Question

Difficulty: HardRheumatology and Immunology

An emergency department evaluation is requested for a 4242-year-old man who reports severe, diffuse abdominal pain that consistently begins 30 minutes30\text{ minutes} after meals over the past 4 weeks4\text{ weeks}. He also reports a 6-kg6\text{-kg} (13-lb13\text{-lb}) weight loss during this period, diffuse muscle aches, and intermittent fevers. Two days ago, the patient developed difficulty lifting his right foot while walking. His temperature is 38.1∘C38.1^\circ\text{C} (100.6∘F100.6^\circ\text{F}) and blood pressure is 168/98 mmHg168/98\text{ mmHg}. Physical examination shows a mottled, purplish, net-like rash on the lower extremities. Neurologic examination demonstrates 2/52/5 motor strength in right foot dorsiflexion and decreased pinprick sensation over the right lateral calf.

Laboratory studies are as follows:

Laboratory TestResult
Hemoglobin10.8 g/dL10.8\text{ g/dL}
Leukocyte count12,500/mm312,500/\text{mm}^3
Platelet count410,000/mm3410,000/\text{mm}^3
Blood urea nitrogen32 mg/dL32\text{ mg/dL}
Serum creatinine1.9 mg/dL1.9\text{ mg/dL}
Erythrocyte sedimentation rate78 mm/h78\text{ mm/h}
Hepatitis B surface antigenPositive

Urinalysis reveals 1+1+ protein, 0−20-2 red blood cells/hpf, and no cellular casts.

Which of the following is the most appropriate next step in diagnosis?

  1. Mesenteric and renal angiographyAnswer
  2. B
    Percutaneous renal biopsy
  3. C
    Testing for antineutrophil cytoplasmic antibodies
  4. D
    Electromyography and nerve conduction studies
  5. E
    Colonoscopy with mucosal biopsy

Answer

The correct answer is mesenteric and renal angiography, which is the preferred diagnostic modality for polyarteritis nodosa (PAN) presenting with intra-abdominal and renal involvement.
The correct next step is mesenteric and renal angiography. This patient's clinical presentation—postprandial abdominal pain (intestinal angina), mononeuritis multiplex (foot drop), livedo reticularis, new-onset hypertension, and acute kidney injury—in the setting of chronic Hepatitis B infection is highly characteristic of polyarteritis nodosa (PAN). PAN is a systemic necrotizing vasculitis that primarily targets medium-sized muscular arteries. When intra-abdominal or renal involvement is prominent, mesenteric and renal angiography is the preferred diagnostic test. It classically reveals multiple microaneurysms and segmental narrowing (often described as a 'string of pearls' appearance) of the medium-sized arteries. Angiography provides a high diagnostic yield while entirely avoiding the significant bleeding risks associated with deep tissue biopsies.

Step-by-Step Solution

1
Identify the defining clinical features and vital sign abnormalities.
The patient presents with intestinal angina, mononeuritis multiplex (foot drop), livedo reticularis, fever, weight loss, and new-onset hypertension.
Recognizing this classic constellation of symptoms is essential for formulating a differential diagnosis of systemic vasculitis.
2
Interpret the laboratory findings in the context of the clinical picture.
The patient has elevated inflammatory markers, acute kidney injury without glomerulonephritis (no RBC casts), and a positive Hepatitis B surface antigen.
The absence of an active urinary sediment points away from small-vessel vasculitis, while Hepatitis B positivity strongly suggests polyarteritis nodosa (PAN), a medium-vessel vasculitis.
3
Determine the most appropriate diagnostic test based on the suspected disease mechanism.
Mesenteric and renal angiography is selected over biopsy.
In PAN, medium-sized muscular arteries develop microaneurysms that are clearly visible on angiography. A renal biopsy carries a high risk of life-threatening hemorrhage from these aneurysms and has low yield because it primarily samples the unaffected glomeruli.

Key Concept

Polyarteritis nodosa (PAN) is a medium-vessel necrotizing vasculitis strongly associated with Hepatitis B, frequently causing renal insufficiency without glomerulonephritis and mesenteric ischemia. It is best diagnosed with mesenteric and renal angiography to identify characteristic microaneurysms.
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