Discussion On Following Headings
Vasculitis should be suspected in patients presenting with any combination of
what clinical manifestations?
Name the primary vasculitic disorders based on the dominant vessel size and
antineutrophil cytoplasmic antibodies (ANCA).
What serologic tests or diagnostic procedures should be performed in patients
with suspected vasculitis?
What more extensive procedures may be of value in helping to establish the
diagnosis of a specific form of vasculitis?
Vasculitis should be suspected in patients presenting with any combination of
what clinical manifestations?
Vasculitis comprises a heterogeneous group of diseases characterized by
inflammatory changes in the blood vessels with subsequent impairment of flow
and tissue/organ ischemia. Patients present with a multisystem inflammatory
disease often with fever of unknown origin and/or unexplained constitutional
symptoms; suspicious skin lesions such as ulcers, livedo reticularis, and
palpable purpura; ischemic neuropathies; and rapidly progressive organ
dysfunction such as strokes, pulmonary–renal syndromes, and other organ
ischemia.
Name the primary vasculitic disorders based on the dominant vessel size and
ANCA.
Vasculitides affecting large arteries:
Takayasu's arteritis: aortic arch and its branches, can involve any part of
the aorta; more claudication of upper than lower extremities, central
nervous system events; granulomatous panarteritis.
Giant cell (temporal) arteritis (GCA): temporal arteries, vessels
originating from the aortic arch, other arteries less common; temporal
headache, jaw claudication, scalp tenderness, visual loss; arteritis with
giant cells and disruption of the internal elastic lamina.
Vasculitides affecting predominantly medium-sized arteries:
Polyarteritis nodosa (PAN): small- and medium-sized arteries; may
affect any organ, but skin, joints, peripheral nerves, gut, and kidney are
most commonly involved; focal but panmural necrotizing arteritis with a
predilection for involvement at the vessel bifurcation.
Kawasaki disease: small- and medium-sized arteries; acute febrile
illness primarily affecting infants and young children; fever, prominent
mucocutaneous changes, cervical lymphadenopathy, polymorphous
rash, erythema and edema of hands and feet, desquamation,
myocarditis, coronary vasculitis; probable infectious vector resulting in
cytokine-mediated endothelial damage.
Vasculitides affecting predominantly small vessels (ANCA-positive):
Wegener's granulomatosis: small- and medium-sized arteries; upper
respiratory tract (sinuses), lungs, and kidneys, may affect other organs;
pauciimmune, necrotizing, granulomatous arteritis usually associated
with serum cytoplasmic–antineutrophil cytoplasmic antibodies (c-ANCA)
usually directed against proteinase 3 in the primary granules of neutrophils.
Microscopic polyangiitis (MPA): arterioles, capillaries, and venules;
pulmonary hemorrhage, glomerulonephritis, palpable purpura,
peripheral neuropathy, joint and abdominal pain; pauciimmune,
necrotizing vasculitis, serum perinuclear–antineutrophil cytoplasmic
antibodies usually directed against myeloperoxidase in the primary
granules of neutrophils.
Churg-Strauss syndrome: small arteries and venules; asthma,
eosinophilia, multiorgan involvement [lungs, skin, peripheral nerves,
gut, heart, and kidneys (rare)]; necrotizing extravascular granulomas
and vasculitis of small arteries and venules, eosinophils present in early
stage.
Vasculitides affecting predominantly small vessels (ANCA-negative):
Henoch-Schönlein purpura (HSP): arterioles and venules; palpable
purpuric skin lesions on lower extremities, arthritis, abdominal pain,
hematuria; leukocytoclastic (neutrophilic perivascular/transmural
infiltrate) or necrotizing vasculitis often with IgA deposition.
Cutaneous leukocytoclastic angiitis: arterioles and venules; palpable
purpuric skin lesions, arthralgias, systemic symptoms may be present,
usually secondary to immune complexes [drugs, bugs (infections), CTD
or malignancy]; leukocytoclastic vasculitis.
Cryoglobulinemic vasculitis: cryoglobulins are immunoglobulins that are
reversibly precipitated by reduced temperatures; cryoglobulins are
deposited in small vessels including glomerulocapillaries; purpura,
arthralgias, peripheral neuropathy, Raynaud's phenomenon, pulmonary
hemorrhage, glomerulonephritis are possible; often RF and hepatitis C
antibody positive
What serologic tests or diagnostic procedures should be performed in patients
with suspected vasculitis?
The diagnostic evaluation of a patient with suspected vasculitis should be
based on the clinical situation but often includes a chest radiographic study,
ESR, CRP, a complete blood count with differential, liver function tests, CPK,
creatinine and urinalysis, tests for the presence of ANAs, ANCAs and RF,
cryoglobulins, and biopsy of a skin lesion or an involved organ. In some types
of vasculitis, complement levels may be low secondary to consumption. An
ESR greater than 100 mm per hour and a CRP greater than 10 mg/dL in the
absence of a widespread malignancy or bacterial infection should suggest a
vasculitic process.
What more extensive procedures may be of value in helping to establish the
diagnosis of a specific form of vasculitis?
More extensive diagnostic procedures for establishing the diagnosis of a
specific form of vasculitis include arteriography of the mesenteric vessels if a
tissue biopsy is inaccessible, and an electromyography with evaluation of
nerve conduction velocities to evaluate a peripheral neuropathy or a
mononeuritis multiplex. A computed tomography (CT) scan of the sinuses and
chest is indicated if a diagnosis of Wegener's granulomatosis is being
considered.
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