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Difficulty: MediumRheumatology and Immunology

Match each clinical presentation of systemic vasculitis with its most likely corresponding diagnosis.

  • Adult-onset asthma, chronic rhinosinusitis, peripheral neuropathy (mononeuritis multiplex), and laboratory findings showing marked eosinophilia.Eosinophilic granulomatosis with polyangiitis
  • Chronic otitis media, refractory sinusitis, hemoptysis from cavitary lung lesions, and a urinalysis demonstrating red blood cell casts.Granulomatosis with polyangiitis
  • Recurrent postprandial abdominal pain, testicular pain, new-onset foot drop, and angiographic demonstration of renal microaneurysms without lung involvement.Polyarteritis nodosa
  • Recurrent episodes of palpable purpura on the lower extremities, severe arthralgias, and laboratory studies revealing profoundly low C4 levels.Cryoglobulinemic vasculitis

Answer

The correct matches rely on identifying the unique organ involvement and laboratory hallmarks of each vasculitis: asthma and eosinophilia match with Eosinophilic granulomatosis with polyangiitis; cavitary lung lesions and glomerulonephritis match with Granulomatosis with polyangiitis; medium-vessel involvement sparing the lungs matches with Polyarteritis nodosa; and palpable purpura with low C4 matches with Cryoglobulinemic vasculitis.
Each systemic vasculitis exhibits a distinct pattern: EGPA features atopy and eosinophilia; GPA features necrotizing upper/lower respiratory granulomas and glomerulonephritis; PAN is a medium-vessel disease sparing the lungs; and Cryoglobulinemic vasculitis is a small-vessel immune complex disease marked by low C4 and purpura.

Step-by-Step Solution

1
Analyze the size of the vessels involved and the specific organs affected in each clinical vignette.
Identified small-vessel patterns (pulmonary-renal syndromes, purpura) and a medium-vessel pattern (microaneurysms, sparing of lungs).
Vasculitides are broadly classified by the size of the predominant blood vessels they affect, which determines their clinical presentation.
2
Differentiate between the ANCA-associated small vessel vasculitides (AAVs).
The presence of adult-onset asthma and eosinophilia points exclusively to Eosinophilic granulomatosis with polyangiitis, while upper/lower destructive respiratory lesions combined with renal disease point to Granulomatosis with polyangiitis.
Although GPA, EGPA, and Microscopic polyangiitis all affect small vessels and kidneys, their pulmonary and systemic features are highly distinct.
3
Identify the remaining conditions by their classic risk factors and laboratory markers.
Microaneurysms and visceral ischemia indicate Polyarteritis nodosa, while palpable purpura and low C4 indicate Cryoglobulinemic vasculitis.
Complement consumption (low C4) is a hallmark of cryoglobulinemia, whereas angiographic evidence of aneurysms is the gold standard for diagnosing PAN.

Key Concept

Clinical differentiation of systemic vasculitides based on classic symptom triads, organ involvement, and serologic markers.
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Topics

Rheumatology and Immunology

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