Necrotizing mesenteric vasculitis after longstanding cutaneous polyarteritis nodosa.

We describe a 21-year-old woman with cutaneous polyarteritis nodosa (PAN) who developed necrotizing mesenteric vasculitis 6 years after the onset of skin disease. Repeated investigation during that 6-year interval failed to reveal any evidence of systemic PAN. We were unable to wean the patient from prednisone or completely control her skin disease with topical and systemic therapy, including supersaturated potassium iodide, dapsone, azathioprine, methotrexate or plasmapheresis before the development of the mesenteric vasculitis, which responded to cyclophosphamide and prednisone. Development of visceral vasculitis after long-standing cutaneous PAN has very rarely been reported.     

Diffuse endothelial dysfunction is common to ANCA associated systemic vasculitis and polyarteritis nodosa

BACKGROUND: Excess cardiovascular mortality complicates systemic rheumatic disease, suggesting an accelerated atheromatous process, which it has been proposed relates to the vascular inflammation common in such diseases. Impaired endothelium dependent vasodilatation is an early marker of atheromatous disease. It has previously been shown that such endothelial cell dysfunction (ECD) occurring in the brachial artery can complicate primary systemic necrotising vasculitis (SNV). OBJECTIVE: To determine if ECD occurs in a wider spectrum of primary SNV, if it is restricted to the major arteries, and whether vasculitis subgroup, ANCA status, or renal involvement influenced the endothelial responses. METHODS: Fifty four patients attending the Birmingham vasculitis clinic, including patients with a range of ANCA and non-ANCA associated primary vasculitides, and a group of age matched controls were recruited. The length of patient follow up and disease activity was variable. Disease activity, damage scores, and cardiovascular risk factors were recorded before assessment of flow mediated brachial artery vasodilatation by high resolution ultrasound. Dermal microvascular responses to acetylcholine were also measured in 32 patients and 21 controls by laser Doppler flowmetry. RESULTS: ECD was demonstrated in all primary SNV subgroups of patients with ANCA associated vasculitis and in polyarteritis nodosa, compared with controls. Significant impairment occurred in both vascular beds, regardless of vessel size targeted in the inflammatory vasculitis, ANCA association and titre, or renal involvement. CONCLUSIONS: Diffuse endothelial dysfunction, a predictor of atherosclerotic disease, is found extensively in primary systemic vasculitis. Involvement of different vascular beds is independent of target vessel size or ANCA association, and is unrelated to local disease expression. It is suggested that this results from a systemic response that may be a consequence of primary vasculitis, but is distinct from the local inflammatory vasculitic process.     

Minocycline-induced vasculitis fulfilling the criteria of polyarteritis nodosa

Abstract

A 47-year-old man who had been taking minocycline for palmoplantar pustulosis developed fever, myalgias, polyneuropathy, and testicular pain, with elevated C-reactive protein (CRP). Neither myeloperoxidase- nor proteinase-3-antineutrophil cytoplasmic antibody was positive. These manifestations met the American College of Rheumatology 1990 criteria for the classification of polyarteritis nodosa. Stopping minocycline led to amelioration of symptoms and normalization of CRP level. To our knowledge, this is the second case of minocycline-induced vasculitis satisfying the criteria. Differential diagnosis for drug-induced disease is invaluable even for patients with classical polyarteritis nodosa.     

Simultaneous occurrence of polyarteritis nodosa and leukocytoclastic vasculitis

We report a patient with cutaneous vasculitis and mononeuritis multiplex. He was found to have both medium size arterial disease, characteristic of polyarteritis nodosa, and small vessel involvement, characteristic of leukocytoclastic vasculitis. This represents a rarely reported combination and serves to emphasize their usual nonoverlapping patterns. The apparent limitation upon the distribution of involved vessels may represent underreporting or signify that different pathophysiologic processes damage vessels of different sizes.     

Minocycline-induced vasculitis fulfilling the criteria of polyarteritis nodosa

A 47-year-old man who had been taking minocycline for palmoplantar pustulosis developed fever, myalgias, polyneuropathy, and testicular pain, with elevated C-reactive protein (CRP). Neither myeloperoxidase- nor proteinase-3-antineutrophil cytoplasmic antibody was positive. These manifestations met the American College of Rheumatology 1990 criteria for the classification of polyarteritis nodosa. Stopping minocycline led to amelioration of symptoms and normalization of CRP level. To our knowledge, this is the second case of minocycline-induced vasculitis satisfying the criteria. Differential diagnosis for drug-induced disease is invaluable even for patients with classical polyarteritis nodosa.     

Successful treatment of pediatric systemic polyarteritis nodosa with cholestatic hepatitis

Polyarteritis nodosa (PAN) is a rare vasculitis in children. We report a case of 5-year-old boy with systemic PAN and cholestatic hepatitis. He had fever, abdominal pain, and gastrointestinal bleeding. Angiography revealed multiple microaneurysms in the renal, hepatic, and superior mesenteric arteries. Clinical manifestations improved slowly after immunosuppressive therapy, but liver enzyme and bilirubin levels elevated gradually. Liver biopsy findings revealed marked centrizonal canalicular cholestasis, bile duct damage, and intact hepatocyte, but there was no evidence of viral hepatitis or vasculitis. Levels of liver enzymes and bilirubin improved after two cycles of cyclophosphamide therapy. We thought that the possible etiology of elevated liver enzyme and bilirubin levels might be a manifestation of PAN.     

Acute abdominal complications of systemic lupus erythematosus and polyarteritis nodosa

Fifteen (11 percent) of 140 patients with systemic lupus erythematosus (SLE) and five (31 percent) of 16 patients with polyarteritis nodosa (PA) developed disease-related signs and symptoms of an acute surgical abdomen. Peripheral vasculitis (p < 0.025), nervous system involvement (p < 0.05), ischemic necrosis of bone (p < 0.05), thrombocytopenia (p < 0.01), and circulating rheumatoid factor (p < 0.01) are all significantly more prevalent in patients with SLE and abdominal involvement compared with those without an abdominal syndrome. Similarly, except for ischemic necrosis of bone, these same features are more prevalent in patients with PA and acute abdomens.Eleven (73 percent) of 15 patients with SLE had exploratory laparotomy for acute abdominal events. Nine were found to have evidence of intra-abdominal arterttis and only two had polyserositis without gross evidence of vasculitis. Eight (53 percent) of the 15 patients with SLE and acute abdomens died as a result of their abdominal crises. Six of the seven surviving patients represent those most recently seen and aggressively treated.All five patients with PA and acute abdomens required operative intervention and all had gross evidence of mesenterlc arterttis with large ischemic segments resulting in infarction and perforation. All died in contrast to only two (18 percent) of the 11 PA patients without abdominal involvement.     

Cyclophosphamide-induced remissions in advanced polyarteritis nodosa.

Two patients with far advanced polyarteritis nodosa involving multiple organ systems and with aneurysm formation demonstrable by celiac axis and renal arteriograms were treated with oral cyclophosphamide, 1 to 2 mg/kg/day, and alternate day prednisone therapy. Dramatic remissions of disease activity were achieved within weeks of initiation of therapy. Repeat angiograms obtained after one year revealed complete resolution of all aneurysms. Both patients are now receiving only cyclophosphamide and are maintained in complete remission 27 and 18 months after the start of therapy. Cyclophosphamide therapy can thus be highly effective even in far advanced polyarteritis nodosa.     

Hepatitis C virus in patients with polyarteritis nodosa. Prevalence in 38 patients.

In order to assess the prevalence of hepatitis C virus (HCV) in polyarteritis nodosa (PN), 38 patients with systemic necrotizing angiitis were retrospectively tested for the presence of anti-HCV antibodies (Ab). Twenty-one patients were hepatitis B virus (HBV) positive, comprising group A, and 17 were HBV negative, comprising group B. Two patients from group A had anti-HCV Ab (2/21: 9.5%). One was treated unsuccessfully with corticosteroids, then with vidarabine and plasma exchanges; HBe/anti-HBe seroconversion was not observed and anti-HCV Ab disappeared 8 months after the onset of PN. The second patient was successfully treated with corticosteroids, then vidarabine and plasma exchanges; he recovered from PN, HBV seroconversion occurred, and the anti HCV Ab remained detectable. These results show that: 1) the prevalence of anti HCV Ab in PN related to HBV is nearly the same (9.5%) as the prevalence of HCV Ab observed in patients with chronic hepatitis related to HBV infection; 2) the course of these two viral infections can be different and the role of HCV as an etiologic factor in PN has not been established.     

The challenge of managing patients with polyarteritis nodosa

PURPOSE OF REVIEW: In this short review we focus on the problems faced by clinicians caused by the changing definitions of polyarteritis nodosa. RECENT FINDINGS: The term polyarteritis nodosa has been used for more than 100 years as a diagnostic term for patients with systemic vasculitis; however, specific vasculitides have been singled out like branches being chopped off a tree. Now, so little is left of the trunk of that tree that it is questionable to what extent we can trust older literature with respect to clinical features, natural history and response to treatment. Many authors of case reports, as well as authors of reviews and book chapters, claim they adhere to the Chapel Hill Consensus Conference definition of polyarteritis nodosa, yet still cite almost exclusively studies using older definitions without highlighting this dilemma. In the past year, two proposals affecting classification have been published: one stating that cutaneous polyarteritis nodosa and hepatitis-associated polyarteritis nodosa are diseases distinct from classical polyarteritis nodosa, and one providing an algorithm to separate microscopic polyangiitis from classical polyarteritis nodosa. SUMMARY: There is hope that a wide acceptance of the new classification principles will lead to a more uniform way to diagnose classical polyarteritis nodosa, which will facilitate clinical studies and eventually improve management.     

Acute pancreatitis as a complication of polyarteritis nodosa.

CONCLUSIONS: Polyarteritis nodosa (PAN) must be considered as one of the rare causes of "idiopathic" acute necrotizing pancreatitis. BACKGROUND: PAN is characterized by panmural inflammation of arterioles causing arteriolar ectasia, aneurysm formation, and thrombosis, resulting in organ ischemia. METHODS: We report a case of necrotizing pancreatitis associated with segmental necrosis of the liver and spleen due to polyarteritis nodosa. RESULTS: Five previously reported cases of documented acute pancreatitis secondary to PAN have been identified from the English literature. The mechanism through which pancreatic ischemia results in acute pancreatitis is unknown. Although limited pancreatic infarction is common in PAN, necrotizing pancreatitis is rare, and the poor overall prognosis of PAN is owing largely to other organ complications.     

Mixed IgM-IgG cryoglobulinemia terminating in polyarteritis nodosa.

A case of mixed IgM-IgG cryoglobulinemia and polyarteritis nodosa is described. The patient exhibited non-deforming arthritis, Raynaud's phenomena, cutaneous vasculitis, and a sensory neuropathy.. The terminal phase of the illness was characterized by hypertension and retroperitoneal hemorrhage, with widespread large vessel arteritis. The isolated cryoprecipitate contained IgM, IgG, and C3 and possessed rheumatoid factor activity. Mild reduction in serum complement (CH50 and C3) was noted on one occasion. The evidence that these cryoprecipitable proteins may be acting as immune complexes and contributing to the arterial lesions is reviewed.     

Pneumatosis intestinalis in a patient with polyarteritis nodosa

Necrotizing enterocolitis of the small bowel in polyarteritis nodosa is rare. In the proper clinical setting, pneumatosis intestinalis and mesenteric air are characteristic of this entity.     

Immunosuppressive and corticosteroid therapy of polyarteritis nodosa.

The clinical manifestations, treatment and survival of 64 patients with polyarteritis nodosa seen from 1955 to 1977 were evaluated. In general, the patients had multisystem involvement. No patient with cutaneous vasculitis alone was accepted into the study. The clinical diagnosis was confirmed by biopsy in 34 patients, by autopsy in 13 and by angiography in 10. The patients were treated at the discretion of the physicians responsible for their care. Eight of the 64 patients received only supportive therapy (group 1), 34 received corticosteroids alone (group 2), and 22 received both corticosteroids and an immunosuppressive agent (group 3). Five patients in group 2 and one patient in group 3 were excluded from survival studies because of insufficient length of therapy. Patients in the three treatment groups were very similar with respect to 18 clinical and laboratory variables. Median survival times for the three groups were three months, 63 months and 149 months, respectively; 5 year survival rates were 12 per cent, 53 per cent and 80 per cent (p less than 0.05). Despite difficulty in precisely defining polyarteritis nodosa, the data suggest a better prognosis for treated patients than has previously been appreciated, with improvement in outcome when an immunosuppressive agent is added to corticosteroid therapy.     

Hemobilia as presenting manifestation of polyarteritis nodosa.

Polyarteritis nodosa (PAN) is a multisystem necrotizing inflammatory vasculitis of medium-sized muscular arteries. Hepatobiliary complications secondary to thrombosis or aneurysm formation in PAN are rare and are associated with poor outcome. We describe an 18-year-old man with PAN who presented with hemobilia secondary to rupture of a hepatic artery aneurysm, which was successfully managed with coil embolization followed by immunosuppressive therapy.