139 - Systemic Necrotizing Arteritis Flashcards
Predominantly small vessel vasculitis
Immune complex mediated:
- Cryoglobulinemia
- HSP
Limited skin
ANCA-associated:
- MPA
- CSS
- GPA
Predominantly medium vessel
Kawasaki
PAN
Predominantly large vessel
GCA
TAK
Isolated aortitis
No predominant size
Behcet
Primary CNS
Relapsing polychondritis
Cogan
Takayasu arteritis is substantially more common in
Women
Kawasaki disease is almost exclusively a disease of
Young children
Giant cell arteritis is limited to
Older adults
Granulomatosis with polyangitis (Wegener) mostly occurs in
Whites
Behcet disease is markedly more common in countries in the
Eastern Mediterranean
Japan
Korea
Infection-related vasculitis: _____ in many cases of polyarteritis nodosa
Hepatitis B virus
Infection-related vasculitis: _____ in most cases of cryoglobulinemic vasculitis
Hepatitis C virus
Small-vessel vasculitis follow one of 2 pathways:
- Immune complex deposition
2. Non-immune complex mediated pathology that likely involves ANCA
ANCA are directed against the neutrophil proteins
Myeloperoxidase (MPO)
Proteinase-3 (PR3)
Large-vessel vasculitis are thought to be medicated by
T cells activating macrophages
HLA locus in GCA
Class II
HLA locus in Takayasu arteritis
Class I
Single most important component of early evaluation of a patient suspected of having vasculitis
Full review of systems with assessment of overall severity of illness
The diseases that cause cutaneous vasculitis can affect all organ systems, and in most cases that involvement will cause symptoms, _____ being a prominent exception
Renal disease
For drug-induced vasculitis, it is useful to ask about prescription, nonprescription, and “alternative” or herbal medication use in the prior
6 to 12 months
It is generally recommended to biopsy a skin lesion that has been clinically apparent for
Less than 48 hours
Histopathology: extravascular granulomas with geographic necrosis
Granulomatosis with polyangitis
Histopathology: eosinophil-rich extravascular granulomas
Eosinophilic granulomatosis with polyangitis
Y/N: Kidney or lung biopsies are less likely than skin biopsies to show pathology diagnostic of a particular disease
No - more likely
Tests for _____ are the most important to order in evaluating a patient suspected of having vasculitis
Renal disease
Common in many vasculitides and is rarely accompanied by signs or symptoms until end-stage renal failure occurs
An elevated absolute eosinophil is found in most untreated patients with eosinophilic granulomatosis with polyangitis (Churg-Strauss), and a count greater than _____ helps differentiated this disease from asthma and atopy
1000 cells/microL
Y/N: The levels of ESR and CRP do not correlate well with stage or severity of disease
Yes
Autoimmune serologies: ANCA-associated vasculitis and lupus can present with vasculitis of the skin, but _____ does not
Anti-glomerular basement membrane (GBM) disease
90% of patients with _____ will test positive for ANCA
Microscopic polyangiitis
75% of patients with _____ will test positive for ANCA
Granulomatosis with polyangiitis
40% of patients with _____ will test positive for ANCA
Eosinophilic granulomatosis with polyangiitis
Pauci-immune glomerulonephritis, pulmonary hemorrhage, peripheral and cranial neuropathy, musculoskeletal, constitutional symptoms
Most patients are positive for ANCA, usually with specificity for antibodies to myeloperoxidase
Microscopic polyangiitis
Encompasses all features of microscopic polyangiitis but also many additional manifestations caused by necrotizing granulomatous inflammation
Chronic inflammation of the upper airway ( nasal cavity, sinuses, auditory tube, middle ear), cavitary pulmonary nodules, orbital pseudotumor, subglottic stenosis, vasculitis of the eye
Neutrophilic and granulomatous dermatitis with papules (particularly on the extensor surfaces of the elbows), subctaneous nodules and ulcers
Majority ANCA type is c-ANCA/anti-PR3
Granulomatosis with polyangiitis
Unique features: history of asthma (often severe or poorly controlled), blood eosinophilia
Nasal polyps, constitutional symptoms, rashes, all typical of atopy
Pulmonary infiltrates on chest imaging (eosinophilic pneumonia)
Eosinophilic granulomatosis with polyangiitis
Most common presentation of severe vasculitis in eosinophilic granulomatosis with polyangiitis
Acute peripheral neuropathy with involvement of the heart, GI tract, brain, eyes
Cutaneous disease is more common in (MPA, GPA, EGPA)
Eosinophilic granulomatosis with polyangiitis
Histologic term defined by the absence of evidence of systemic disease
Cutaneous leukocytoclastic angiitis
Cutaneous leukocytoclastic angiitis: _____% of cases follow a wide range of infections
20
Cutaneous leukocytoclastic angiitis: _____% of cases are associated with drug exposure
20
Skin disease, myalgia, hypertension (from renal artery involvement), abdominal pain, neuropathy and/or testicular pain
“Classic” polyarteritis nodosa
Most common cutaneous features of polyarteritis nodosa
Livedo reticularis/racemosa
Disease of adults older than 50 years
Mostly a disease of people of Northern European ancestry
Cranial arteritis is a common feature, polymyalgia rheumatica, fever, malaise, weight loss
Giant cell arteritis
Diagnosis of GCA is usually confirmed by
Temporal artery biopsy
Only cutaneous manifestation of GCA apparent on examination
Present in 30% to 40% of cases
Palpable nodularity of the temporal artery
Rare form of vasculitis involving the aorta and its major branches
Many patients are diagnosed as young adults, 90% are female
Takayasu arteritis
Typical presentation of Takayasu arteritis
Limb claudication
Diagnosis of Takayasu arteritis is made by
Angiography
Mortality in the first 3 to 6 months of severe vasculitis affecting vital organs is around
10%
Other than advanced age, the predictors of higher risk of mortality reflect prior organ damage: _____ damage in the case of granulomatosis with polyangiits and microscopic polyangiitis, and _____ damage in the case of eosinophilic granulomatosis with polyangiitis
Kidney
Cardiac
For vasculitides expected to have extended courses and/or to include severe manifestations, the general approach is to plan for 2 phases of treatment:
Remission induction
Remission maintenance
Mainstay of therapy for vasculitis
Glucocorticoids
Proven effective for ANCA-associated vasculitides and, to a lesser extent, other forms of vasculitis
Cyclophosphamide
Serious toxicities of cyclophosphamide which are related to total cumulative dose
Female and male infertility
Bladder cancer
Apremilast has now been demonstrated to have efficacy in the treatment of
Mucocutaneous manifestations of Behcet disease
Biologic demonstrated to be as effective as cyclophosphamide for induction of remission of AAV
Rituximab
Monoclonal antibody to anti-interleukin 5
Demonstrated to have efficacy in the treatment of eosinophilic granulomatosis with polyangiitis
Mepolizumab
Monoclonal antibody to interleukin-6
Demonstrated efficacy for the treatment of giant cell arteritis
Tocilizumab
CTLA-4 immunoglobulin
Promising data on its use for giant cell arteritis
Abatacept
