Polyarteritis nodosa associated with essential mixed cryglobulinemia revealed by subarachnoid hemorrhage

Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis mediated by intravascular deposition of immune complexes. We report a 55-year-old man with PAN revealed by CNS vasculitis with subarachnoid hemorrhage and brainstem vasogenic oedema, associated with essential mixed cryoglobulinemia. In spite of aggressive treatment (steroids and cyclophosphamide), the patient became tetraplegic. A pathophysiological relationship between cryoglobulin and PAN can be suspected. Mixed cryoglobulinemia may trigger vasculitis with PAN features, possibly explaining the lack of treatment response.     

Polyarteritis nodosa revisited

Polyarteritis nodosa (PAN), the prototype of systemic vasculitis, is a rare condition characterized by necrotizing inflammation of medium-sized or small arteries without glomerulonephritis or vasculitis in arterioles, capillaries, or venules. Signs and symptoms of this disease are primarily attributable to diffuse vascular inflammation and ischemia of affected organs. Virtually any organ with the exception of the lungs may be affected, with peripheral neuropathy and symptoms from osteoarticular, renal artery, and gastrointestinal tract involvement being the most frequent clinical manifestations. A clear distinction between limited versus systemic disease and idiopathic versus hepatitis B related PAN should be done because there are differences in the implicated pathogenetic mechanisms, their treatment, and prognosis. Currently, corticosteroids plus cyclophosphamide is the standard of care for idiopathic PAN, in particular for patients with adverse prognostic factors (more severe disease), in whom this combination prolonged survival. In contrast for hepatitis B related PAN treatment consists of schemes that include plasmapheresis and antiviral agents.     

Polyarteritis nodosa in childhood

The clinical and pathologic findings of 2 infants and 7 older children with polyarteritis nodosa who were autopsied are reported. The most frequent clinical features included prolonged high fever, skin rash, abdominal symptoms, leukocytosis, proteinuria, and signs of either cardiac or renal failure. The 2 infants died of cardiac arrest, whereas renal or neurologic involyement was the most common cause of death in the older children. A consistent finding at autopsy was arteritis of the epicardial coronary arteries.     

Polyarteritis nodosa associated with ulcerative colitis

Three months after colectomy for severe ulcerative colitis, an 18-year-old male presented with a multisystem illness characterized by fever, arthralgias, hypertension, and declining renal function. A diagnosis of polyarteritis nodosa was made histologically and confirmed angiographically. Clinical and pathological findings suggest that the patient had 2 separate illnesses. Potential implications of this unique association are briefly discussed.     

Polyarteritis nodosa presenting as polymyositis

BACKGROUND: Skeletal muscle involvement has been well documented in patients with polyarteritis nodosa (PAN), and symptoms referable to skeletal muscle are not uncommon. However, polymyositis as a mode of presentation of PAN is uncommon. This unusual presentation of PAN has been reported only once previously in the English literature. OBJECTIVE: This study describes a patient who had diffuse weakness, myalgias, and markedly elevated serum creatinine phosphokinase, mimicking polymyositis. The literature dealing with the clinical aspects of muscle involvement in PAN is reviewed. RESULTS: A 24-year-old man was admitted to the hospital with a 1-month history of fever, myalgia, and muscle weakness. Necrotizing vasculitis was shown on subsequent muscle biopsy, consistent with PAN. Literature review indicated that muscle involvement is common in PAN, as has been shown by the frequency of muscular symptoms and by histologic evidence obtained from both clinical and autopsy studies. Nineteen percent of patients with PAN had documented myopathy, and autopsy series have shown skeletal muscle involvement in 30% to 48% of cases. However, polymyositis as a mode of presentation of PAN is rare. We found only 1 other patient with PAN who had elevated creatinine phosphokinase and diffuse myopathy suggestive of polymyositis. CONCLUSIONS: PAN should be suspected in cases of focal or diffuse myopathy, especially in the context of a systemic disease. Biopsy of symptomatic muscles or EMG-directed biopsies can be helpful in establishing a diagnosis of PAN to allow the physician to provide early treatment.     

Polyarteritis nodosa and myocardial infarction

A 35-year-old man with a history of polyarteritis nodosa is presented. The patient presented with acute anterolateral myocardial infarction that was complicated by diffuse coronary artery aneurysms found during cardiac catheterization. The complication of acute myocardial infarction in a patient with diffuse coronary artery aneurysms associated with polyarteritis nodosa is rare.     

Polyarteritis nodosa mimicking prostatic cancer

We describe a 72-year-old man with prostate enlargement, prostate-specific antigen level of 35 ng/dl, mild polyarthritis, and constitutional symptoms. Prostatic ultrasonography suggested neoplasm; however, transrectal biopsy revealed findings consistent with polyarteritis nodosa (PAN). The patient went on to develop leg paresthesia and dysesthesia, increased serum creatinine, and systemic hypertension. Steroids and intravenous cyclophosphamide were administered, followed by improvement. Our case emphasizes the protean onset of PAN, and provides a new differential diagnosis of prostatic diseases related to elevated prostate-specific antigen.     

Polyarteritis nodosa associated with acute cytomegalovirus infection.

A case of polyarteritis nodosa is described in which the onset of the disease was associated with acute infection by cytomegalovirus. Peripheral neuropathy was the predominant clinical feature, and death occurred 4 years after the onset. This is the first recorded case of polyarteritis in which cytomegalovirus is of possible aetiological significance.     

Polyarteritis nodosa and the antiphospholipid syndrome

Summary We report a case of biopsy-proven polyarteritis nodosa (classic type) in association with the antiphospholipid syndrome.Medium-sized arteriopathy was confirmed on visceral angiography. Elevated anticardiolipin antibodies were detected before initiating therapy with methylprednisolone and IV pulse cyclophosphamide. Rapid subsidence of symptoms correlated with a gradual normalisation of the erythrocyte sedimentation rate. After 6 months of therapy anticardiolipin antibodies were within normal limits. Only one similar case has been reported so far.