Question

Difficulty: MediumRheumatology and Immunology

Match each of the following patient clinical vignettes to the most likely specific vasculitis diagnosis.

  • A 35-year-old man with chronic sinusitis, hemoptysis, and acute kidney injury; testing reveals c-ANCA (PR3-ANCA) positivity.Granulomatosis with polyangiitis
  • A 40-year-old man with a history of severe adult-onset asthma and allergic rhinitis presents with a new-onset foot drop; p-ANCA (MPO-ANCA) is positive.Eosinophilic granulomatosis with polyangiitis
  • A 45-year-old man with postprandial abdominal pain, livedo reticularis, and a recent hepatitis B infection; ANCA testing is negative.Polyarteritis nodosa
  • A 25-year-old woman presents with upper extremity claudication, absent radial pulses, and systemic constitutional symptoms; ESR is markedly elevated.Takayasu arteritis
  • A 7-year-old boy presents with abdominal pain, diffuse arthralgia, and a palpable purpuric rash on his buttocks and lower extremities following an upper respiratory tract infection.IgA vasculitis

Answer

The presentations match as follows: the c-ANCA positive patient with respiratory/renal disease matches Granulomatosis with polyangiitis; the p-ANCA positive asthmatic with foot drop matches Eosinophilic granulomatosis with polyangiitis; the Hepatitis B positive patient with abdominal angina matches Polyarteritis nodosa; the young pulseless woman matches Takayasu arteritis; and the child with purpura matches IgA vasculitis.
The diagnosis of systemic vasculitis relies heavily on recognizing distinct clinical syndromes. Granulomatosis with polyangiitis classically presents with upper respiratory tract disease, lower respiratory tract disease, and glomerulonephritis, alongside c-ANCA (PR3) positivity. Eosinophilic granulomatosis with polyangiitis is distinguished by asthma, allergic rhinitis, and eosinophilia, typically with p-ANCA (MPO) positivity. Polyarteritis nodosa is a medium-vessel vasculitis that typically spares the lungs, frequently involves the mesenteric and renal arteries, and has a known strong association with hepatitis B infection. Takayasu arteritis involves the aorta and its major branches, frequently causing arm claudication and absent pulses in young women. IgA vasculitis is the most common childhood vasculitis, recognized by the distinct tetrad of palpable purpura on gravity-dependent areas, arthralgia, abdominal pain, and renal involvement.

Step-by-Step Solution

1
Analyze each patient's demographic profile, organ system involvement, and serological findings.
Identified unique patterns such as c-ANCA with lung/renal involvement, p-ANCA with asthma/neuropathy, Hepatitis B association, pulseless disease in a young woman, and a pediatric lower-extremity purpuric rash.
Systemic vasculitides are categorized by vessel size and present with distinct, recognizable syndromic clusters.
2
Match the identified syndromic clusters to the established diagnostic criteria for systemic vasculitides.
The vignettes correctly pair with their respective small, medium, and large vessel vasculitic syndromes.
Accurate diagnosis based on clinical and laboratory patterns dictates the specific therapeutic intervention and prognostic counseling.

Key Concept

Clinical recognition and differentiation of systemic vasculitides based on vessel size, specific organ involvement, demographic factors, and serological markers.
Rate this question