvetitis
And neurologic lesions than mimic MS
Define Polyartetits Nodosa
Necrotizing inflammatory vasculitis of medium arteries and muscular arterioles
Spares venous circulation and capillaries (confined to arterial circulation)
What is required for Dx of Polyartertis Nodosa
Angiogram or biopsy of involved organ required for diagnosis
- Lung is usually spared
- Granulomatous inflammation absent
What is the onset of PAN following HEP B infection
Within 6 months
A pt presents with severe abdominal pain + peripheral neuropathy and mononerutitis multiplex (wrist/foot drop), Palpable purpura, lívido reticularis, and skin ulcers
Think of what condition that is assoc with HEP B infection
PAN
What separates polyangitis nodosa from microscopic polyangitis
PAN does not involve the lungs/ Pulm
What are the key Lab tests for PAN
HBV testing at time of initial diagnosis
True PAN is ANCA-negative
And Bx: Characteristic transmural necrosis and a homogeneous, eosinophilic appearance of the blood vessel wall (fibrinoid necrosis)
What is the Tx approach to PAN
Prolonged high dosed corticosteroids
Cyclophosphamide (Cytoxan)
-For patients whose disease is refractory to glucocorticoids
or
-who have serious involvement of major organs (GI; mononeuritis multiplex)
What are the 4 keys to Dx buergers
recognition of clinical findings-digital ischemia without other organ involvement
identification of typical pattern in angiography
exclusion of diseases that may mimic Buerger
confirmation that ongoing tobacco exposure is present
What is the age of onset for Kawasakis
Most commonly in kids 3 months to 5 years old
-Typically Asians or native Pacific Islanders
A 4 year old pt presents with a fever for 5 days!, with bilateral non exudative conjunctivitis, strawberry tongue/ cracked lips, with erythema to the hands and feet, + a polymorphous rash
Think
Kawasaki’s
What is the treatment for a child with kawasakis
IVIG within the first 10 days of illness!
-Dramatically reduce risk of cardiac morbidity (coronary aneurysms)
+Aspirin (low-dose as effective as high-dose)
If unresponsive to IVIG, can use cyclosporine
Glucocorticoids reserved for salvage therapy in which IVIG and ASA failed
What is the classic finding for small vessel vasculitis
Palpable purpura
What is the most common vasculitis in children
HENOCH-SCHONLEIN PURPURA
What are the hallmarks of HENOCH-SCHONLEIN PURPURA
Pathologic hallmarks of HSP are a leukocytoclastic (neutrophil debris) vasculitis and deposition of IgA in the walls of involved blood vessels.
Onset typically ~10 days after a URI
What is the tetrad of henoch schonlein purpura
The tetrad of purpura, arthritis, glomerulonephritis, and abdominal pain is often observed.
However, this is not required for Dx
A child (less than 20 yo) presents with palpable purpura, bowel angina, and vessel wall granuloctyes on BX
Think
Henoch Scholein
HENOCH-SCHONLEIN PURPURA Tx
NSAIDs
-can aggravate GI symptoms and should be avoided with renal disease
Dapsone
-Antibiotic & anti-inflammatory
Glucocorticoids
Hospitalization
-Severe cases (Renal Failure)
What are cryoglobulins
Immunoglobulins (antibodies IgG, IgM) that precipitate out of blood at low temperatures (< 37oC)
What is the most common involved organ for Cryoglobulinemia
The skin
Other frequently affected organs include the joints, peripheral nerves, and kidneys.
What is the most common serum marker found in Cryoglobulinemia
RF postive
What is the pt demographic that has the worst prognosis with Cryoglobulinemia
Lower survival rates are observed with age over 60 years, male sex, and renal involvement.
What are the diseases that are assoc with Cryoglobulinemia
Associated with malignancy (multiple myeloma), Hepatitis C, Sjogren and systemic lupus erythematosus
What are the labs for Cryoglobulinemia
RF present in Types II & III
ESR/CRP elevated
Complement is decreased (just like in Sjogren)
What is the tx for Cryoglobulinemia
Usually focused on the underlying cause (usually hepatitis C)
- Ledipasvir/sofosbuvir (Harvoni)
- Ribavarin (Copegus)
Antiviral strategies are commonly combined with B cell depletion approaches
-Rituximab (Rituxan)
Some cases can evolve to malignant B cell lymphoma
A pt that presents iwth necrotizing vasculitis + upper/ lower resp and renal manifestations
Think
GRANULOMATOSIS WITH POLYANGIITIS
What is the hallmark of GRANULOMATOSIS WITH POLYANGIITIS
Hallmarks: Granulomatous inflammation, vasculitis, and necrosis
Age onset; 50
What are the key findings in Granuloatousus with polyangitis
ANCA positive
+ saddle nose (sinus: rhino, epistaxis)
Normochromic normocytic anemia
Must get cANCA and BX ( Lung>kidneys>URI)
(CT>CXR)
What is the most common CXR finding in GPA
Pulm infiltrates and nodules
What is the treatment for GPA (severe and Limited)
Severe: life threatening: Rituxiab and steroids
Or Cyclophosphamide and steroids
Limited: Methotrexate and steroids
All should receive Pneumocystis jiroveci pneumonia prophylaxis
-Sulfamethoxazole-trimethoprim, Dapsone
What are the hallmarks of eosinophila granulomatous polyangitis
Asthma, eosinophilia, and systemic vasculitis are the hallmarks of eosinophilic granulomatosis with polyangitis
What are the classic necrotizing features of EGPA
Allergic rhinitis and nasal polyposis Reactive airway disease Peripheral eosinophilia Fleeting pulmonary infiltrates and occasional alveolar hemorrhage Vasculitic neuropathy Congestive heart failure
What is the serum marker for EGPA
Approximately 50% of patients with EGPA have antineutrophil cytoplasmic antibodies (ANCAs), usually with a specificity for myeloperoxidase (MPO).
MPO-ANCAs usually produce a perinuclear-ANCA (P-ANCA) pattern on serum immunofluorescence testing
What are the three phases of EGPA
Prodromal phase: characterized by allergic disease, (allergic rhinitis, or asthma)
Eosinophilic phase: peripheral blood eosinophilia and eosinophilic infiltration of multiple organs, heart, lung, GI tract and others.
Vasculitic phase: a life-threatening systemic necrotizing vasculitis of small & medium vessels – wide range of organs including heart, lungs, peripheral nerves and skin
What is the key differnce in GPA vs EGPA
ASTHA in EGPA
What is the most common cardiac manifestation of EGPA
CHF
What is the treatment for EGPA
Steroids to induce remission
Azathioprine, methotrexate, or mycophenolate can be added to corticosteroids for moderate cases
What is the most common cause of pulmonary renal syndomre of alveolar hemorrhage and Glomerulonephritis
Microscopic polyangitis
What are the main differences in microscopic polyangitis and polyarteritis nodosa
Main differences: ANCA positivity, vein AND artery involvement, glomerulonephritis & lung involvement
If a pt is on PTU for hyperthyroidism
What is the vasculitis d/o that is an increased risk
Microscopic polyangitis
What is the treatment of choice in microscopic polyangitis
Traditionally combo therapy with glucocorticoids and cytotoxic agents (cyclophosphamide)
Rituximab now preferred over cyclophosphamide due to superior long-term side effect profile.
If rituximab cannot be given or if the patient does not respond to regimen, cyclophosphamide is treatment of choice.
