STEP2: VASCULIDITIES Flashcards
(50 cards)
What are vasculitides?
A heterogeneous group of rare autoimmune diseases characterized by blood vessel inflammation (vasculitis)
Inflammation can lead to ischemia, necrosis, and/or hemorrhage, resulting in end-organ damage.
What are the two main categories of vasculitides?
Primary (idiopathic) and secondary to an underlying disease or drug use
Examples of secondary causes include HBV infection, cancer, and systemic lupus erythematosus.
How are vasculitides classified based on vessel size?
Small-, medium-, or large-vessel vasculitis, or variable vessel vasculitis.
What symptoms should prompt consideration of vasculitides?
Constitutional symptoms and signs of multisystem disease, such as palpable purpura, pulmonary infiltrates, unexplained ischemic events.
What diagnostic methods are often required to confirm vasculitides?
Laboratory studies, imaging, and histopathology.
What is the management approach for vasculitides?
Involves a multidisciplinary team and aims to prevent progression of vascular inflammation with immunosuppressive therapy.
What is the primary treatment for Giant cell arteritis?
High-dose glucocorticoids to prevent permanent vision loss.
Which demographic is most commonly affected by Takayasu arteritis?
Asian women < 40 years of age.
What are the clinical features of Kawasaki disease?
CRASH (Conjunctivitis, Rash, Adenopathy, Strawberry tongue, Hand-foot changes) and BURN (≥ 5 days of fever).
What is the recommended treatment for Polyarteritis nodosa?
Glucocorticoids PLUS cyclophosphamide.
What is the most common age range for Granulomatosis with polyangiitis?
Adults 40–60 years of age.
What laboratory finding is associated with Eosinophilic granulomatosis with polyangiitis?
Peripheral blood eosinophilia.
What is the primary characteristic of Microscopic polyangiitis?
Pauci-immune glomerulonephritis.
What type of vasculitis is IgA vasculitis most commonly associated with?
Children, with 90% of patients being < 10 years of age.
What is the main clinical feature of Cryoglobulinemic vasculitis?
Fatigue and arthralgia.
What is the common treatment for mild cases of Cutaneous small-vessel vasculitis?
Symptomatic treatment, e.g., with NSAIDs.
What is a key diagnostic feature of Behcet disease?
Oral and genital ulcers.
What are the clinical manifestations of Cogan syndrome?
Inflammatory ocular lesions and inner ear disease.
What is the classification system for vasculitides based on vessel size according to the 2012 Chapel Hill Consensus Nomenclature?
Large-vessel vasculitis, medium-vessel vasculitis, small-vessel vasculitis, ANCA-associated vasculitis, non-ANCA-associated vasculitis, variable vessel vasculitis.
What are the large vessel vasculitidies?
Takayasu and giant cell
Giant cell: age over 50, associated with polymyalgia rheumatica
Takayasu: women under 40
What are the typical demographics and the primary vessels affected in Giant Cell Arteritis (GCA)?
Demographics: Patients over 50 years old, typically women, often of Northern European descent.
Primary Vessels: Cranial arteries (temporal, ophthalmic), aorta and its major branches.
List the classic and highly suspicious symptoms of Giant Cell Arteritis (GCA).
New-onset headache (often temporal, severe, with scalp tenderness)
Jaw claudication
Visual disturbances (amaurosis fugax, sudden permanent vision loss)
Associated with Polymyalgia Rheumatica (PMR) (proximal muscle pain/stiffness, especially morning)
Constitutional symptoms (fever, fatigue, weight loss)
How is Giant Cell Arteritis (GCA) diagnosed, and what is the crucial acute management?
Diagnosis:
Highly elevated ESR and CRP (almost always)
Gold standard: Temporal artery biopsy (granulomatous inflammation with giant cells)
Acute Management: Immediate high-dose corticosteroids (e.g., prednisone) to prevent irreversible blindness, even before biopsy confirmation.
