Polyarteritis nodosa presenting with hemobilia and intestinal hemorrhage

Polyarteritis nodosa is a multisystem disease primarily involving the small and medium-sized vessels. Prognosis depends on the presence and severity of visceral involvement. Thrombosis and aneurysm formation commonly occur in kidney and gastrointestinal tract. Although hepatic involvement is also common, hepatic aneurysmal rupture with intrahepatic or perihepatic hematoma formation is infrequent. Hemobilia secondary to aneurysmal rupture is a very rare condition. We present a case of polyarteritis nodosa accompanied by hemobilia and intestinal hemorrhage secondary to hepatic and mesenteric aneurysmal rupture. Received 12 March 1996; Revision received 12 September 1996; Accepted 6 November 1996     

Outcome of polyarteritis nodosa in northern India

Over the last 10 years, 17 patients (13 males and 4 females) diagnosed as having classical polyarteritis nodosa (PAN) were treated and followed up in the rheumatology clinic of our institute. The median age and duration of symptoms at presentation were 29 years (range 13–59) and 9.5 months, respectively. Patients presented with the typical clinical picture of classical PAN. The diagnosis was established with the help of an aortogram (7/10), sural nerve biopsy (7/8), muscle biopsy (5/7) and renal biopsy (3/4). For reasons not known, none of the 17 patients was HBsAg positive. Patients were treated with a combination of oral prednisolone (1 mg/kg per day) for 6 weeks, which was slowly tapered off over 6 months, and monthly intravenous cyclophosphamide pulses (15 mg/kg) for the first 6 months, followed by 3-monthly pulses for a total of 2 years. Remission was achieved in 14 patients after a median of 5 months of treatment. Remission was stable for a median of 5 years of follow-up. Three patients did not respond well and died within 6 months of diagnosis. The causes of death in these were intracerebral haemorrhage in one patient and gastrointestinal bleeding in two patients. This experience is in accord with the reported literature that classical PAN is mostly a monophasic disease with either an excellent response to the appropriate immunosuppressive therapy and a long remission or a downhill course culminating in death. A chronic course is rare. Received: 8 October 1996 / Accepted: 4 April 1997     

Polyarteritis Nodosa in Arab Children in Saudi Arabia

At both King Khalid University Hospital and King Faisal Specialist Hospital in Riyadh, Saudi Arabia, between 1990 and 1997, we identified 11 children with polyarteritis nodosa (PAN). Nine of them satisfied the American College of Rheumatology 1990 criteria for the classification of PAN. All patients were followed for at least 12 months. The male-to-female ratio was 1.8:1. Their mean age at disease onset was 5.8 years (range 2–11.5 years) and the mean duration of the disease was 2.5 years (range 1–7 years). The clinical presentations in the children were musculoskeletal symptoms in all patients, mucocutaneous findings in 10 (91%), fever in eight (73%), gastrointestinal manifestations in six (55%) and hypertension in five (45%). The most frequent laboratory findings were leucocytosis, anaemia, thrombocytosis and a high erythrocyte sedimentation rate. Both histopathological and angiographic findings were an effective means of diagnosis. Nine children responded to glucocorticoids (GC) with cytotoxic drugs while the rest improved on GC alone. None of the children died from the disease. A high index of suspicion with early combination therapy will help in decreasing the morality in these cases. Received: 20 January 1998 / Accepted: 29 October 1998     

Fatal Polyarteritis Nodosa with Massive Mesenteric Necrosis in a Child

Polyarteritis nodosa (PAN) is a rare vasculitic syndrome in childhood. There are few reported cases of ischaemic necrosis of the intestine and even fewer survivors in adults. We report the case of a 10-year-old boy with PAN and an acute abdomen that required operative intervention. Evidence was found of mesenteric arteritis with large ischaemic segments resulting in infarction and perforation. Received: 23 March 1998 / Accepted: 14 August 1998     

Case reports: testicular pain as a manifestation of polyarteritis nodosa

Polyarteritis nodosa (PAN) is a necrotising vasculitis of medium-sized vessels of unknown origin. This type of vasculitis is usually systemic, but restriction to a single organ, for example the testis, the appendix or the gall bladder, can occur. Testicular pain or tenderness are frequent clinical features. In this report, we present three cases of PAN. In every patient, testicular pain was the main symptom or first sign of systemic disease. We state that a thorough history taking, clinical examination and biochemical analyses are obligatory in patients presenting with acute or chronic scrotal pain. Polyarteritis nodosa should always be taken into account, and a search for systemic spread is mandatory. We emphasize that before initiation of systemic therapy with corticosteroids and/or cyclophosphamide, a Five Factor Score should be obtained, which also gives crucial prognostic information.     

Polyarteritis Nodosa: State of the art

Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis that preferentially affects medium-sized vessels. The idiopathic form has become rare. Its treatment relies on corticosteroid therapy and is combined with cyclophosphamide infusions for severe forms. Secondary PANs were mainly associated with hepatitis B virus infection; they were treated with plasma exchange and antivirals in combination with short-term corticosteroid therapy. Other secondary forms of PAN are now becoming more common, such as those due to blood disorders. More recently, a monogenic form linked to adenosine deaminase-2 mutations has been identified. It requires treatment with TNF inhibitors to decrease the occurrence of ischemic central nervous system complications, which make it serious. Once remission is obtained, relapses are typically rare during PAN and affect 28% of idiopathic PANs, within an average of 26 months from the diagnosis. The prognosis has improved considerably, with 5- and 10-year survival rates of 83% and 74%.     

Ultrastructural changes of skeletal muscles in polyarteritis nodosa and in arteritis associated with rheumatoid arthritis

Summary Muscle biopsies from two cases of polyarteritis nodosa (PN) and one of arteritis in association with rheumatoid arthritis (RA) were examined by electron microscopy.The histological changes were similar in all three cases. The endothelial cells of the small blood vessels were often hypertrophied. Inflammatory reaction was present mainly in the vicinity of the blood vessels. Individual muscle fibres showed mostly nonspecific degenerative changes. In a case of PN, however, annulate lamellae were present in a small number of the muscle fibres. The annulate lamellae have been reported, to our knowledge, in the human skeletal muscles only in a few cases of polymyositis. In addition, two cases, one of PN and one of arteritis with RA, showed fine filamentous inclusions in the muscle fibres. Changes were also noted in the motor end-plate.A sural nerve biopsy in a case of arteritis with RA showed changes both in axons and myelin sheaths, in addition to the changes in the blood vessels similar to those in the muscle.     

Systemic necrotizing vasculitides in Turkey: a comparative analysis of 40 consecutive patients

Objective: The aim of this study was to analyze and compare the demographic and clinical features and prognosis of patients with different systemic necrotizing vasculitides (SNV) in Turkey. Patients and methods: Twenty-three patients with Wegeners granulomatosis (WG), 15 with polyarteritis nodosa (PAN), and two with Churg-Strauss syndrome were included in the study. The clinical and laboratory features of patients with WG and PAN were compared, and survival analysis was performed for the WG patients. Results: Twenty-one patients with WG had systemic disease involving kidneys, and two had localized disease. Fifteen patients were placed in the PAN group, 12 of whom were classified as having classic PAN and three with microscopic polyangiitis. Median follow-up time was 37 months (range 1–81) for WG patients and 41 months (range 5–132) for the PAN group. Upper respiratory tract, pulmonary, and renal involvement were significantly more frequent in the WG group than in PAN. Peripheral nervous system involvement was more frequent in the PAN group. In WG, survival was calculated as 59% at 35 months. High initial vasculitis damage index scores were found to be predictive for mortality. Conclusion: This study revealed that the most frequent type of SNV was WG in a tertiary rheumatology setting in Turkey. There was initial organ damage in most of the patients, frequently caused by severe renal involvement. In contrast to other published series, overt cardiovascular and gastrointestinal involvement were not observed in our patients with SNV.     

Vasculitis in children

Vasculitis in children is uncommon and hardly any information is available from India. We, at PGIMER, Chandigarh, have diagnosed and followed many children with vasculitis of different types though not all, which occur in children. In this article, we have given an overview of the vasculitides that we have encountered alongwith a review of relevant literature. We have described 8 children with classical PAN and have highlighted a higher frequency of CNS involvement in our patients. Amongst the 10 BCPAN children, as many as 8 had peripheral gangrene which resulted in autoamputation in 7. Gangrene of such severity has not been previously reported in this condition. We have also included 30 children with HSP. Gastrointestinal involvement was noted in 86.7% of children and in one of these, it was severe encough to result in hypovolemic shock. Such severe bleeding is very rare. Two of our patients with HSP came late to us after having been operated for an acute abdomen elsewhere. Although renal involvement was seen less frequently than reported in the literature, the severity of involvement was greater (nephrotic range proteinuria in 62% and azotemia in 50%). We have only limited experience of Kawasaki Disease but it appears that children with this disorder are probably not being diagnosed in the acute stage in our country.     

Isolated Central Nervous System Arteriopathy with Multiple Aneurysms in an Adolescent A Case Report

A 17 year old male suffered recurrent subarachnoid hemorrhages due to multiple aneurysms in the distal branches of the cerebral arteries. Autopsy revealed arteriopathy as well as the aneurysms. The arteriopathy was widespread, affecting the distal branches of small and medium-sized muscular cerebral arteries, as well as the anterior and posterior spinal arteries, and vasocorona. The arteriopathy was characterized by prominent intimal thickening, discontinuity or absence of the internal elastic lamina, and thinning and/or disappearance of smooth muscle in the tunica media with fibroplasia. Slight intimal thickening was also observed in the arteries of the circle of Willis and its major branches, as well as in the basilar artery. However, the arterioles, venules and veins showed no remarkable features. The arterial lesions were found only in the central nervous system. The multiple aneurysms in the distal branches of the cerebral arteries, which had produced the main symptoms and clinical signs, were due to the arteriopathy.