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PubMed1999

Periarteritis nodosa presenting as a breast lesion: report of a case and review of the literature.

We describe a 34-year-old woman with periarteritis nodosa (PAN) presenting as a breast lesion. Localized involvement of the breast is an unusual manifestation of PAN. To date, 10 cases have been reported: all were in women with an age range of 45-78 years (mean 63). In most cases, breast lesions were an isolated finding, and the prognosis was favourable, setting them apart from the more common form of systemic PAN. The case presented is unusual in that vasculitis developed in the postpartum period, and was associated with cutaneous PAN-like lesions elsewhere on the body, and digital artery occlusion. The most important differential diagnoses of PAN of the breast are infectious mastitis, mammary malignancy and other forms of idiopathic vasculitides of the breast, e.g. giant cell arteritis and Wegener granulomatosis.

Adult↗
PubMed1987

Suicide in a patient with undiagnosed periarteritis nodosa.

Surveys of medical examiners' records have shown that as many as one-third of suicide victims have serious physical illnesses. We report the case of a 56-year-old woman who committed suicide by drug overdose. At autopsy, she had severe widespread periarteritis nodosa, which had been unsuspected during life. The identification of treatable physical illness in suicide victims is important in suicide prevention, yet such illness may be overlooked after death unless a forensic autopsy is performed.

Female↗
PubMed1990

Retroperitoneal fibrosis after surgery for aortic aneurysm in a patient with periarteritis nodosa: successful treatment with corticosteroids.

A 54-year-old man with hepatitis B virus-related periarteritis nodosa developed retroperitoneal fibrosis with bilateral hydronephrosis 2.5 months after placement of an aortobifemoral prosthesis for abdominal aortic aneurysm. Retroperitoneal fibrosis disappeared after treatment with corticosteroids. This observation is interesting in the light of the hypothesis that retroperitoneal fibrosis is caused by vasculitis.

Aorta, Abdominal↗
PubMed1979

Periarteritis nodosa in spontaneously hypertensive rats -- incidence and distribution.

SHR rats (Nihon Rat Inc.) were used to study spontaneous arterial lesions morphologically resembling human periarteritis nodosa (PN). Male SHR rats (159) developed PN in 76.1%, whereas females (39) in 10.3% during a period of 2 to 15 months of age. The frequency of PN in male SHR rats tended to increase with ageing and reached 100% at 9 months of age. PN lesions were widely distributed with only the lungs, brain and aorta being spared. PN in females was restricted to the tongue, parametrium and mesenterium. Male SHR rats with hypertension of more than 180 mm Hg showed PN in 81.7%, whereas in females with the same hypertension PN was found in only 17.4%. Although there was apparent sex difference in the incidence and background of hypertension, male SHR rats might be a useful animal model of human PN.

Age Factors↗
PubMed1977

Infantile periarteritis nodosa or mucocutaneous lymph node syndrome. A report on four cases and diagnostic considerations.

Coronary artery aneurysm in childhood is a rare disease and has in most cases been ascribed to infantile periarteritis nodosa (IPN). In recent years a mucocutaneous lymph node syndrom (MLNS) has been found almost exclusively in Japan first described by Kawasaki 1967; this disease frequently involves the coronary arteries and myocardium. Four cases with coronary aneurysms are presented from Sweden and seem to be first described from Scandinavia. Three of these patients died a sudden death with cardiac arrest. Since MLNS and IPN have identical clinical and pathological features, we suggest that MLNS and IPN constitute a pathologic entity and that to separate them on a clinical or histological basis is nonsensical. The risk of coronary aneurysm and possible sudden death must be considered in patients with uncharacteristic symptoms including prolonged fever, conjunctivitis, exanthema, lesions in the oral mucosa, elevated sedimintation rate, and leukocytosis.

Cardiomegaly↗
PubMed1984

Chronic coronary periarteritis in two patients with chronic periaortitis.

Two cases of chronic coronary periarteritis associated with chronic periaortitis are described. In one, the chronic periaortitis took the form of perianeurysmal fibrosis around a thoracic atherosclerotic aneurysm; in the other it resembled idiopathic retroperitoneal fibrosis. The findings in the two subjects support the unifying concept of chronic periaortitis, to include conditions previously known as idiopathic retroperitoneal or mediastinal fibrosis, perianeurysmal retroperitoneal fibrosis and inflammatory aneurysms of the aorta. The observations are also compatible with the view that the disease is due to hypersensitivity to atheroma.

Aged↗
PubMed1999

[Cutaneous periarteritis nodosa: diagnostic and therapeutic aspects of 9 cases].

BACKGROUND: Cutaneous periarteritis nodosa (PAN) is distinguished from systemic PAN by the lack of visceral involvement. The aim of this study was to describe the clinical presentation, laboratory findings, clinical course, and treatment in cutaneous PAN. PATIENTS AND METHODS: We retrospectively reviewed the files of patients hospitalized for vasculitis in our Dermatology unit where approximately 20 cases of vasculitis are seen each year. Inclusion criteria were skin signs suggestive of PAN and a histological image of leukocytoclastic vasculitis of an arteriole. RESULTS: Nine cases of cutaneous PAN were treated in our unit between 1976 and 1997. Follow-up ranged from 32 months to 22 years. No cases of systemic PAN had been diagnosed during this period. These 9 cases of cutaneous PAN all had the same clinical presentation: nodules on the lower limbs in all cases associated with nodules on the upper limbs in half of the cases. Neuropathy was found in 3 of the 9 cases. No systemic involvement was observed. The most frequently used treatment protocol was general corticosteroid therapy (0.5 mg/kg/d prednisone or prednisolone). Immunosuppressive drugs, colchicine, dapsone, non-steroidal anti-inflammatory drugs and intravenous immunoglobulins were also used with efficacy. DISCUSSION: Cutaneous PAN is a particular form of vasculitis associating skin signs with locoregional neuromuscular involvement. The differential diagnosis with other types of vasculitis is sometimes a difficult task. The clinical course is the fundamental diagnostic clue in cutaneous PAN. A benign course and the absence of visceral involvement allow initiating a symptomatic treatment such as colchicine. The development of neuromuscular signs may warrant the use of general corticosteroid therapy.

Adrenal Cortex Hormones↗
PubMed2000

Juvenile relapsing periarteritis nodosa and streptococcal infection.

Classic polyarteritis nodosa is a multisystem inflammatory disease associated with necrotizing vasculitis of small and medium arteries. In most cases, the causes of polyarteritis nodosa remain unknown, but viruses (HBV, HCV, HIV) and microbes (especially streptococcus) have been considered as etiologic or contributing factors. A 13-year-old boy was admitted with fever, skin lesions, polyarthritis and muscle involvement. A muscle biopsy demonstrated a necrotizing vasculitis and antistreptolysin titre was tremendously increased. His condition improved following the administration of oral steroids but he experienced relapses 5 and 12 years later when penicillin withdrawal was attempted. The flares were accompanied by a major increase of antistreptolysin titre and response to corticosteroid was obtained. He is currently 38 years old and he remains well on prophylactic penicillin. Polyarteritis nodosa in children may occur after a streptococcal infection. It may be prudent to consider penicillin prophylaxis in patients with periarteritis nodosa when a streptococcal etiology is documented or highly suspected.

Adolescent↗
PubMed1976

[Early neuro-ophthalmological symptoms of periarteritis nodosa. A case report (author's transl)].

A report is given on a 47-year-old woman with periarteritis nodosa. A unilateral retinopathy with disseminated cotton-wool-spots surrounded by retinal haemorrhages and edema was observed. Fluoresceinangiography showed a multifocal constriction of the retinal capillaries. This lesion of the retinal vessels can be distinguished from those caused by hypertension. An acute immunological reaction has been suggested as the pathogenesis. Some authors have described this retinal lesion in collagen diseases as a "toxic" retinopathy. We prefer the term acute immunological microangiopathy (AIM).

Eye Manifestations↗
PubMedDate not supplied

HBV-related cutaneous periarteritis nodosa in a patient 16 years after renal transplantation: efficacy of lamivudine.

Cutaneous periarteritis nodosa (PAN) is a clinical feature characterized by chronic, benign course; its pathogenesis is unknown. In patients submitted to renal transplantation cutaneous PAN is a rare complication. We report a case of cutaneous PAN associated with the reappearance of hepatitis B antigen 16 years after kidney transplantation. A 44-year-old man underwent successful renal transplantation in June 1980. In December 1996 he presented multiple painful erythematous subcutaneous nodules on both legs. Skin lesion biopsy showed the presence of cutaneous PAN. Six months later laboratory data demonstrated the presence of HbsAg. HBeAg, HBcAb and detectable HBV-DNA serum by polymerase-chain-reaction (PCR) assay. Anti-HBs and anti-HBe proved negative. In July 1998 the laboratory tests showed an important increase of HBV-DNA (5.1 billion by Branched DNA), and so lamivudine (100 mg/day) was introduced. HBV-DNA became undetectable by PCR after 3 months of therapy. Seven months later a new skin biopsy was performed. The typical signs of PAN were no longer evident. As HBV infecion was demonstrated six months after the clinical appearance of the PAN, in a patient who was believed to be immune to the virus, it is possible that, in the early stages, the hepatitis B antigen title was methodologically indeterminable, but sufficient to form circulating immune complexes responsible for vasculitis primer. Lamivudine therapy resulted efficacious in favouring the regression of cutaneous PAN, but its long-term efficacy requires further evaluation as regards potential selection of drug resistant hepatitis B virus (HBV) mutants during treatment.

Adult↗
PubMed1992

[Multiple cholesterol embolism mimicking periarteritis nodosa].

Ten men aged 56 to 84 were hospitalized with a diagnosis of periarteritis nodosa, whereas they had multiple cholesterol embolism. The diagnosis was corrected post mortem in the first 3 patients and subsequently in live patients. The particularly misleading clinical manifestations were neurological (polyneuritis in 5 cases, mononeuritis in 1, central nervous system disorders in 3), pulmonary (alveolar haemorrhage in 2 cases, respiratory failure of unknown mechanism in 4) and pericardial (2 cases). Five patients had eosinophilia (more than 500 eosinophils/mm3). The elements that led to the correct diagnosis were the presence of vascular risk factors in all 10 patients (but hyperlipidaemia in only one), severe complications of the atheromatous disease in all cases, a precipitating or aggravating factor in 8 patients (anticoagulant therapy in 7, arteriography in 6) and the finding of purple or necrotic toes (6 cases). Histological (5 cases) and/or ophthalmological (2 cases) evidence was obtained in only 6 patients. Seven patients died 1 to 3 years after the onset of the disorders. Studies on low-density lipoprotein metabolism are in progress to determine the mechanism of clinical manifestations unexplainable by embolism.

Aged↗
PubMedDate not supplied

[Functional activity of polymorphonuclear leukocytes in systemic lupus erythematosus and periarteritis nodosa].

The functional activity of polymorphonuclear leukocytes (PMNL) was studied in the test of biochemical luminescence (BCL) in 43 patients including 31 patients with systemic lupus erythematosus (SLE) and 12 with nodular periarteritis (NP). Generation of the active forms of oxygen depended on the activity of the pathological process and the level of the circulating immune complexes (CIC). As established, under the conditions of hyperproductions IC PMNL in patients with SLE and NP preserve a rather high capacity to generate active forms of oxygen into intracellular space that allows one to speak about the role of oxygen radicals in the development of vascular lesions in patients with rheumatic diseases.

Adolescent↗
PubMed1991

[Periodic disease and periarteritis nodosa in the same patient: coincidence?].

Familial Mediterranean fever (FMF) chiefly affects patients of Arabic, Jewish, Armenian or Turkish origin and takes the form of recurrent episodes of peritonitis, arthritis or pleurisy. Periarteritis nodosa (PAN) is a vasculitis affecting elderly people and manifested by a general deterioration, unexplained fever and peripheral neuropathy or muscular weakness. We describe a patient presenting both diseases. Ours is the seventh reported case associating these two affections. This association was suspected by SACHS and co-workers who discovered an increased frequency of PAN in patients with FMF compared to the expected rate for the whole population (7). These observations warrant a search for PAN in young patients affected by FMF and showing signs of vasculitis.

Adult↗
PubMed1990

[Alithiasic cholecystitis and viral hepatitis B disclosing periarteritis nodosa].

The authors report the case of a patient with acute alithiasic cholecystitis associated with viral B hepatitis revealing periarteritis nodosa. Histopathological results showed signs of focal arteritis in the gallbladder and liver. Because of the negativity of the viral DNA in serum and the lack of histopathological necrosis in hepatic specimen, the patient was treated by steroid therapy only with a rapid regression of signs of vasculitis and the disappearance of the hepatitis markers.

Adult↗
PubMed1978

[State of the microcirculation in periarteritis nodosa based on morphological and biomicroscopic data].

The status of microcirculation in periarteritis nodosa was studied by impregnation method (serous membranes) and biomicroscopy (eyeball conjunctiva). Pathological and adaptative changes in terminal vessels were found. It is suggested that the involvement of microcirculation ways in this disease is systemic and that morphological changes in microvessels are comparable to biomicroscopic changes.

Adolescent↗
PubMed1989

[Periarteritis nodosa linked to the hepatitis B virus].

Periarteritis nodosa is a necrotizing vasculitis which, in more than one-third of the cases, is consecutive to an hepatitis B virus infection. This aetiological form is usually characterized by the absence of respiratory manifestations and by a greater frequency of hepatic cytolysis. In terms of survival, its prognosis is similar to that of other forms, but a persistent viral replication facilitates the subsequent development of chronic liver diseases and cirrhosis. Its modern treatment consists of a short course of corticosteroids combined with antiviral agent and plasma exchanges. Long-term corticosteroid therapy and immunodepressants are reserved for cases where the above-mentioned treatment has failed. This therapeutic approach results in a survival rate of 80 p. 100 at 4 years.

Female↗