Review Question - QID 217692

This patient likely has polyarteritis nodosa as evidenced by hypertension, fever, malaise, unintentional weight loss, peripheral neuropathy, elevated ESR and CRP, renal injury (elevated BUN and creatinine), purpuras, and hepatitis B seropositivity. Polyarteritis nodosa is associated with vascular constrictions and aneurysms.

Polyarteritis nodosa is a systemic vasculitis that affects medium-sized arterial vessels. In the disease, vessels become inflamed and thickened, leading to worsening blood flow and thrombosis. Additionally, the inflammation weakens the vessels and can lead to aneurysm development. Patients often have hypertension, systemic symptoms (e.g., fatigue, fever, weight loss, arthralgias, and skin lesions), and organ system-specific symptoms (GI symptoms develop from mesenteric artery involvement). Diagnosis is primarily clinical, but labs are critical to assess the extent of disease progression and organ involvement; a biopsy is confirmatory. A pattern of aneurysms and constrictions, like a string of pearls, is a classic finding on arteriogram. Patients are treated with corticosteroids and cyclophosphamide.

Figure A demonstrates purpuras on the patient’s ankle.

De Virgilio et al. discuss the pathogenesis of polyarteritis nodosa. While immunological mechanisms are likely at play, the actual pathogenesis of idiopathic polyarteritis nodosa is still not elucidated. Biopsies of vessels demonstrate the presence of macrophages and CD8 T cells, suggesting their involvement. A milder form of disease is known as cutaneous polyarteritis nodosa and, as its name suggests, is restricted to the skin.

Incorrect Answers:
Answer 2: Conjunctivitis is seen in Kawasaki disease. It is a medium vessel vasculitis that affects children and is also associated with Asian ethnicities. The presentation includes fever, cervical lymphadenitis, as well as desquamative skin and mucosal changes (peeling skin and “strawberry tongue”). A key complication is myocardial infarction since coronary arteries are commonly affected. Patients are treated with IV immunoglobulin and aspirin. Peripheral neuropathy would not be expected in Kawasaki disease.

Answer 3: Diplopia is seen in giant cell arteritis. It is a large vessel vasculitis primarily seen in women greater than 50 years of age. Giant cell arteritis normally involves cranial arteries and temporal arteries. Patients can also present with temporal headaches, amaurosis fugax, and morning stiffness. Diplopia may develop when the ophthalmic artery is affected. High-dose corticosteroids are needed for treatment. This patient's young age makes giant cell arteritis unlikely.

Answer 4: Intermittent claudication is seen in Buerger disease, also known as thromboangiitis obliterans. It is a medium vessel vasculitis seen in men who heavily use tobacco between the ages of 25 and 50. Other symptoms include painful ulcerations, Raynaud phenomenon, and digit autoamputation. The most important component of management is smoking cessation. This patient is a women who does not smoke making Buerger disease less likely.

Answer 5: Nasal polyps are seen in Churg-Strauss syndrome, also known as eosinophilic granulomatosis with polyangiitis. It is a necrotizing, small vessel vasculitis characterized by multisystem involvement. Patients can present with palpable purpura, upper respiratory signs (e.g., nasal polyps, chronic sinusitis, etc.), as well as peripheral neuropathy; gastrointestinal, cardiac, and renal involvement are also possible. Classical lab findings include peripheral eosinophilia and positive p-ANCA. Treatment involves glucocorticoids and cyclophosphamide. Despite her neuropathy, this patient did not have any other signs of Churg-Strauss syndrome and lacks eosinophilia.

Bullet Summary:
Marked by elevated ESR and CRP as well as a "string of pearls" pattern on an arteriogram, polyarteritis nodosa presents with hypertension, flu-like symptoms, GI symptoms, and peripheral neurologic dysfunction.