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Difficulty: MediumRheumatology and Immunology

Match each clinical patient presentation to the most likely vasculitic syndrome.

  • An adult male with a history of late-onset asthma develops sudden right foot drop and demonstrates marked eosinophilia on routine blood testing.Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)
  • Evaluation of a female patient reveals saddle-nose deformity, a history of hemoptysis, and acute kidney injury with red blood cell casts.Granulomatosis with polyangiitis (Wegener)
  • A male patient with chronic hepatitis B infection experiences severe postprandial abdominal pain, testicular pain, and newly elevated blood pressure.Polyarteritis nodosa
  • A school-aged boy is brought in for lower extremity joint swelling, crampy abdominal pain, and a raised purpuric rash on his buttocks following a minor respiratory illness.IgA vasculitis (Henoch-Schönlein purpura)

Answer

The presentations map to: 1) Asthma/eosinophilia matches with Eosinophilic granulomatosis with polyangiitis; 2) Saddle-nose/hemoptysis/nephritis matches with Granulomatosis with polyangiitis; 3) Hepatitis B/abdominal pain matches with Polyarteritis nodosa; 4) Pediatric purpura/arthritis matches with IgA vasculitis.
Accurate diagnosis of systemic vasculitides requires recognizing unique symptom combinations. EGPA features asthma and eosinophilia; GPA features necrotizing granulomas of the upper/lower respiratory tract and kidneys; PAN features medium-vessel ischemia (mesenteric, renal) often linked to Hepatitis B and sparing the lungs; and IgA vasculitis presents with a classic tetrad in pediatric populations.

Step-by-Step Solution

1
Analyze the first presentation emphasizing respiratory history, peripheral nerve deficit, and abnormal cell counts.
Identified asthma, foot drop, and eosinophilia.
These findings are virtually pathognomonic for Eosinophilic granulomatosis with polyangiitis.
2
Examine the second presentation focusing on structural airway changes, pulmonary bleeding, and renal findings.
Identified upper airway (saddle-nose), lower airway (hemoptysis), and kidney (RBC casts) triad.
This specific multi-organ triad strongly points to Granulomatosis with polyangiitis.
3
Evaluate the third presentation for underlying viral infections and specific organ involvement patterns.
Identified Hepatitis B infection, mesenteric ischemia, and new hypertension, notably without pulmonary symptoms.
The association with Hepatitis B and lack of lung involvement confirms Polyarteritis nodosa.
4
Assess the pediatric presentation for characteristic rash location and associated systemic symptoms.
Identified lower extremity/buttock purpura, abdominal pain, and joint swelling after a viral illness.
This is the classic presentation of IgA vasculitis, the most common systemic vasculitis in childhood.

Key Concept

Clinical differentiation of systemic vasculitides based on classic symptom triads, organ involvement patterns, and demographic associations.
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Topics

Rheumatology and Immunology

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