Polyarteritis Nodosa Inducing Symmetric Peripheral Gangrene

Abstract

A 47-year-old man with a 1-month history of generalized arthralgias experienced the abrupt onset of fever, myalgias, abdominal pain, and diarrhea. Twenty-four hours later, his fingers and toes appeared cyanotic and then rapidly became necrotic (Figure 1). Aside from an oral temperature of 102°F, his physical examination was otherwise unremarkable.Pertinent laboratory data included 3ϩ proteinuria, a serum creatinine value of 4.3 mg/dL, sterile blood and urine cultures, and a negative hepatitis panel. A host of other serological and hematologic studies gave negative or normal results. The chest radiograph disclosed cardiomegaly. Percutaneous renal biopsy showed necrotic parenchyma. Renal angiography demonstrated bilateral arterial microaneurysms (Figure 2).With a presumptive diagnosis of polyarteritis nodosa, the patient received prednisone and cyclophosphamide. His condition stabilized, and he underwent amputation of the necrotic digits. However, 4 months after onset of his symptoms, he suffered a cerebellar hemorrhage and died. At autopsy, the significant finding was polyarteritis nodosa affecting multiple organs.

A Case of Polyarteritis Nodosa Presenting Initially as Peripheral Vascular Disease

“…9 To date, there have been fewer than ten case reports of PAN presenting as peripheral vascular disease. [10][11][12] There is no reference standard test to diagnose PAN, but the classic histopathology shows segmental transmural inflammation of muscular arteries with an eosinophilic infiltration called fibrinoid necrosis. 13 While the differential diagnosis of PAN is broad, the autoimmune diseases that can resemble PAN include Wegener's granulomatosis, Churg Strauss syndrome, microscopic polyangiitis, giant cell arteritis, and vasculitis secondary to diseases such as lupus.…”

Successful bilateral popliteal-plantar bypasses for polyarteritis nodosa induced ischemia

“…6 There are two reports of patients presenting with symmetric digital gangrene of both upper and lower extremities due to PAN without revascularization attempt. 4,5 The case reported here similarly had quite symmetric disease of his lower extremities. Héron reported a case of a 33-year-old with PAN who developed acute limb ischemia with foot parasthesias and rest pain due to tibial occlusion which was managed with anticoagulation.…”

“…1 PAN characteristically affects people in their 40s to 60s with a male predominance 2 and involves visceral vessels, however there are case reports of peripheral vascular involvement. [3][4][5][6][7][8][9] Criteria for diagnosis of PAN include 3 or more of the following symptoms or signs: weight loss of 4kg or more since illness began; livedo reticularis; tesitular pain or tenderness; myalgias, weakness, or leg tenderness; mononeuropathy or polyneuropathy; diastolic blood pressure >90 mmHg; elevated blood urea nitrogen > 40mg/dL or creatinine >1.5 mg/dL; hepatitis B virus; arteriogram showing aneurysms or occlusions of visceral arteries; and biopsy of small or medium-sized artery containing neutrophils, granulocytes, or mononuclear leukocytes in the artery wall. 10 This case of PAN caused critical limb-threatening ischemia with digital gangrene and contralateral intermittent claudication, and was successfully treated with bilateral revascularization by popliteal-plantar bypass.…”

An Atypical Presentation of Polyarteritis Nodosa: Case Report and Review of the Literature

“…As the patient was of Japanese descent, Kawasaki was in the differential first but the absence of lymphadenopathy, age of presentation, and response to steroids rule it out. There are multiple case reports of PAN among adults, who presented with symmetrical progressive intermittent claudication of lower limbs [7][8][9][10][11][12][13][14][15].…”