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Background: Childhood Polyarteritis nodosa (PAN) is a systemic vasculitis with necrotizing inflammation of medium- and small-sized arteries. Disease evolution may be severe and refractory to standard treatment including prednisone, azathioprine and cyclophosphamide., Case Presentation: We present the case of a young girl with severe PAN resulting in progressive ischemia and necrosis of fingers and toes. Biological work-up revealed increased acute phase reactants and interleukin-6 levels. She was only partially controlled despite high-dose corticosteroids and cyclophosphamide infusions, and eventually achieved rapid improvement and sustained remission on tocilizumab. Further, we review the current evidence of the interleukin-6-inhibitor tocilizumab for the treatment of PAN., Conclusion: Tocilizumab may be an efficient therapeutic option in a subset of treatment-refractory children with PAN., (© 2021. The Author(s).)

Childhood-onset polyarteritis nodosa (PAN) is a rare and systemic necrotising vasculitis in children affecting small- to medium-sized arteries. To date, there have been only a few reports because of its rarity. Thus, we aimed to investigate the clinical manifestations, laboratory findings, treatment, and long-term outcomes in patients with childhood-onset PAN and to evaluate the usefulness of the paediatric vasculitis activity score (PVAS). We retrospectively analysed the data of nine patients with childhood-onset PAN from March 2003 to February 2020. The median ages at symptom onset, diagnosis, and follow-up duration were 7.6 (3-17.5), 7.7 (3.5-17.6), and 7.0 (1.6-16.3) years, respectively. All patients had constitutional symptoms and skin manifestations, while five exhibited Raynaud's phenomenon. Organ involvement was observed in one patient. The median PVAS at diagnosis was 7 (range: 2-32). Prednisolone was initially used for induction in all patients, and other drugs were added in cases refractory to prednisolone. All patients survived, but three patients with high PVAS at diagnosis experienced irreversible sequelae, including intracranial haemorrhage and digital amputation. In conclusion, early diagnosis and treatment may minimise sequelae in patients with childhood-onset PAN. This study suggests that high PVAS score at diagnosis may be associated with poor prognosis.

Arthralgia is a well-known complication of cutaneous polyarteritis nodosa (CPN). However, few cases of destructive arthritis with CPN resulting in chronic pain and disability have been documented. Here we report a case of CPN associated with destructive arthritis of the right foot, accompanied by seronegative arthritis in the right ankle, for which orthopaedic surgery was necessary. X-ray and computed tomography revealed progressive joint destruction in the right talonavicular joint, and magnetic resonance imaging demonstrated severe synovitis of the right talocrural joint. When the talonavicular joint was opened, we observed marked synovitis and a defect in the cartilage that had expanded within the joint. Although mild cartilage degeneration was observed in the talocrural joint, arthroscopic observation revealed no massive defect on the articulated surface. Arthrodesis and arthroscopic synovectomy yielded a good midterm clinical outcome, indicating that surgical intervention was useful for the treatment for destructive arthritis with CPN. Rheumatologists and orthopaedic surgeons should be aware of potential complications such as destructive arthritis when treating patients with CPN.

Background: Polyarteritis nodosa is a small vessel to medium vessel vasculitis that frequently presents with multi-organ involvement, but can sometimes be limited to single organs such as the testes. Patients often require treatment with glucocorticoids, plus or minus additional immunosuppressive therapy depending on the severity of the disease. We describe a rare case of polyarteritis nodosa involving the right testis and urinary bladder without other systemic features of vasculitis., Case Presentation: A previously healthy 54-year-old First Nations Canadian man presented with intermittent gross hematuria. He underwent surgical excision of his right testis for cryptorchidism and a transurethral resection of a bladder mass. Histology showed an active medium vessel vasculitis in both organs. On extensive clinical, laboratory, and radiographic review, he had no systemic features of vasculitis. On 2-year follow-up, he has not required any systemic therapy and has not developed further symptoms., Conclusion: Single organ polyarteritis nodosa can sometimes be managed with surgical excision of the involved organ alone. Although our patient had two organs involved, we extrapolated the results of our literature search to guide his care. This case highlights the potential for surgical excision to cure polyarteritis nodosa despite the involvement of two organs in the absence of symptoms and signs of systemic vasculitis.

A middle-aged man presented with a fever, arthralgia, gastrointestinal symptoms, headache, and rash. After two weeks, the patient suddenly complained of severe abdominal pain, and computed tomography revealed aneurysms in the hepatic and splenic arteries, which increased in size progressively. Given the elevated levels of inflammatory markers and orchitis, polyarteritis nodosa (PN) was initially suspected. Catheter embolization for the ruptured hepatic aneurysm and splenectomy for the large splenic ones were performed, and the pathological finding was consistent with segmental arterial mediolysis (SAM). Changes in inflammatory marker levels and aneurysmal size are also informative to differentiate SAM from PN.

Extraintestinal infections due to Clostridium difficile are uncommon. When such infections occur, extraintestinal C. difficile isolates are usually identical to fecal isolates. We present a rare case of a large postoperative abscess caused by C. difficile infection, in which different C. difficile strains were isolated from the abscess and from feces of the patient. An 82-year-old woman with cutaneous polyarteritis nodosa developed pain, skin ulcers, and extensive necrosis of the right leg. Above-knee amputation was performed without stopping antiplatelet therapy, leading to postoperative hematoma. Six weeks after surgery, a large femoral abscess was detected and C. difficile was isolated. Repeat amputation of the thigh was required to remove the abscess. C. difficile was also cultured from feces despite the lack of intestinal symptoms. However, genetic analysis confirmed that the C. difficile isolates from the abscess and feces were different strains. Thus, C. difficile can cause postoperative infection of a hematoma and the extraintestinal and fecal C. difficile isolates are not necessarily identical in the same patient., (Copyright © 2017 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Polyarteritis nodosum belongs to a group of inflammatory disorders characterized by necrotizing vasculitis of small and medium-sized blood vessels. To date, there are 14 publications that document involvement of the breast. Our publication is the second study documenting polyarteritis nodosum of the breast in a patient who had previously had augmentation mammoplasty. Level of Evidence V This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .

This report documents a case of asynchronous bilateral testicular infarction. The patient was a 42- year-old man who presented with left testicular pain and swelling. He had a past history of right idiopathic testicular infarction and underwent a right orchiectomy 6 years ago. He also had received treatment for 5 years for suspected polyarteritis nodosa (PAN). The left scrotal pain persisted for a week and left orchiectomy was performed. Pathological evaluations demonstrated a benign testis with testicular hemorrhage and chronic vasculopathy. There was no fibrinoid necrosis of medium-size vessel walls which characterizes PAN. In this report, we review the pathogenesis, risk of contralateral testicular infarction, and management of testicular infarction.

Intracranial aneurysms in polyarteritis nodosa (PAN) are exceedingly rare lesions with unpredictable behavior that pose real challenges to microsurgical and endovascular interventions owing to their inflammatory nature. We introduce a safe and effective alternative for treating these aneurysms using Pipeline embolization devices (PEDs). A 20-year-old man presented with diplopia, headaches, chronic abdominal pain, and weight loss. Diagnostic evaluations confirmed PAN, including bilateral giant cavernous carotid aneurysms. Cyclophosphamide and steroids achieved significant and sustained clinical improvement, with a decision to follow the aneurysms serially. Seven years later the left unruptured aneurysm enlarged, causing a sudden severe headache and a cavernous sinus syndrome. Treatment of the symptomatic aneurysm was pursued using flow diversion (PED) and the internal carotid artery was successfully reconstructed with a total of four overlapping PEDs. At 6 months follow-up, complete exclusion of the aneurysm was demonstrated, with symptomatic recovery. This is the first description of using a flow-diverting technique in an inflammatory vasculitis. In this case, PEDs not only attained a definitive closure of the aneurysm but also reconstructed the damaged and fragile arterial segment affected with vasculitis., (2015 BMJ Publishing Group Ltd.)

Cutaneous polyarteritis nodosa (cutaneous PAN) is a form of necrotizing vasculitis of small- and medium-sized arteries, primarily involving the skin. In juvenile cases, cutaneous PAN is known to be frequently associated with Group A β-hemolytic Streptococcus (GAS) infections. We herein describe the first reported juvenile case of GAS-associated recurrent cutaneous PAN successfully improved with tonsillectomy. To avoid the use of steroids and immunosuppressive drugs, especially in juvenile cases, tonsillectomy is a possible treatment for GAS-associated recurrent cutaneous PAN.

Intracranial hemorrhage has been rarely reported during the course of polyarteritis nodosa. We describe a 6-year-old boy who presented with fever, altered sensorium, skin rash, hypertension, and catastrophic intracranial hemorrhage. After surgical evacuation of the intracranial hematoma, he underwent a computerized tomography angiogram that showed narrowing of the right anterior cerebral artery. Skin biopsy showed small vessel vasculitis. Nerve conduction studies were suggestive of mononeuritis multiplex. He was diagnosed as polyarteritis nodosa and managed with immunosuppressants, to which he responded favorably. The most interesting aspect of the child's presentation was the catastrophic onset of altered sensorium with raised intracranial pressure, which was a diagnostic challenge. The mechanisms of intracranial hemorrhage in polyarteritis nodosa and a review of the literature are discussed. The authors emphasize that it is important to recognize intracranial hemorrhage as a life-threatening complication in children with polyarteritis nodosa to institute timely therapy., (© The Author(s) 2013.)

Drug-eluting stents are largely used in coronary arteries and more recently in tibial arteries owing to their potentially better outcomes compared with bare metal stents. A patient with polyarteritis nodosa and critical limb ischemia and a dorsal foot ulcer was previously unsuccessfully treated with multiple angioplasties and subsequently underwent implantation of a drug-eluting stent in the pedal artery. At 6 months, stent patency on color Doppler ultrasound and complete healing of the foot ulcer were observed., (Copyright © 2013 SIR. Published by Elsevier Inc. All rights reserved.)

The correct identification of vessel type is crucial in distinguishing cutaneous polyarteritis nodosa from superficial thrombophlebitis. As the treatment and prognosis of these conditions are very different, correct diagnosis is critical, but they have overlapping clinical and histopathologic features, which can sometimes make the distinction difficult. Features commonly used to distinguish an artery from vein include vessel shape and diameter, the presence or absence of an internal elastic lamina, smooth muscle pattern, and the presence or absence of valves. Recently, it has been proposed that the amount and distribution of elastic fibers in the medial muscular layer are the most reliable features to make this distinction. The first part of this study used prosector-identified vessels to determine which of these features is most sensitive and specific for identifying an artery and vein. A total of 19 arteries and 16 veins were dissected from autopsy and amputation specimens. For each specimen, the smooth muscle pattern, elastic fiber pattern, the presence of valves, and the presence or absence of an internal elastic membrane were determined. The quantity of elastic fibers in the muscular wall of each sample was also determined. Although the elastic fiber pattern was the most specific feature in identifying a vein, it suffered from low sensitivity (43.8%). The smooth muscle pattern had the highest combined sensitivity and specificity. In the second part of this study, the histologic features listed above were examined in previously diagnosed cases of superficial thrombophlebitis and arteritis. When inflammation is present within and around the wall of the vessel, all of the studied histologic features become less reliable, and the interobserver reliability of distinguishing arteritis from thrombophlebitis was low. Our findings suggest that no single histopathologic feature is completely reliable and combining the histopathologic features with clinicopathologic correlation is essential for correct vessel identification.

Polyarteritis nodosa (PAN) is a rare multisystem disease characterized by systemic necrotizing arteritis of small and medium size arteries. The skin, joints, kidneys, gastrointestinal tract and peripheral nerves are most commonly involved. Although aneurysms are commonly seen in the visceral vessels, intracranial aneurysms are rare with 15 reported cases. The intracranial aneurysms are usually multiple and located in supra- as well as infra-tentorial compartments. Most of the cases presented with subarachnoid or parenchymal hemorrhage. The aneurysms were usually small, although large cavernous aneurysms were reported in one case. Treatment guidelines are not clear regarding the management of these cases. Most patients were treated conservatively by medical management with surgical excision performed in only two cases and coiling done in one patient with cavernous aneurysms. Repeat hemorrhages or re-bleed in spite of medical treatment have also been reported. We describe the case of a 22-year-old woman, a known case of PAN who presented with subarachnoid hemorrhage. Cerebral angiogram showed a ruptured right middle cerebral artery bifurcation aneurysm along with unruptured left middle cerebral, right posterior communicating and left posterior inferior cerebellar artery aneurysms. Her previous abdominal angiogram had revealed multiple aneurysms in visceral arteries. Successful coil embolization of the ruptured right MCA bifurcation aneurysm was performed with preservation of the parent vessel. The patient made a complete recovery and was placed on medical treatment for PAN. Follow-up MR angiography at three months revealed stable occlusion of the embolized aneurysm with no change in the unruptured aneurysms. Although rare, PAN can be associated with intracranial aneurysms which can cause subarachnoid or parenchymal hemorrhage. Selected cases can be treated safely by coil embolization.

The differential diagnosis of acute abdomen is typically extremely broad in range, with vasculitis posing a rare but potentially life-threatening cause of acute abdomen. Here, we report a case of acute abdomen with bowel wall thickening limited to jejunum, accompanied by unexplained renal dysfunction. Later, the patient was diagnosed as having polyarteritis nodosa based on surgically resected jejunal necrosis. Despite aggressive treatment, including the use of steroid pulse therapy and continuous hemodiafiltration, the patient died. Although polyarteritis nodosa is extremely rare in patients with acute abdomen, acute abdomen is relatively common manifestation of that. And it is reported that involvement of small intestine suggests poorer prognosis. Our case highlights the importance of vasculitis as a differential diagnosis of patients with atypical acute abdomen. In this report, we not only review possible clues that might have led to an earlier diagnosis in this case, but also attempt to draw some lessons for treating similar cases in the future.

Coronary involvement of polyarteritis nodosa (PAN) has been mostly identified in postmortem studies. We report a case with inferior myocardial infarction (MI) because of coronary dissection and thrombosis in PAN. A 23-year-old woman with chest pain was admitted to the emergency department. The admission ECG was suggestive of inferior MI with no right ventricular infarction. Coronary angiography revealed an occluded right coronary artery because of coronary dissection and concomitant thrombosis. Coronary angioplasty and stent implantation were performed successfully without complications. This report emphasizes the importance of PAN in spontaneous coronary dissection and thrombosis even in young patients.

Polyarteritis nodosa (PAN) is a rare, systemic necrotizing vasculitis of small and medium size arteries that leads to aneurysms in various organs. Aneurysms associated with PAN are common in visceral arteries, however, intracranial aneurysms are rare, especially in childhood. A pediatric patient with PAN developed serial hemorrhagic strokes from a ruptured superior cerebellar artery aneurysm (subarachnoid hemorrhage) and a de novo aneurysm of the frontoorbital artery (intracerebral hemorrhage) after 9 months. Patients with PAN who present with intracranial aneurysms are candidates for intervention even if the aneurysm is unruptured and still small, and close observation is needed to detect de novo aneurysms in patients with chronic history of PAN.

Systemic polyarteritis nodosa (PAN) is a rare disease in childhood affecting small and midsized arteries. The typical presentation in children is of isolated 1- or 2-organ involvement, and the diagnosis is often based on the histopathology. We report a case of pediatric PAN whose presenting symptom was jejunal obstruction owing to ischemic necrosis of the small bowel. The postoperative recovery was uneventful, and the patient responded well to steroid therapy. There are only a few cases of PAN with intestinal involvement described in the literature, some of them fatal. The diagnosis of PAN should be considered in children with acute intestinal obstruction with no obvious etiology, as early recognition and treatment may reduce its high mortality., (Copyright © 2011 Elsevier Inc. All rights reserved.)

A 50-year-old man visited our hospital with a complaint of painful masses in bilateral scrotums for 2 months. Ultra sonography shows isoechoic mass in both scrotum. Antibiotic was treated for 2 weeks but had no effects on scrotal swelling and testicular neoplasm cannot ruled out, so we performed bilateral high orchiectomy. Pathological examination shows necrotizing vasculaitis surrounded by glanulomas and pathologically diagnosed as Polyarteritis Nodosa in bilateral epididymis. After orchiectomy, blood examination of immunity was revealed no specific findings, that made us diagnosed as isolated PN. 2 years after operation, he had no reccurence.

A 46-year-old man with polyarteritis nodosa and multiple myocardial infarctions treated with multiple percutaneous coronary interventions presented again with atypical angina. Coronary angiography revealed triple-vessel coronary artery disease. This patient underwent four-vessel coronary artery bypass graft and recovered uneventfully. A review of the literature and discussion of the surgical management of this patient is presented., (2010 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Autoimmune lymphoproliferative syndrome (ALPS) is an uncommon disorder of Fas-mediated apoptosis that results in impaired lymphocyte death and, therefore, disturbed immune homeostasis. Besides presentation with lymphadenopathy and splenomegaly, patients with ALPS have a high incidence of autoimmune phenomena. To our knowledge, this is the first description of polyarteritis nodosa that includes numerous arterial aneurysms in a child with ALPS. Active vasculitis resolved after allogeneic hematopoietic stem cell transplantation. This report of polyarteritis nodosa associated with human ALPS supports previous findings in Fas-deficient mouse models that frequently develop vasculitic manifestations and suggests that apoptotic defects of lymphocytes may play a role in the pathophysiology of systemic vasculitis. Thus, patients with ALPS might be more susceptible to autoimmune vessel inflammation. This case furthermore emphasizes that even rare autoimmune manifestations should be considered and investigated in patients with immunodeficiencies, because that might help in planning treatment strategies for these patients.

Polyarteritis nodosa is a form of vasculitis that affects several organs. Gastrointestinal involvement is frequent, but cases in which the gastrointestinal tract is the only site of disease are rare. In this paper we report a case of a 70-year-old patient with polyarteritis nodosa restricted to the large intestine, who underwent a total colectomy. The patient had no other signs of chronic vasculitis and for this reason surgical treatment resolved the clinical symptoms.

Introduction: The recurrence of primary disease in transplantation is a well-known problem. We report our single-center experience to assess the frequency of the recurrence of primary glomerulonephritis in children after renal transplantation., Patients and Methods: Medical reports of 14 children with primary glomerular disease were evaluated. Among the 14 grafts were 10 from living related and four from cadaveric donors. Ten were diagnosed as focal segmental glomerulosclerosis (FSGS), two membranoproliferative glomerulonephritis (MPGN), and two polyarteritis nodosa (PAN). The original diagnosis was biopsy-proven in every case. All patients were treated with calcineurin-based immunosuppressive therapy., Results: The mean age was 15.5 +/- 5.4 years. The median transplantation duration was 47 months; however, one of the FSGS patient had hyperacute rejection. Five years later she received a second graft with a serum creatinine of 0.7 mg/dL at 7 years after transplantation. Posttransplant recurrence of FSGS was confirmed in two patients (20%), who were treated with plasmapheresis with no improvement of proteinuria, two FSGS patients had thromboses after transplantation. One had a cardiac thrombosis with heterozygote MTHFR mutation and one, a renal artery thrombosis and loss of graft with prothrombin 20210A mutation. They all have functioning grafts except these two. We did not observe recurrence of PAN or MPGN in patients., Conclusion: Although the number of patients is quite small, our recurrence rate was compatible with the previous reports. Additionally, we strongly recommend evaluation of all risk factors for thrombosis and give appropriate anticoagulation.

To provide a clinicopathologic review of vasculitis confined to the female genital tract, we describe three cases, and we searched PubMed from 1965 to 2006 with analysis of all relevant articles. We identified 118 additional cases in the literature of whom 108 had isolated necrotizing vasculitis similar to classical polyarteritis nodosa (PAN-type), and 10 presented isolated giant cell arteritis (GCA-type) of the female genital tract. In most cases, arteritis was discovered surprisingly. The mean age of these patients was 48.6 years for the PAN-type and 64.1 for the GCA-type. Vasculitis affected a single organ in 88 (81.5%) cases of which 71 (65.7%) involved the cervix in the PAN-type, whereas it was limited in the myometrium in five (50%) cases in the GCA-type. There was no progression to a systemic vasculitis in 99.1% of the cases. A comparable favorable outcome was reported after surgery in all cases with a mean follow-up of 40.8 months. We conclude that isolated vasculitis of the female genital tract is a distinct condition, with two different patterns but a similar favorable outcome after resection. Its knowledge is needed to avoid aggressive evaluation and therapy.

Polyarteritis nodosa (PAN) presents mostly as a systemic disease with poor prognosis, rarely in an isolated form with a usually favorable outcome. Both forms may affect the male reproductive system and both forms have been associated with malignancies. We describe for the first time the occurrence of isolated PAN in the reproductive system combined with a mixed germ cell tumor of the testis in a 21-year-old man presenting with symptoms of chronic epididymitis. Two years after surgery he is without evidence of recurrence of either the tumor or PAN.

Polyarteritis nodosa is a systemic disease which affects the small to medium-sized muscular arteries. Sudden or progressive, bilateral hearing loss is a presenting otologic manifestation. To date, no case of cochlear implantation in patients with polyarteritis nodosa has been reported. The authors present a case of polyarteritis nodosa (confirmed by biopsy) in a 71-year-old man with progressive, bilateral sensorineural hearing loss who underwent cochlear implantation. A successful full insertion of the Nucleus 3G electrode array was achieved without surgical or post-operative complications. The patient immediately showed a positive subjective response and, at three month post-operative evaluation, had gained useful open-set speech perception. A review of five temporal bone cases with hearing loss and polyarteritis nodosa revealed the possibility of fibrosis and ossification in the basal turn of the cochlea, of which the surgeon should be aware prior to cochlear implantation.

This is a case report of a younger woman admitted to hospital with abdominal pain and weight loss. Four weeks after admittance, she was diagnosed with severe bowel ischaemia at laparotomy. Arteriography showed occlusive disease in the mesenteric arteries, which were treated with a stent. The entire intestine was preserved, apart from 10 cm of jejunum due to bowel perforation. The diagnosis of polyarteritis nodosa was confirmed based on biopsy and clinical symptoms.

A 44 year-old man presented to the Emergency Department of American Hospital with severe pain persisting in the left upper quadrant for several hours. A computed tomographic scanning (CT scan) and celiac digital substraction angiography (DSA) of the abdomen demonstrated a splenic infarct. Histopathological examinations showed diffuse acute vasculitis, thrombosis, panvasculitis which led us to diagnose the case as "polyarteritis nodosa". The diagnosis is usually difficult to establish and it is usually delayed due to variable clinical manifestations dependent on the site and the extent of arterial involvement.

The authors report a rare case of an acalculous gangrenous cholecystitis due to a form of vasculitis, polyarteritis nodosa (PAN). An 18-year-old man was admitted to the hospital with worsening symptoms of nausea, fever, intermittent abdominal pain, and high blood pressure that lasted for 4 days. After a sequential work-up, a diagnostic laparoscopy was performed and revealed a gangrenous cholecystitis with spontaneous perforation. Laparoscopic cholecystectomy was performed successfully. The patient had an uneventful recovery period and was discharged on the second postoperative day. The histopathologic examination showed gangrenous and perforated gallbladder, vasculitis, and clues of PAN. The purpose of this article is to describe a rare condition in a young patient that was diagnosed and treated with minimally invasive surgery.

Introduction: Polyarteritis nodosa is a systemic necrotizing vasculitis that may become serious, even with no usual poor prognosis factors., Exegesis: We report two cases of polyarteritis nodosa with negative histology, starting only with an extensive necrosis of the extremities. The treatment, associating corticosteroids and, secondarily, immunosuppressors, did not prevent a bilateral half-leg amputation for the two patients. In the first case the disease stabilized, but in the second one, it worsened, leading to death within 2 years., Conclusion: This clinical aspect of the disease is unusual and should be identified because of its bad prognosis. It might benefit from a treatment from the outset associating corticosteroids and immunosuppressors, even with no usual bad prognosis factors.

Background: Polyarteritis nodosa (PAN) is a multisystem necrotising small and medium sized vasculitis that when left untreated carries a grave prognosis, with a five year survival of 10-15%. Prolonged immunosuppressive treatment with cyclophosphamide and steroids leads to high remission rates while carrying the risk of life threatening complications. The diagnostic and therapeutic approach for patients with isolated genital tract PAN is not well defined., Objective: To present the management and follow up of two patients with limited PAN localised to the male and female reproductive system., Case Reports: A 26 year old man presented with an "acute scrotum". He was afebrile and had no other sign or symptom. Laboratory tests, including complete blood count, erythrocyte sedimentation rate, liver and renal function tests, C reactive protein, antinuclear antibody, cryoglobulins, complement levels, antineutrophil cytoplasmic antibodies, and hepatitis B surface antigen, were all normal. His left testis was excised. Histopathology disclosed PAN of medium sized arteries with testicular infarction but no signs of torsion or infection. The other patient was a 51 year old woman who had had a total hysterectomy for a uterine myoma; incidentally PAN of the uterus and fallopian tubes was discovered. Neither patient received any immunosuppressive treatment after surgical removal of the affected organ. On prolonged follow up (clinical and laboratory evaluation) both patients are healthy with no sign of local recurrence or systemic PAN.

Polyarteritis nodosa (PAN) is a rare systemic necrotizing arteritis that involves small- and medium-sized arteries in various organs. Although aneurysm formation in visceral arteries is a typical finding in PAN, intracranial aneurysms are much less common, and only a few cases of aneurysm rupture associated with this disease have been documented. In this paper, the authors report on a ruptured PAN aneurysm of the anterior cerebral artery; the lesion was trapped and resected. On histological examination, extensive fibrinoid necrosis and an inflammatory infiltration of leukocytes were seen in the aneurysm wall. To the authors' knowledge this is the first report of subarachnoid hemorrhage from a histologically confirmed PAN aneurysm.

Arteriovenous malformation of the pancreas is a very rare entity. We describe a case involving the head of the pancreas associated with progressive hemobilia bleeding from the lower part of the common bile duct. The patient was a 50-year-old man with acute epigastralgia. Endoscopic retrograde cholangiopancreatography revealed hemobilia and cystic dilation of the common bile duct. Angiography demonstrated increased blood volume in the head of the pancreas and early filling of the superior mesenteric and portal veins. Abdominal pain and progressive anemia caused by hemobilia required surgical treatment. Histologic examination of the resected specimen revealed marked proliferation of the blood vessels in the pericholedochal area and the exact point of bleeding from the pancreaticobiliary arteriovenous malformation.

A 16-year-old boy with a painful tumor in the left scrotum was referred to our department. CT scans showed a low density area in the left testis, so we diagnosed a left testicular tumor and performed left inguinal orchiectomy. Histological examination revealed polyarteritis nodosa (PN) of the testis and epididymis. Systemic examination revealed no other evidence of PN. Although induration developed in the right epididymis after the operation, it resolved with steroid therapy. The patient is currently asymptomatic and is being followed at our clinic. The pathogenesis and management of this rare condition are discussed.

We describe magnetic resonance imaging findings in a 37-year-old man with a rare entity of isolated polyarteritis nodosa of the epididymis, which correlated well with the histopathologic findings.

Aims: The vasculitides represent a heterogenous set of disorders that differ in prognosis and response to therapy. Beside systemic vasculitides, the development of localized forms of arteritis is well known though uncommon and the etiopathogenesis is not yet definitely clear., Methods: Patients with necrotizing arteritis of the female genital tract proven by histology are studied in a retrospective analysis., Results: Three cases of necrotizing arteritis with histological features of panarteritis nodosa apparently confined to the female genital tract are presented. None of these patients had prior history of systemic vasculitis. The acute necrotizing vasculitis was confined only to the uterine cervix in two patients and involved all the internal genital organs in the third patient. The patients have been observed for up to 4 years without any therapy for these lesions and without any manifestation of systemic vasculitic progression., Conclusion: It is to speculate that focal arteritis of the female genital tract is a benign form of panarteritis nodosa or moreover a totally different entity with identical morphogenesis but possibly different pathogenesis. Furthermore it seems to be important to be aware of the specificity of focal arteritis in female genital tract as distinct from the generalized form to prevent unnecessary surgical or chemotherapeutical therapy for this lesion. The benign entity of local arteritis in the female genital tract is discussed in contrast to the severe prognosis of systemic panarteritis nodosa.

Forty-six cases of vasculitis affecting the female genital tract are described; only 41 similar cases have been previously reported, either as case reports or small series. The age range of the patients was from 22 to 80 years, and most of them presented with abnormal bleeding or were being treated conditions unrelated to the vasculitis. There were 39 hysterectomy specimens (26 of which were derived from total abdominal hysterectomies) and seven specimens of the cervix only. The vasculitis was confined to the cervix in 30 of the 46 cases; in 24 of these, the entire uterus was available for examination. In 23 cases, only a single vessel was involved, and in the other 23 there was more extensive vessel involvement. In all cases, the involved vessels were arterioles and small arteries. In 42 cases, the arteritis was of the polyarteritis nodosa (PAN) type, and in four, of the giant cell type (GCA). Follow-up ranged from < 1 year to 23 (mean, 3) years. Systemic manifestations were previously diagnosed or subsequently developed in only four patients, three with PAN and one with GCA; in each of them, the genital tract vasculitis was found only in the cervix (in one of these, however, the specimen was a loop excision of the cervix and the rest of the uterus was not assessable). The three patients with PAN subsequently developed extragenital PAN (one case), PAN and rheumatoid arthritis (one case), or PAN and polymyalgia rheumatica (one case). The patient with GCA had previously documented temporal arteritis and temperomandibular arthritis. The findings in this series and in previously reported cases indicate that vasculitis of the female genital tract is only rarely associated with systemic vasculitis.

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.