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[Angina without involvement of large coronary trunks and precapillary pulmonary hypertension in a patient with periarteritis nodosa].

A patient affected with periarteritis nodosa and angina, presented a normal coronary angiography of the coronary trunks and an alteration of the left ventricular systolic function, improved by nicardipine, as well as a pre-capillary pulmonary hypertension with isolated, right cardiac repercussions, without pulmonary fibrosis nor thromboembolic disease. In addition, he presented three transient cerebral ischaemic accidents, with different localizations, which could not be related to a right aneurysm of the sylvian artery. Isolated precapillary pulmonary hypertension, without pulmonary fibrosis, which is not described in the course of periarteritis nodosa, seems related to an arteriolar involvement which is the known substratum of other visceral involvement in periarteritis nodosa. Improvement of the left ventricular function with nicardipine, administered acutely, suggest a partially reversible disorder of the coronary microcirculation.

Angina Pectoris↗

[Arteritis and fatty degeneration of the spermatic cord resembling periarteritis nodosa: a case report].

A 23-year old male was admitted to our hospital because of swelling of the left scrotum for one month without any particular past history. Blood count, chemistry, urinalysis, chest X-ray and electrocardiogram revealed normal findings. Scrotal exploration was performed. A soft, dumbbell-like tumor enveloped in a thin membrane was found above the left testis. Pathology revealed fibrinous exudation and fibrinoid necrosis in the whole vessel wall indicating resemblance to the panarteritis in periarteritis nodosa. Some granulomatous lesions with many histiocytes were also noted around these arteries. The findings suggested that a inflammation like periarteritis nodosa had occurred at the spermatic cord and subsequently developed into fatty degeneration in the surroundings. Since local periarteritis nodosa-like lesion may progress to systematic disease, further observation is mandatory for this case.

Adult↗

[Periarteritis nodosa in an addict with a hepatitis B injection (author's transl)].

The authors describe a case of a drug addict with a hepatitis B who was found to have periarteritis nodosa and a nephrotic syndrome. The course was rapidly fatal and autopsy confirmed the diagnosis. This new case questions the hypothesis suggested of possible toxicity of hard drugs (especially the amphetamines) as being an etiological factor for periarteritis nodosa observed in addicts. Hepatitis B virus, which is involved in the etiology of periarteritis nodosa and some nephrotic syndromes (also reported in addicts) is, in fact, very prevalent in users of hard drugs by the parenteral route.

Adult↗

Cutaneous periarteritis nodosa: immunofluorescence studies.

The study of ten cases of cutaneous periarteritis nodosa by direct immunofluorescence microscopy of excision biopsy specimens revealed positive findings in nine. Deposition of IgM was found in the vessel walls in six cases, and C3 was observed in four; however, only in two cases was C3 found with IgM in the vessel walls. Deposits of IgM in the superficial vessels were found in five cases, although only the deep muscular vessels showed evidence of the vasculitis in cutaneous periarteritis nodosa. Serum hepatitis B antigen was absent in the nine cases that were tested. Positive immunofluorescence findings in superficial as well as in deep vessels suggest that although periarteritis nodosa is characterized by an inflammatory panarteritis, occasional vasculitis of small vessels might be expected.

Complement C3↗

[Polyneuritis in periarteritis nodosa].

The authors observed 16 patients suffering from nodular periarteritis with the polyneuritis syndrome. The development and clinical characteristics of polyneuritis due to nodular periarteritis were studied, its course and outcome were also recorded. Some data helpful for recognizing the etiology of polyneuritis are presented. It is demonstrated that when multiple modality treatment (with corticosteroids as a component) is initiated at the early stages of disease, some patients exhibit prolonged remission of nodular periarteritis, including notable regression of polyneuritis manifestations.

Adolescent↗

Course of cerebral lesions in a patient with periarteritis nodosa studied by magnetic resonance imaging.

The course is reported of a patient with periarteritis nodosa who initially presented with neurological symptoms. Multiple cerebral lesions were documented by the first magnetic resonance imaging (MRI) investigation. The majority of these had disappeared completely in the follow-up MRI studies. In contrast to neurological improvement the patient eventually died due to multiorgan failure. Postmortem histological examination revealed no pathological findings in the brain except one single necrotic area already known from MRI. Remissions of histological and angiographic alterations in periarteritis nodosa have been described as "local healing" leading to fibrosis and scarring. Our findings suggest that restitutio ad integrum may occur, at least in cerebral lesions.

Adult↗

Immune complexes in hepatitis B antigen-associated periarteritis nodosa. Detection by antibody-dependent cell-mediated cytotoxicity and the Raji cell assay.

A subpopulation of peripheral blood lymphocytes with the ability to lyse target cells coated with specific antibody (antibody-dependent cell-mediated cytotoxicity, ADCC) was serially studied in a patient with hepatitis B antigen-associated periarteritis nodosa. The effector lymphocytes possess FC and complement receptors but do not require complement for functional activity. We found that the patient's ADCC was decreased during periods of disease activity and was almost normal during remission. The patient's serum could block ADCC in normal lymphocytes, and the blocking ability correlated with the concentration of immune complexes as determined by the Raji cell assay (a radioimmunoassay using complement receptors on human cultured lymphoblastoid cells). The concentration of immune complexes and the ADCC blocking ability of ther serum both correlated with disease activity. Serum from five other patients with active vasculitis was found to contain significant amounts of immune complexes and was able to block normal ADCC. It appears that the ADCC assay can be used to detect the presence of circulating immune complexes and to monitor disease activity in periarteritis nodosa.

Adult↗

Hepatitis B antigen-associated periarteritis nodosa in patients undergoing long-term hemodialysis.

Periarteritis nodosa was observed in three of 266 persistent hepatitis B antigen (HBsAg) carriers undergoing long-term hemodialysis; no cases of necrotizing vasculitis occurred among 384 other patients undergoing dialysis having either no or transient antigenemia. Circulating e antigen, but no e antibody, was found in two of these three patients. The serum level of the third component of complement (C3) was normal in two patients and low in the third. Circulating immune complexes were demonstrated in all three patients, using polyethylene-glycol (PEG) precipitation, PEG-C4, and solid phase C1q tests. HBsAg and anti-hepatitis B antibody (HBsAb) were identified in the PEG precipitates using radioimmunoassay and electron microscopy technics. Direct immunofluorescence performed on a muscle biopsy specimen from one patient was positive for HBsAg, but not for immunoglobulin G (IgG), immunoglobulin M (IgM), C3 or C1q. These data support the hypothesis that circulating immune complexes involving HBsAg may be involved in the pathogenesis of periarteritis nodosa.

Antigen-Antibody Complex↗

[Interferon-alpha and ribavirin treatment in a patient with hepatitis C virus-associated cutaneous periarteritis nodosa].

BACKGROUND: Hepatitis C virus (HCV) frequently causes leucocytoclastic vasculitis as a result of type II or III cryoglobulinemia. HCV-associated vasculitis without cryoglobulinemia is less common. PATIENTS AND METHODS: A 33-year-old woman consulted for infiltrative necrotic purpura of the lower limbs, responsible for leg ulcers measuring less than 1 cm. Histopathological examination revealed vasculitis affecting the hypodermic arterioles and caused by periarteritis nodosa. No extracutaneous involvement was observed. The patient had presented asymptomatic untreated HVC infection (genotype 3) for two years. Antiviral treatment resulted in elimination of the patient's viremia and no relapse of skin lesions was observed two years after the end of treatment. COMMENTS: This patient presented vasculitis due to cutaneous nodular periarteritis associated with HVC without cryoglobulinemia. Hepatic impairment was mild and did not require any antiviral treatment. No further skin involvement was seen after treatment with colchicine and because the patient's viral genotype was favorable, we decided to initiate antiviral therapy. This therapeutic approach should be considered by dermatologists, but it is nevertheless important to assess the risk of interferon-induced aggravation of vasculitis.

Adult↗

An unusual presentation of periarteritis nodosa.

Periarteritis nodosa is characterized by generalized inflammation of medium and small arteries that leads to thrombosis or aneurysmal dilatation. We report the case of a 30-year-old man with no preceding signs or symptoms who presented initially with a chief complaint of mild abdominal pain. He deteriorated clinically during the next six to eight hours, and developed shock secondary to a ruptured aneurysm of the left gastric artery. His postoperative course was complicated by recurrent bleeding and death within 48 hours. Our case represents a protean clinical manifestation of periarteritis nodosa and expands the differential diagnosis of acute abdominal pain.

Abdomen↗

[Periarteritis nodosa disclosed by epilepsy in a drug addict with hepatitis B and C virus carrier state].

The authors report a case of periarteritis nodosa (PAN) with an inaugural symptom of febril epilepsy. The patient was drug addict with hepatitis B and C virus. A toxoplasmic lesion originally noted on the cranial computed tomography scan was confirmed by cerebral magnetic resonance imaging showing encephalitis, and a diagnosis of periarteritis nodosa was suggested by clinical signs and laboratory data. The responsibility of hepatitis B virus in the genesis of this PAN seems quite certain whereas possible others factors such as hepatitis C virus or cocaine are discussed.

Adult↗

[Recurrent periarteritis nodosa with predominantly cerebral signs in children (author's transl)].

Periarteritis nodosa occurs also in children and has the same wide spectrum of signs and symptoms as in adults. In a 4 1/2 year-old girl the disease at first was characterised by cerebral, encephalitis-like symptoms, arterial hypertension, intestinal wall necrosis and, finally, pericardial tamponade as a result of rupture of an arteritic coronary artery aneurysm. An earlier attack had involved liver and skeletal muscle, with necrosis in the liver. Peculiar morphological features within the "classical" course of periarteritis nodosa are endarteritic changes of single retroperitoneal and coronary arteries.

Aneurysm↗

Management of intra-abdominal aneurysms associated with periarteritis nodosa.

Periarteritis nodosa is a disease of small and medium-sized arteries, frequently associated with multiple visceral artery aneurysms. Infrequently, these aneurysms rupture, usually with fatal results. A case of spontaneous rupture of a middle colic artery aneurysm in a patient with periarteritis nodosa is reported, and similar cases in the literature are reviewed. Treatment of a ruptured visceral artery aneurysm requires ligation or resection of the aneurysm without delay. Residual aneurysms are treated with cyclophosphamide and/or prednisone in an attempt to induce regression of the aneurysms. An arteriogram performed after 3 to 4 months of medical therapy determines the need for further surgical intervention.

Aneurysm↗

An autopsy case of isolated eosinophilic coronary periarteritis: a limited form of Churg-Strauss syndrome or a new entity?

A 52-year-old man without a history of asthma or allergic diseases died of ventricular fibrillation early in the morning. His autopsy revealed no significant findings, except for a mild mural-thickening localized at the proximal region of the right coronary artery. Microscopic examination showed periarteritis with infiltration of numerous eosinophils in the adventitia. No significant vasculitis was found in any other organs. Based on the findings this seems to be the second reported case of isolated eosinophilic coronary periarteritis.

Arteritis↗

Pulmonary artery stenosis and periarteritis nodosa.

A severe arterial stenosis involving the origin of the posterior and anterior basal branches of the right lower lobe artery was discovered in a 86 year-old Caucasian woman who presented with a typical periarteritis nodosa. We believe it was specific because its discovery was concomitant to the diagnosis of periarteritis nodosa and no other cause was obvious.

Aged↗

Periarteritis nodosa in rats treated with chronic excess sodium chloride (NaCl) after X-irradiation.

Five-week-old male Crj:CD (SD) rats were treated with excess sodium chloride after abdominal X-irradiation. The gastric regions of the rats were irradiated with a total dose of 20 Gy given in two equal fractions separated by 3 days. After X-irradiation, animals were fed a diet containing 10% sodium chloride. Red blood cell anemia appeared 22 weeks after the last irradiation. By gross observation, the mesenteric arteries became reddish in color, and bead- or lead pipe-like nodular thickenings were present. Microscopically, these nodularly thickened mesenteric arteries showed fibrinoid necrosis with massive inflammatory infiltration including eosinophils and neutrophils. In more advanced lesions, elastica interna and externa and medial smooth muscle cells disappeared completely and were replaced by granulation tissue. In old lesions, arterial walls were markedly thickened with fibrous or fibromuscular tissue. These findings were quite similar to those of the human periarteritis nodosa. These arterial lesions could not be found in the rats with X-irradiation only, sodium chloride only, or in nontreated animals. This study demonstrates X-ray-induced, NaCl-promoted periarteritis nodosa-like lesions in rats.

Animals↗

[Periarteritis nodosa associated with lung cancer. A new observation].

The cases of vasculitis associated to malignant tumour are uncommon, their course which is often parallel suggests a direct link between them. We report the observation of periarteritis nodosa associated with a lung cancer discovered in the following of vasculitis; whereas the course is favorable in 83 years old man without particular history. The frequency of neoplasia with vasculitis is estimated between 3 and 8%, and periarteritis nodosa can't be a paraneoplastic syndrome. Lung and colic tumours are the most frequent reported of the solid tumours. The delay of appearance of vasculitis varies from 25 months before to 9 months after cancer diagnosis. There are many factors, either some treatment, the neoantigens involve the formation of immune complexes, lymphokines and some vasoactive substances.

Aged↗

[Periarteritis nodosa].

Periarteritis nodosa is a systemic vasculitis involving arteries with a diameter greater than 70-150 microns. Large vaccination against hepatitis B virus explains the decreased frequency of its association with hepatitis B virus observed in less than 10% of cases. Weight loss, fever, livedo, myalgias or arthralgias, mononeuropathy, gastrointestinal tract involvement, renal insufficiency, and cardiac failure are the most frequent clinical manifestations. Inflammatory syndrome and increased white blood cell count are common. Renal or digestive microaneurysms are present in more than 60% of cases. Prognosis is dependent of a five factors score corticosteroids with or without immunosuppressive drugs, result in 5-year survival rate over 60%. In hepatitis B virus-related periarteritis nodosa, corticosteroids are rapidly stopped; plasma exchanges in combination with antiviral agents (interferon alpha or lamivudine) give excellent results.

Humans↗