A patient with fulminant systemic vasculitis type polyarteritis nodosa and negative histology of small bowel infarction

A patient with fatal polyarteritis nodosa is described. In spite of overwhelming clinical signs the diagnosis of PAN could not be confirmed by angiography nor histology of macroscopic clearly involved small bowel tissue. Histology of lower limb tissue after amputation confirmed the diagnosis.     

Relapsing cutaneous polyarteritis nodosa associated with streptococcal infections

BACKGROUND: Polyarteritis nodosa is an aggressive, often fatal form of vasculitis associated with multi-organ involvement. Cutaneous polyarteritis nodosa is purported to be a more benign form of this disorder with involvement limited to the skin. METHODS: The identification of a female patient from childhood to adulthood documenting repeated episodes of cutaneous polyarteritis nodosa following bouts of recurrent streptococcal pharyngitis. RESULTS: Repeated bouts of streptococcal pharyngitis at ages 11, 28, and 33 years were followed by episodes of cutaneous polyarteritis nodosa, documented by histopathologic skin changes and clinical presentation, and confirmed by therapeutic management. CONCLUSIONS: Various infectious and non-infectious conditions have been linked both to the initiation and relapse of this disease. We describe a patient with recurrent episodes of cutaneous polyarteritis nodosa spanning a period of over 20 years with each episode appearing to be linked to a prior streptococcal infection.     

Inferior suprarenal artery aneurysm in polyarteritis nodosa

A 16-year-old boy with the complaints of fever, abdominal pain, arthralgia and hypertension was found to have polyarteritis nodosa. Selective renal angiography disclosed a 5-mm microaneurysm of the right inferior suprarenal artery, which has not been previously reported in polyarteritis nodosa. Aside from the diagnostic view-point, some of the perirenal and retroperitoneal hemorrhages of unknown origin seen in polyarteritis nodosa might be due to the rupture of suprarenal artery aneurysms.     

Polyarteritis nodosa and antiglomerular basement membrane disease without antineutrophil cytoplasm antibodies

Antiglomerular basement membrane (GBM) antibodies have been described previously in patients with microscopic polyarteritis but not in patients with polyarteritis nodosa alone. Where anti-GBM antibodies occur in microscopic polyarteritis, antineutrophil cytoplasm antibodies (ANCA) are usually present. We describe here a patient with polyarteritis nodosa and anti-GBM antibodies in whom ANCA could not be demonstrated. A 72-year-old woman presented with abdominal pain, diarrhoea and acute renal failure. A renal biopsy showed crescentic glomerulonephritis and linear immunofluorescence of the GBM consistent with anti-GBM disease. In addition, there was evidence of large-and medium-sized vessel vasculitis on abdominal angiography, performed because of persisting abdominal pain. There was no small vessel vasculitis on histological examination of the renal biopsy and ANCA could not be demonstrated by indirect immunofluorescence or ELISA.     

Characteristics of the effect of parathyroid hormone on rat myocardial relaxation]

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.     

Pregnancy and intestinal inflammatory disease

Classic polyarteritis nodosa (PAN) is a rare necrotising vasculitis and can result in intrarenal or perirenal bleeding because of aneurysm formation. This report describes the case of a patient with severe manifestation of PAN who developed a subcapsular hematoma from a spontaneously ruptured aneurysm in the spleen and a retroperitoneal hematoma from a renal cortex aneurysm. For the first time angiographic controls have been documented over years in this disease. The value of angiographic procedures for diagnosis and for evaluation of the disease course of PAN is discussed.     

Intrahepatic bile duct injury and nodular regenerative hyperplasia of the liver in a patient with polyarteritis nodosa

Although involvement of the hepatic vasculature in patients with polyarteritis nodosa is not unusual, biliary manifestations are very rare. We describe a patient with polyarteritis nodosa presenting with a febrile cholestatic anicteric syndrome. Histological examination of the liver revealed necrotizing arteritis of small hepatic arteries associated with significant lesions of intrahepatic bile ducts of the sclerosing cholangitis type, i.e. fibrous collar around the ducts, periductal inflammation and ductal proliferation. Concomitant nodular regenerative hyperplasia was found, a condition which has rarely been described in association with polyarteritis nodosa. We think that hepatic arteritis compromising arterial blood flow to the liver was responsible for the most likely ischemic nature of the bile duct injury and the nodular regenerative hyperplasia seen in our patient.     

Steroid and non-steroidal anti-inflammatory drug therapy of vasculitis

Before corticosteroids were used in the treatment of the systemic necrotizing vasculitides of the polyarteritis nodosa, the disease almost always progressed with variable degree of fulminance leading to death. The use of corticosteroids improves the 5-year survival. The most dramatic break-through in the therapeutic approach to the vasculitides has been clinical experience with cyclophosphamides in treating Wegener's granulomatosis. With this therapeutic regimen, Wegener's granulomatosis can now be considered a curable disease, if treated early and appropriately with therapeutic regimen. These therapeutic strategy can be extended to that of polyarteritis nodosa group of vasculitides. Now, it is clear that cyclophosphamide, either alone or in combination with pre-existing corticosteroid therapy, can effect a dramatic response if instituted early in the course of the disease process. With the advent of deeper insight as to immuno-pathogenesis and pathophysiologic mechanism, the striking therapeutic success can be expected in near future.     

Severe preeclampsia superimposed on polyarteritis nodosa

A patient with a history of drug abuse and histologically diagnosed hepatitis B-related polyarteritis nodosa was admitted for severe hypertension. After a cesarean section because of worsening of her clinical status with severe proteinuria and edema, she improved dramatically. No sign of acute polyarteritis nodosa occurred.     

A case of microscopic polyarteritis nodosa with interstitial pneumonia successfully treated with steroid pulse therapy and immunosuppressive agents

We report a patient with microscopic polyarteritis nodosa (mPN) and interstitial pneumonia, who was subjected to investigation by bronchoalveolar lavage (BAL), thoracic computerized tomography (CT) and gallium-67 citrate (67Ga) scintigraphy before and after administration of glucocorticoid and immunosuppressive agents. Renal function, renal histology, interstitial inflammation of the lung, and pulmonary function and histology improved cytoplasmic autoantibody (MPO-ANCA), which decreased with decreasing disease activity after starting treatment. Interstitial pneumonia may be associated with pulmonary capillaritis due to mPN. Methylprednisolone pulse therapy followed by oral prednisolone and immunosuppressive agents is considered to be an effective therapeutic strategy for combined mPN and interstitial pneumonia.     

Meditation in palliative care--a practical tool for self-management

A thirty four-year-old-white man in good health developed an acute anterior wall myocardial infarction (AMI), Killip II with normal coronary arteries. No thrombolytic therapy was given. Selective angiography revealed multiple aneurysms in mesenteric and renal arteries. The diagnosis of polyarteritis nodosa (PAN) was performed. AMI in PAN is secondary to arteritis with thrombosis, or to atherosclerosis due to steroid therapy. This case, having multiorgan vascular aneurysms involvement without previous cardiac symptomatology nor steroid therapy, presented as his first cardiac complication an AMI with normal coronary arteries probably due to selective arteritis.     

Deletion and down-regulation of mts1/p16 gene in human gastric cancer]

An adult male diver developed limb pains and peripheral neurologic signs following a series of dives. He was treated for acute neurologic decompression illness (DCI) and responded well, but subsequently he deteriorated and developed features of a progressive multisystem disease. Investigation confirmed a clinical diagnosis of polyarteritis nodosa complicated by a mononeuritis multiplex. Vasculitis is uncommon and its masquerade as neurologic DCI may be unique. However, this case empha-sizes the importance of careful clinical assessment and illustrates the potentially wide differential diagnosis of DCI.     

Isolated ovarian polyarteritis nodosa

Polyarteritis nodosa is a rare disorder and a form of systemic vasculitis. A 48 year-old female was admitted to the hospital because menorrhagia and pelvic pain in February 1993. The patient underwent exploratory laparotomy resulting in a total hysterectomy and bilateral salpingo-oophorectomy for myoma of uterus and a right adnexal cystic mass. Histopathologic examination revealed left ovarian periarteritis nodosa. Further investigation and 9 months follow-up failed to show any systemic involvement. To our knowledge the isolated ovarian polyarteritis nodosa is the first case in the literature.     

Cryptosporidium parvum: an attempt at experimental infection in rainbow trout Oncorhynchus mykiss

Segmental mediolytic arteriopathy, a rare, noninflammatory arterial disease, is fundamentally a variant of fibromuscular dysplasia. The characteristic angiographic findings of segmental mediolytic arteriopathy include the "string of beads" and microaneurysms which are indistinguishable from those of vasculitis, and the correct diagnosis can be made only after histopathologic evaluation of the arterial lesions. Thrombosis, arterial wall hemorrhage, and dissection are among the complications of segmental mediolytic arteriopathy. We describe herein a patient with segmental mediolytic arteriopathy who presented with hemoperitoneum. The patient underwent urgent surgical repair of a ruptured hepatic artery aneurysm. The postoperative visceral arteriography findings led to a clinical diagnosis of polyarteritis nodosa, and immunosuppressive therapy was initiated. This treatment was stopped as soon as the correct biopsy diagnosis of segmental mediolytic arteriopathy was obtained through outside consultation. The patient recovered without drug treatment and was spared the potentially life-threatening complications of immunosuppression.     

Polyarteritis nodosa and the antiphospholipid syndrome

We describe a case of classical polyarteritis nodosa (PAN) with visceral aneurysms presenting with renal infarction and hypertension. The female patient also had all the laboratory features of the antiphospholipid syndrome (APS) and 2 months into her illness developed a large iliofemoral thrombosis. She responded well to immunosuppressive therapy and anti-coagulation. Repeat arteriogram showed regression of the visceral aneurysms. The link between PAN and APS, and the therapeutic dilemma posed by this association, are discussed.     

Polyarteritis nodosa associated with hepatitis C virus

BACKGROUND: To study the seroprevalence of hepatitis C virus in a cohort of six patients with a diagnosis of polyarteritis nodosa (PAN). METHODS: There have been included six patients with a diagnosis of PAN, carrying out a serodiagnosis of hepatitis B virus (VHB) and C (VHC) this last one by means of the following methods: ELISA, RIBA-II and PCR. RESULTS: These cases (50%) showed exclusive positivity to VHC by means of the three ways of diagnosis, two cases showed positivity to VHB (33.3%), one case (16.6%) showed positivity to both virus (VHB and (VHC) and one case didn't show positivity virus. CONCLUSIONS: It is probable a ethipatogenic relation between hepatitis C virus and polyarteritis nodosa, our sample doesn't show any difference from that written in the literature. The positive rheumatoid factors can give false positive for VHC by means of the technique ELISA because of this it is necessary to confirm the positive by means of the techniques RIBA-II and PCR.     

Antineutrophil cytoplasmic antibodies (ANCA) and abnormal angiograms in polyarteritis nodosa and Churg-Strauss syndrome: indications for the diagnosis of microscopic polyangiitis

Microscopic polyangiitis (MPA) is one of the vasculitides previously included in the polyarteritis nodosa (PAN) group. A diagnosis of MPA is usually considered when glomerulonephritis and/or lung hemorrhage are present. Small-sized vessels are involved and this sign is considered, for some authors, to be the main diagnostic criterion. The present study attempted to define clinical, radiological and immunological characteristics of MPA and to separate them from classic PAN (c-PAN) and Churg-Strauss syndrome. We have shown that, in most cases, patients presenting microaneurysms and/or multiple vessel stenoses, which reflect medium-sized vessel involvement, do not have ANCA. Conversely, patients with glomerulonephritis have almost never had abnormal angiograms. Furthermore, the clinical characteristics of ANCA-positive patients also indicate small-sized vessel involvement. Although at present it is not possible to definitively separate MPA from c-PAN, our results show that ANCA should be considered diagnostic for MPA and, in most cases, should be an exclusion criterion for c-PAN.     

Isolation and characterization of a novel facultatively alkaliphilic Nitrobacter species, N. alkalicus sp. nov

BACKGROUND: The American College of Rheumatology (ACR) established criteria to discriminate among patients with seven types of vasculitis. Although designated as "classification criteria" for research, these criteria are often used for diagnosis. OBJECTIVE: To examine the operating characteristics of the 1990 ACR classification criteria in the diagnosis of Wegener granulomatosis, giant-cell arteritis, polyarteritis nodosa, and hypersensitivity vasculitis. DESIGN: Prospective cohort study. SETTING: University medical center and Veterans Affairs medical center. PATIENTS: 198 consecutive patients referred to rheumatologists for evaluation of possible vasculitis. Measurements: Blinded chart audits were done to classify patients according to the 1990 ACR classification criteria for Wegener granulomatosis, polyarteritis nodosa, giant-cell arteritis, and hypersensitivity vasculitis on the basis of the patients' initial presentation. Chart audits done 2 to 8 months after baseline provided the patients' final diagnoses, which were considered the gold standard, as in the development of the ACR criteria. Test operating characteristics of the ACR classification criteria were calculated according to 2 x 2 tables for the entire cohort and for only the patients with a final diagnosis of vasculitis. RESULTS: Vasculitis was diagnosed in 51 (26%) patients. Thirty-eight (75%) of 51 patients with vasculitis and 31 (21%) of 147 patients without vasculitis met ACR criteria for one or more types of vasculitis. The positive predictive values for the four vasculitides according to ACR criteria were 17% to 29% for the entire cohort and 29% to 75% for only the patients with a final diagnosis of vasculitis. CONCLUSION: The 1990 ACR classification criteria function poorly in the diagnosis of specific vasculitides.     

Immunohistochemistry of secretory particles (''vesiculosomes'') from the epithelium of bovine seminal vesicles and ampulla of the vas deferens

Several syndromes involving antiphospholipid antibodies have been described in the literature. Although the varied clinical manifestations have been well delineated, the vascular pathophysiology in patients with these antibodies remains unclear. Vascular damage is often described as a vasculopathy; however, several case reports have described an associated vasculitis. We report two patients with manifestations of antiphospholipid antibody syndrome (APLS) and concurrent vasculitis. The first patient, a 42-year-old man, presented with abdominal pain and fevers. The second patient, a 39-year-old man, presented with fever and testicular pain. Both were ultimately felt to have polyarteritis nodosa associated with APLS. Their complicated hospital courses and difficulties we encountered in diagnosing and treating them are discussed. The literature describing other cases of vasculitis associated with antiphospholipid antibody syndrome is reviewed. Whether the presence of antiphospholipid antibodies favors the development of vasculitis or vice versa is not clear. Further studies are needed to address this question and to determine optimal therapeutic regimens in these critically ill patients.