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Are infantile periarteritis nodosa with coronary artery involvement and fatal mucocutaneous lymph node syndrome the same? Comparison of 20 patients from North America with patients from Hawaii and Japan.

We reviewed available clinical and pathologic autopsy material from 20 patients with infantile periarteritis nodosa with coronary artery involvement (IPN) from the continental United States, two Hawaiian patients with fatal mucocutaneous lymph node syndrome (MCLS; Kawasaki disease), and three patients with classical periarteritis nodosa (CPN). Comparison of the findings in patients with IPN and in patients with MCLS from Hawaii to material from patients with fatal MCLS from Japan showed no definite clinical reason to distinguish IPN from MCLS; neither gross nor microscopic features of the vascular lesions nor their pattern of distribution appears to warrant separation of IPN from fatal MCLS. CPN differs, both clinically and pathologically, from IPN/MCLS, and may well have a different etiology.

Adult↗

Third nerve palsy and internuclear ophthalmoplegia in periarteritis nodosa.

There is a large range of neurologic complications in periarteritis nodosa, but oculomotor motility disorders are rarely seen. Two patients presented with third nerve palsy and one presented with internuclear ophthalmoplegia due to periarteritis nodosa vasculitis. We hypothesize that the third nerve palsy and internuclear ophthalmoplegia was caused by a vascular infarction in the brain stem.

Adolescent↗

Immune complexes of hepatitis B surface antigen in the pathogenesis of periarteritis nodosa. A study of seven necropsy cases.

In 7 unselected necropsy cases of clinically diagnosed periarteritis nodosa, the detection of hepatitis B surface antigen (HBsAg) and hepatitis B core antigen (HBcAg) in the cytoplasm and nuclei of hepatocytes indicated an ongoing infection with hepititis B virus (HBV). In all these cases histologic changes found in the liver varied from "minimal" to chronic aggressive hepatitis. In all the cases, deposits of HBsAg, immunoglobulins, beta1C-globulin and C1q were detected in vascular lesions. That these deposits could represent HBsAg-anti-HBs immune complexes was supported by demonstrating their strong binding of guinea pig complement and by the successful elution of all HBsAg and part of the immunoglobulin from these deposits by treatment with buffers known to dissociate antigen-antibody bonds but not with phosphate-buffered saline, pH 7.6 (PBS). Glomerulonephritis associated with these immune complexes was found in 6 cases. The presence of larger masses of HBsAg immune complexes, chiefly in recent insudative and fibrinoid vascular lesions, their lesser amounts in lesions undergoing involution, and their absence from healed lesions strongly suggest that these complexes play a primary role in the pathogenesis of acute vascular damage in periarteritis nodosa.

Adult↗

[Periosteal reaction in periarteritis nodosa in children].

Bone involvement has exceptionally been reported in children with periarteritis nodosa. A 5-year old girl was admitted to hospital for fever and arthralgias. Three months later, myalgias and painful subcutaneous nodules developed on the legs and ankles. Skin biopsy yielded a diagnosis of periarteritis nodosa. X-ray films revealed a bilateral periosteal reaction with images of laminae in the tibia and fibula. After a 4-year remission under corticosteroid therapy, the periosteal reaction persisted.

Ankle↗

Atraumatic rupture of the spleen in periarteritis nodosa.

A case of atraumatic rupture of the spleen is described. The underlying disease was found to be periarteritis nodosa. Involvement of the spleen is common in this disease, but to our knowledge atraumatic rupture of this organ in a patient with histologic verified periarteritis nodosa has not previously been reported. In the present case the splenic rupture was the incident which led to the diagnosis. A possible mechanism of the rupture is proposed.

Aneurysm↗

[Periarteritis nodosa in children. Diagnostic difficulties in 2 cases].

Periarteritis nodosa is a rare multisystemic disease whose diagnosis is difficult: diagnostic approach by biopsies in various involved organs is of poor efficacy; abdominal selective angiography yields a precise diagnosis in 50 to 60 of the studied cases as far as the celiac, mesenteric and renal circulations are investigated selectively. Digitalized intravenous angiography (DIVA) carried out in two patients of 9 and 13 years of age allowed to demonstrate aneurysm formations in hepatic, renal and cerebral circulations. Its true efficacy in the diagnosis of periarteritis nodosa of children remains to be determined.

Adolescent↗

[Temporal arteritis: a syndrome. From Horton's disease to periarteritis nodosa].

Four cases of histologically proven temporal arteritis presenting with atypical clinical, anatomical or evolutive features and raising nosological problems concerning the type of vasculitis involved are presented. In patients with temporal arteritis, some elements are suggestive of periarteritis nodosa. They include peripheral neurological lesions, renal or pleuro-pulmonary lesions and the histological appearance of the temporal artery. Diagnosing periarteritis nodosa in a case of temporal arteritis leads to a special therapeutic strategy.

Adrenal Cortex Hormones↗

[Hyperacute periarteritis nodosa complicating Basedow's disease].

The symptoms and the evolution of necrotizing vasculitis vary greatly. The authors illustrate the case of an 18 year old patient with a history of frequent allergic manifestations (urticaria and others) who was found to have, in septembre 1975, a typical case of Grave's disease. During the following 2 months she was treated with an iodide derivative. One year later the clinical signs increased to the point where a treatment associating lugol, carbamizole and propanolol was deemed necessary. Less than 2 months later there developed a polyvisceral disease with oscillating fever, polyarthralgia and necrotizing vasculitis. The plurivisceral nature of the illness was further illustrated by the presence of a hyperreflexia, a glomerulopathy and retinal exsudats. A muscle biopsy revealed the necrotizing vasculitis with granulomas typical of periarteritis nodosa. Cardiac, neurologic and renal complications were responsable for a rapid down-hill course and despite corticoïdes and immunosuppresive drugs, the patient died after a few weeks of treatment. That periarteritis nodosa should complicate the evolution of Grave's disease suggests a connection between the two, very probably immunologic in nature. The role of drugs capable of inducing vasculitis must be explored, especially the iodide derivatives, the antithyroïd medications or their association. Such cases, even though they may be rare, should incite special care in the prescription of antithyroïd drugs in the allergic patient.

Acute Disease↗

[Chronic lymphocytic leukaemia with cutaneous lesions and deafness following periarteritis nodosa (author's transl)].

A case is reported of a 49 years-old man who presented with cutaneous and muscular manifestations revealing periarteritis nodosa. Two years after detection of this disease occured a preleukemic condition and four years later a chronic lymphocytic leukaemia is discovered. In clinical course appeared a tuberous infiltration of the face and a sudden bilateral deafness. Regression of these two abnormalities is obtained after administration of prednisolone. The patient died of generalized infectious process postoperatively. From these clinical and pathological findings relations between periarteritis and CLL, occurence of deafness and cutaneous lesions are discussed. The relationship between the development of CLL and the immunosuppressive treatment is considered.

Deafness↗

Periarteritis nodosa in pregnancy: report of a case and review of the literature.

This article presents a case report and review of the literature on periarteritis nodosa. The purpose is to alert physicians that there are causes of hypertension in pregnancy other than preeclampsia and chronic essential hypertension. Periarteritis nodosa is rare in pregnancy and is usually fatal in the gravid patient.

Adult↗

[Multiple affections of the cranial nerves as a pathognomonic sign of periarteritis nodosa. Relationship with Cogna's syndrome (author's transl)].

A 43-year-old woman was found to have multiple cranial neuritis affections, present as an isolated disorder over a long period, and characterized by bilateral deafness and associated lesions of the VII and V cranial nerves. On investigation, visual disturbances were discovered which were of a transitory nature and made up a total clinical picture suggesting Cogan's syndrome. Neuropathological examination revealed the presence of typical periarteritis nodosa lesions in the cranial nerves. The authors suggest, therefore, that certain cases described as Cogan's syndrome are in fact particular forms of periarteritis nodosa.

Adult↗

[Periarteritis nodosa associated with hepatitis B virus. 42 cases (author's transl)].

HBs antigen was detected by immunodiffusion or radioimmune assay in the blood of 19 out of 42 patients with periarteritis nodosa examined between 1970 and 1978. Histological or biochemical signs of hepatitis were present in 6% of HBs--positive patients and 1% of HBs-negative patients. The incidence of clinical symptoms was similar in both groups, except for multiple neuritis, which was much more frequent in HBs-positive patients (89%) than in HBs-negative patients (52%; p less than 0.001), suggesting a close relationship between neuritis and HBs and HBs antigen. There was a negative correlation between myalgias and skin lesions and the presence of the antigen. The prognosis of periarteritis nodosa was similar in both groups. The course of the disease seemed to be more prolonged in HBs-positive patients, but there was no significant difference in mortality rate.

Adult↗

Periarteritis nodosa and pregnancy.

Pregnancy in patients with periarteritis resulted in maternal death in 7 of the 8 cases that have been reported. In the present case, periarteritis was in remission throughout the pregnancy; the patient was thus the second known maternal survivor. The infant also did well. It is suggested that pregnancy probably does not have as direct an effect on the course of the disorder as appears from the outcome of the previous cases. Extreme caution must prevail, especially as diagnosis is often difficult and experience so limited.

Adult↗

[Periarteritis nodosa and viral hepatitis B, conjugal forms. Apropos of 4 cases].

Four cases of periarteritis nodosa (PAN) associated with HB virus are reported in three couples. The spouse also had periarteritis nodosa (cases 1 and 2) or had hepatitis B (spouses of cases 3 and 4). These observations underscore the part played by conjugal transmission of HB virus and the risk of secondary PAN. Such observations are uncommon as the four reported cases occurred among a group of 95 PAN patients. Nevertheless, they are additional evidence in support of the role of HB virus which is associated with PAN in 45% of cases in this series.

Adult↗

[Association of Horton's disease and periarteritis nodosa. Apropos of 3 cases].

Three cases of giant cell arteritis associated with periarteritis nodosa are reported. A review of the literature showed 16 similar reports. Giant cell arteritis features in temporal arteries were associated with periarteritis features in visceral or peripheral arteries in 5 cases only. Association of these two conditions seems to be uncommon and may be coincidental but does not justify including giant cell arteritis within the group of necrotizing angiitis. Awareness of this association nevertheless has practical implications as these cases should be treated as PAN patients.

Aged↗

[Clinical and morphological manifestations of the pathomorphosis of periarteritis nodosa].

Clinical, immunological, morphological and immunomorphological data are provided showing the presence of induced (therapeutic) pathomorphosis in Kussmaul-Meier's disease. The conclusion is drawn that at present periarteritis nodosa is characterized by a chronic course with a domination of productive (immunocellular) vasculitis over necrotic (immunocomplex) vasculitis. It is shown that modern polychemotherapy of Kussmaul-Meier's disease favours mainly the correction of humoral immunity alterations while the pathology of cell immunity is retained. It is noted that long interruptions in therapy result in a reversion of the clinical-anatomical manifestations of periarteritis nodosa.

Antibody Formation↗

[Fibrous periarteritis of the retroperitoneal space and mediastinum as a cause of extravasal arterial compression].

Under analysis there were 14 patients operated upon for fibrous periarteritis. In 10 patients there was a retroperitoneal localization of the pathological process, and 4 patients had the mediastinal localization. The both localizations have similar features of etiology, pathogenesis, clinical course and lead to the formation of a stenosing scarry housing around the arteries with an issue into the occlusion. The operation volume for the retroperitoneal and mediastinal localization of the fibrous periarteritis depends on the stage of the disease: decompression and arteriolysis at first stages for stenosis and reconstructive operations for occlusions of the arteries.

Adult↗