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An 80-year-old woman positive for myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) was admitted with a 3-month history of fever, general malaise, and weight loss, after unsuccessful treatment with antibiotics. Upon admission, her fever persisted, and there was concomitant deterioration of renal function without active urine sediments. Furthermore, she developed hemoptysis, and chest computed tomography (CT) scan revealed bilateral diffuse alveolar hemorrhage. Although a renal biopsy was not performed because of her dementia, we initially suspected microscopic polyangiitis (MPA) on the basis of her clinical course. Because of her poor general condition, she was administered a low dose of prednisolone. Although her fever subsided, she suffered from intractable alveolar hemorrhage and eventually died from respiratory failure. During the autopsy, fibrinoid necrosis was restricted to medium-sized arteries, including the arcuate arteries of the kidneys and the bronchial arteries, without necrotizing crescentic glomerulonephritis and alveolar capillaritis. Therefore, polyarteritis nodosa (PAN) was diagnosed. It is important to distinguish between MPA and PAN because they can lead to life-threatening complications, and their treatment strategies and prognosis are different. When a patient presents with MPO-ANCA, alveolar hemorrhage, and acute renal failure with little evidence of glomerulonephritis, a differential diagnosis of PAN should be made; however, it is difficult to do so without pathological findings. Therefore, pathological examination should be carried out whenever possible.

Typical angiographic finding of polyarteritis nodosa (PAN) shows aneurysms in small- and medium-size arteries, which can assist in diagnosis. Mimics of vasculitis share similar angiographic and clinical manifestations with PAN, making diagnosis confusing. We report 2 patients admitted with hemobilia, one of whom had vasculitis and case of the other mimicked vasculitis.The first patient was an 18-year-old man with epigastric pain. On serial workup, hemobilia with multiple hepatic artery aneurysms was diagnosed and embolized. Later, PAN was confirmed on pathologic examination of gallbladder after cholecystectomy.Second patient was an 18-year-old woman also with epigastric pain. Serial workup revealed multiple microaneurysms of the hepatic artery without bleeding, but 4 days later, fistula between hepatic artery and bile duct was found, and embolization was performed. She was readmitted 1 month later because of hemorrhage of gallbladder, and pathologic examination of gallbladder showed evidence of fibromuscular dysplasia.Clinicians should be aware of mimics of vasculitis when diagnosing gastrointestinal involvement of vasculitis.

We examined the prevalence of various cutaneous symptoms including livedo racemosa and inflammatory plaques, lupus anticoagulant (LA), anticardiolipin (aCL) antibodies (Abs), and anti-phosphatidylserine-prothrombin complex (anti-PS/PT) Abs in patients with cutaneous polyarteritis nodosa (PAN) to determine if any of them correlate with the clinical and/or serologic features. If such correlations exist, the clinical and serologic features of the cutaneous manifestations could aid in the early diagnosis and/or treatment of cutaneous PAN. We retrospectively investigated the clinical and serologic features, direct immunofluorescence findings, and treatment methods used in 50 patients with cutaneous PAN seen at our Department of Dermatology between 2003 and 2009. Subcutaneous nodules were observed in all 50 patients, 44 (88.0%) had livedo racemosa, 30 (60.0%) had skin ulcers, and 14 (28.0%) had inflammatory plaques. Levels of serum IgM anti-PS/PT Abs were significantly higher in patients with livedo racemosa than in patients without livedo racemosa. Serum IgG anti-PS/PT Ab levels differed significantly between patients with inflammatory plaques (12.86 ± 13.16 U/mL) and those without inflammatory plaques (6.53 ± 5.92 U/mL). Similar trends were seen with respect to IgG aCL Ab levels. In contrast, levels of IgM anti-PS/PT Abs were significantly lower in patients with inflammatory plaques compared to patients without them. Inflammatory plaques were significantly more prevalent in patients with skin ulcers. Warfarin and prednisolone were selected as the primary therapy at a significantly higher rate in patients with inflammatory plaques and skin ulcers than in patients without them. We suggest that a variety of antiphospholipid Abs could influence the cutaneous patterns of cutaneous PAN. In particular, IgG anti-PS/PT Abs and/or IgG aCL Abs could indicate the presence of inflammatory plaques as a specific cutaneous manifestation of cutaneous PAN.

The presence of an internal elastic lamina and a compact concentric muscular layer are the cardinal histologic clues for distinguishing a small muscular artery from small muscular vein. However, the subcutaneous muscular veins in the lower legs usually have thick muscular layers with the proliferation of concentric intimal elastic fibers, which resembles the internal elastic lamina of an artery. Moreover, vertical biopsy specimens of the muscular veins can reveal a compact concentric muscular layer with a round luminal appearance, which also resembles the muscular layer in an artery. As these 2 histologic features are commonly accepted as crucial clues for identifying small to medium-sized muscular arteries, it seems that many cases that are histopathologically proven to be deep dermal or subcutaneous arteritis-including cases documented in numerous dermatology, rheumatology, and dermatopathology-related journals as cutaneous polyarteritis nodosa in Behçet's disease and relapsing polychondritis or granulomatous arteritis in nodular vasculitis-are actually consistent with the features of phlebitis or thrombophlebitis. Cutaneous polyarteritis nodosa and subcutaneous thrombophlebitis are usually found in the lower legs and may present with the same cutaneous manifestation of widespread tender or painful nodular erythema. This also accounts for the difficulty in clinically and histopathologically distinguishing between these 2 disorders. Nevertheless, it is important to make a distinction between arteritis and phlebitis because misdiagnosing subcutaneous thrombophlebitis as polyarteritis nodosa may lead to overtreatment with high doses of systemic steroids. Although the veins in the lower legs may have a compact concentric smooth muscle pattern with a round lumen and the intimal elastic fiber proliferation mimicking the characteristic features of arteries, the elastic fibers in the muscular layer are distributed between the bundled smooth muscle in veins, whereas the elastic fibers are scantly distributed in the medial muscular layer in arteries. A diagnostic assessment that is based on the amount of the elastic fibers in the muscular vessel wall more reliably distinguishes a vein from an artery than does the presence or absence of the internal elastic lamina or a smooth muscle pattern.

Background: Cutaneous polyarteritis nodosa (CPN) is an uncommon disorder that can be difficult to manage effectively. We have previously suggested that CPN might be associated with the presence of anti-phosphatidylserine-prothrombin complex (anti-PS/PT) antibodies, members of the antiphospholipid antibody family., Objective: To evaluate clinical manifestations and effective treatments of CPN., Methods: We conducted a retrospective analysis of three patients with CPN who responded to warfarin therapy. IgG and IgM anti-PS/PT antibodies were measured with a specific enzyme-linked immunosorbent assay., Results: There was a dramatic improvement in our three CPN patients following warfarin therapy adjusted to a target international normalized ratio (INR) of about 3.0. Active disease progression was halted by sustained warfarin therapy during which the patients experienced resolution of their skin manifestations., Limitations: A small number of cases were studied and the study design was retrospective., Conclusion: We propose that warfarin therapy at a target INR of roughly 3.0 could be effective for treating patients with CPN. We further believe that treatment with warfarin led to the effective attenuation of anti-PS/PT antibodies related to prothrombin, and improved the symptoms in our CPN patients., (Copyright © 2009 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.)

Pulmonary hypertension is clinically defined as a pulmonary arterial pressure greater than 25 mmHg at rest or greater than 30 mmHg during exercise. We report a unique case of an infant with congenital heart disease causing pulmonary hypertension and necrotizing pulmonary arteritis but affecting only one lung. In conclusion, in pulmonary valve atresia, the lungs may be supplied via aortopulmonary collaterals. Necrotizing pulmonary arteritis is a severe lesion seen in irreversible pulmonary hypertension., (Copyright 2009 Elsevier GmbH. All rights reserved.)

Cutaneous polyarteritis nodosa is a rare form of vasculitis relating to small-to-medium-sized arteries. Its etiology is unknown. Clinical manifestations include tender subcutaneous nodules, livedo reticularis, cutaneous ulcers and necrosis. Although it is distinct from systemic polyarteritris nodosa in that it lacks significant internal organ involvement, extra-cutaneous manifestations may be evident. Commonly encountered symptoms include fever, malaise, myalgias, arthralgias, and paresthesias. Exclusion of systemic polyarteritis nodosa is essential in diagnosis. The clinical course is chronic with remissions, relapses, and a favorable prognosis. Mild cases may resolve with nonsteroidal anti-inflammatory drugs. If more severe, treatment with systemic corticosteroids generally achieves adequate response; however, adjunctive therapy is often necessary to allow reduction in steroid dosage.

Purpose: Polyarteritis nodosa is one of the common forms of vasculitis with multiorgan involvement. Hearing loss may be the presenting symptom of this disease. The aim of this histopathologic study was to evaluate temporal bone changes in polyarteritis nodosa and assess the correlation between otologic manifestations and histopathologic findings., Materials and Methods: A retrospective human temporal bone analysis was performed at an otopathology laboratory in a tertiary academic medical center. Three cases (5 temporal bones) with pathologically confirmed diagnosis of polyarteritis nodosa were selected for study. The processed temporal bone sections were studied under light microscopy., Results: Two of the 3 cases presented with hearing loss, and one of them had facial palsy. Audiometric data available for one patient demonstrated a bilateral, rapidly progressive, mixed hearing loss. Histopathologically, we found generalized vasculitis involving small- and medium-sized arteries in all cases. Of the 5 temporal bones available for study, 3 had chronic otitis media and 2 had serous otitis media. Arteritis was seen in all middle ears. There was disruption of the organ of Corti and spiral ganglion cells in 4 temporal bones. One temporal bone showed fibrosis and osteogenesis in the scala tympani of the basal turn. Vasculitis of stylomastoid and branches of labyrinthine artery were also found in temporal bones., Conclusions: Significant temporal bone findings were seen in patients with polyarteritis nodosa, including otitis media, cochlear damage, neo-osteogenesis, and middle and inner ear vasculitis. This temporal bone series also suggests an interesting relationship between sensorineural hearing loss and labyrinthine vasculitis., (Copyright 2010 Elsevier Inc. All rights reserved.)

Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis involving predominantly medium-sized muscular arteries. It commonly involves skin, kidney, cardiovascular system, gastrointestinal system, and neurological system. But bronchial artery involvement of PAN is rarely identified. We report a case of PAN with initial presentation of hemoptysis. On admission, chest radiograph and chest CT angiography revealed no focus of bleeding. Angiography showed a bronchial artery aneurysm and multiple arterial aneurysms in both renal, hepatic, mesenteric and branches of small bowel arteries. These findings were compatible with the diagnosis of PAN. The patient was started on steroid and cyclophosphamide.

Polyarteritis nodosa is a necrotizing vasculitis of medium-sized arteries of unknown origin. Hypertension is present in 30% of patients with polyarteritis nodosa. In those cases, high renin levels are thought to be secondary to renal involvement. The present study was performed to identify causal factors of polyarteritis nodosa. In cyp1a1ren-2 transgenic rats, vasculitis of medium-sized arteries resembling classical polyarteritis nodosa can be induced. In this model, oral administration of indole-3-carbinol (I3C) activates the liver-specific cyp1a1 promoter, leading to prorenin expression in a dose-dependent manner. After the first 6 weeks of chronic induction with 0.125% I3C, the mean arterial pressure reached a plateau of about 170 mmHg. Ten out of 11 I3C-treated rats, which were chronically instrumented with a telemetric device to measure blood pressure, developed polyarteritis nodosa within 10 weeks of I3C treatment. I3C alone or instrumentation alone did not cause polyarteritis nodosa. The angiotensin-converting enzyme inhibitor captopril completely prevented the development of polyarteritis nodosa, indicating that local angiotensin II generation is a pathogenetic factor in this model. The renin-angiotensin system can play a primary role in the development of polyarteritis nodosa in rats.

Although Kawasaki disease (KD) was first discovered and identified in Japan by Kawasaki in the 1960s, fatal KD cases resulting from coronary artery aneurysms had been identified retrospectively in the West as early as 1871. Kawasaki initially postulated that this disease was a new, as yet unidentified, self-limiting illness with no fatal coronary sequelae. The connection between fatal cases, then diagnosed as infantile polyarteritis nodosa, was not made until the late 1970s. Kawasaki's thoughts were reinforced by an apparent absence of nonfatal cases in the West before 1967. Close examination of a 1948 autopsy report suggests that nonfatal cases of KD did indeed exist, at least in the United States, before its emergence in Japan in the early 1950s. These nonfatal cases of KD were misdiagnosed as Stevens-Johnson syndrome. The autopsy report reviewed in this article reinforces the likelihood that KD did occur in the United States before it was identified as Kawasaki disease in Japan.

Subacute or chronic orbital inflammation can cause proptosis or painful myositis. It is either primitive or secondary to systemic diseases such as polyarteritis nodosa or Wegener's granulomatosis. Corticosteroids are the basic treatment, allowing biopsy and thus anatomopathological diagnosis. Some authors advocate surgery., (Copyright 2010 Elsevier Masson SAS. 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 6-year-old boy presented with post-burn like cutaneous scars over the buttocks and the back of thighs, following skin ulceration. Cutaneous polyarteritis (CPA) was diagnosed based on the histopathological examination of the skin biopsy specimen from one of the scars. Such a presentation of childhood CPA, to our knowledge, is not documented in the English literature.

Objective: Previous studies of polyarteritis nodosa (PAN) included patients with microscopic polyangiitis, because these entities were not distinguished prior to the Chapel Hill Consensus Conference (CHCC). This study was undertaken to describe the main characteristics of and long-term outcomes in patients with well-characterized PAN diagnoses., Methods: We conducted a systematic retrospective study of 348 patients who were diagnosed as having PAN between March 1963 and October 2005, were registered in the French Vasculitis Study Group database, and satisfied the American College of Rheumatology and CHCC criteria. Patient characteristics and outcomes were analyzed and compared according to hepatitis B virus (HBV) status., Results: At diagnosis, the mean +/- SD age was 51.2 +/- 17.3 years. The most frequent findings were general symptoms (93.1%), neurologic manifestations (79%), skin involvement (49.7%), abdominal pain (35.6%), and hypertension (34.8%); 66.2% had renal artery microaneurysms; 70.1% had histologically proven PAN. Patients with HBV-related PAN (n = 123) had more frequent peripheral neuropathy, abdominal pain, cardiomyopathy, orchitis, and hypertension compared with patients with non-HBV-related PAN (n = 225). During a mean +/- SD followup of 68.3 +/- 63.5 months, 76 patients (21.8%) relapsed (63 with non-HBV-related PAN [28%] versus 13 with HBV-related PAN [10.6%]; P < 0.001); 86 patients (24.7%) died (44 with non-HBV-related PAN [19.6%] versus 42 with HBV-related PAN [34.1%]; P = 0.003). Five-year relapse-free survival rates were 59.4% (95% confidence interval [95% CI] 52.6-67.0) versus 67.0% (95% CI 58.5-76.8) for non-HBV-related PAN and HBV-related PAN, respectively. Multivariate analysis retained age >65 years, hypertension, and gastrointestinal manifestations requiring surgery or at least consultation with a surgeon as independent predictors of death, whereas patients with cutaneous manifestations or non-HBV-related PAN had a higher risk of relapse., Conclusion: Our findings indicate that the rate of mortality from PAN remains high, especially for the elderly, and relapses do occur, particularly in patients with non-HBV-related PAN with cutaneous manifestations.

History: A 65-year-old female was admitted with fever of unknown origin., Diagnostic Procedures: Abdominal computed tomography showed a solid mass (7.5 cm in diameter) with central fluid, located in the right lobe of the liver. Fine-needle aspiration cytology was unremarkable. Further work-up procedures for suspected liver abscess included colonoscopy, which surprisingly revealed adenocarcinoma at 13 cm from the anal orifice., Therapy and Clinical Course: Both lesions in the rectum and liver were resected. While a moderately differentiated (G2) adenocarcinoma of the rectosigmoid junction (stage T3/ N0) was confirmed, histology of the hepatic mass showed liver infarction due to polyarteritis nodosa of the medium-sized arteries. Treatment with 20 mg/d prednisolone was initiated and tapered off over the next three months. The clinical course after discontinuation of corticosteroids was unremarkable over a 6-month follow-up., Conclusion: It is suggested that polyarteritis nodosa of the liver occurred in this patient as a paraneoplastic phenomenon and subsided after resection of colorectal cancer and short-term immunosuppression with prednisolone., (Copyright Georg Thieme Verlag KG Stuttgart . New York.)

A 25-year-old-man was referred with an approximately 1-year history of recurrent fever, tonsillitis, testicular pain and testicular swelling in June 2009. He also complained of visual disturbance, some erythema nodosum (EN) like lesions on his lower extremities and oral aphthous ulcerations for 5 months. Opthalmological consultation confirmed retinochoroiditis, and ultrasonography revealed epididymitis. A biopsy of the EN like lesion showed a necrotizing vasculitis of the small and medium-sized vessels with septal panniculitis in the subcutis. The laboratory findings revealed an elevation of CRP and positive HLA-B51. He was diagnosed with incomplete Behcet's disease according to the Japanese criteria (1987). After the initiation of the therapy with 10 mg/day of prednisolone, his symptoms promptly ameliorated. In our case, although epididymitis as well as the histopathological findings suggested polyarteritis nodosa (PN), a diagnosis of BD was established on the basis of other contemporaneous findings. Necrotizing vasculitis as a cutaneous manifestation in patients with BD has been rarely reported. However, BD and PN, including cutaneous PN, have rarely described in conjunction. Therefore, we propose that PN-like necrotizing vasculitis might be the subtype of BD.

Sixteen cases of cutaneous polyarteritis nodosa referred to our Department from 1985 to 2003 were studied clinically and histopathologically. Laboratory data, treatments and clinical courses were also evaluated retrospectively. All cases had nodules and/or indurated erythemas on their lower extremities. All cases showed necrotizing vasculitis of small muscular arteries in the subcutaneous tissues and/or occlusion of those arteries histopathologically. Fifteen cases also had accumulation of plasma protein in vessels of the dermis and subcutaneous tissues. Laboratory data showed high activity of platelets and coagulation in some cases. Eleven cases had been effectively treated with non-steroidal anti-inflammatory drugs. Eight cases were observed for at least 5 years (the longest for approximately 19 years) and had good prognoses and no systemic involvement. Cutaneous polyarteritis nodosa seems to be a benign disease, and differs from systemic polyarteritis nodosa although their histopathological features are common. Cutaneous polyarteritis nodosa might involve local dysfunction of the circulation from the dermis to the subcutaneous area. A review of the published work shows that the cause(s) of most cases of cutaneous polyarteritis nodosa is unknown, that no controlled trials for treatment of cutaneous polyarteritis nodosa compared to polyarteritis nodosa have been reported, and that no definitively effective therapy for cutaneous polyarteritis nodosa has been established.

Objective: Myocardium and coronary arteries can occasionally be affected in patients with systemic necrotizing vasculitides; however, such involvement has not been systematically assessed using cardiovascular magnetic resonance imaging (MRI)., Methods: Magnetic resonance angiography and contrast-enhanced MRI were applied for the assessment of coronary arteries (the left anterior descending [LAD], left circumflex [LCx], and right coronary artery [RCA]) and myocardium, respectively, in 39 patients with vasculitis who were asymptomatic for cardiac disease (16 with microscopic polyangiitis [MPA], 11 with Wegener's granulomatosis [WG], 9 with Churg-Strauss syndrome [CSS], and 3 with polyarteritis nodosa [PAN]). Data were compared with age-matched disease-control patients with rheumatoid arthritis (n = 20) or systemic lupus erythematosus (n = 13), and with healthy control individuals with normal coronaries (n = 40)., Results: Patients with MPA, WG, and PAN (but not with CSS) were found to display significantly increased maximal diameters of coronary arteries compared with healthy controls (for MPA and WG; P < 0.001 for LAD and RCA, and P < 0.01 for LCx) and with both disease-control groups (for only MPA; P < 0.01 for LAD and RCA, and P < 0.05 for LCx). Fusiform coronary aneurysms were detected in patients with MPA (4/16) and PAN (2/3), whereas coronary ectasias were evident in patients with MPA (14/16) and WG (2/11). The presence of myocardial necrosis (by assessment of late gadolinium-enhanced images) was identified only in patients with MPA (2/16) and CSS (3/8 studied)., Conclusion: Cardiovascular MRI assessment of patients with systemic vasculitis revealed coronary ectatic disease in the majority of patients with MPA and PAN, as well as in several patients with WG. Myocardial necrosis can be detected in MPA and CSS.

Subcutaneous calcification often occurs in connective tissue diseases, most commonly scleroderma and dermatomyositis, but is rarely found in mixed connective tissue disease (MCTD). Cutaneous polyarteritis nodosa (PAN) is usually a primary skin disorder and although associated with connective tissue disease, has not been reported previously in MCTD. Calcinosis in cutaneous PAN is not a recognized feature. We describe the case of a 37-year-old woman who presented with tender ulcerated subcutaneous nodules on the lower legs consistent with cutaneous PAN, and she also showed features of MCTD with extensive secondary subcutaneous calcification. The use of systemic immunosuppressive treatment has improved the clinical features of PAN and MCTD but treatment of the calcification has proved challenging. No single medical or surgical treatment has been shown to be consistently effective in subcutaneous calcification, but the introduction of diltiazem in our patient has resulted in some improvement.

A 48-year-old patient presented with a nonhealing leg ulcer and a raised white blood cell count. He was diagnosed with pyoderma gangrenosum (PG) and small lymphocytic leukaemia/chronic lymphocytic leukaemia (SLL/CLL). Eight months later, after undergoing treatment with chlorambucil for the SLL/CLL, and prednisone, ciclosporin and intravenous immunoglobulin for the PG, the patient developed livedo reticularis and palpable purpura, and was diagnosed with systemic polyarteritis nodosa (PAN). The case highlights the difficulty in establishing a diagnosis of PAN by biopsy of cutaneous ulcers alone, and that a diagnosis of PG should raise suspicion of another aetiology.

Background and Purpose: Lung fibrosis as first symptomatic manifestation of microscopic polyangiitis (MPA) is rare. It is characterized by progressive exertional dyspnea, radiologic detection of reticular shadowing, and increased titers of MPA-associated autoantibodies. This case study shall remind of the rare treatable cause of lung fibrosis., Case Report: A 78-year-old male patient with long-standing exertional dyspnea and typical chest radiograph appearances of advanced lung fibrosis is presented. The finding of MPA autoantibodies in laboratory tests was decisive to confirm the diagnosis of MPA, and a therapy adapted to the disease severity could be initiated., Conclusion: An early diagnosis of MPA and a fast onset of therapy are important, because the influence of an already existent lung fibrosis is uncertain.

Introduction: Cutaneous polyarteritis nodosa (CPAN) is an entity which needs to be acknowledged, since it can have a spontaneously adverse outcome. We report two cases of CPAN associated with Crohn's disease., Case Reports: The first patient was suffering from Crohn's disease for 9 years when she was referred for a necrotic toe. A diagnosis of necrotizing angeitis was confirmed by histological examination of a skin biopsy. Despite systemic corticosteroids, the lesions became more severe, requiring immunosuppressive treatment. The second patient was a female patient referred with forefoot ischemia. Cutaneous histology confirmed the diagnosis of necrotizing angeitis that responded favourably to corticosteroid treatment. The patient had been diagnosed with Crohn's disease 2 months previously., Conclusion: CPAN differed from systemic PAN by the absence of visceral involvement. Its association with Crohn's disease, although uncommon, must be recognized as it affects treatment and monitoring.

This is the first report of segmental arterial mediolysis (SAM) accompanied with polyarteritis nodosa (PN), and manifesting aneurysms of the renal arteries. A 73-year-old woman was admitted to hospital because of a high fever. Laboratory tests showed leukocytosis with increased CRP level in the serum. Myeloperoxidase-anti-neutrophil cytoplasmic antibody (MPO-ANCA) and proteinase 3 (PR3)-ANCA were negative. There were no signs indicating infection or malignancy. After admission renal function rapidly deteriorated. Treatment was then started with daily oral prednisolone and hemodialysis. On the 40th day of hospitalization the patient suddenly became comatose. Cranial CT showed a subarachnoid hemorrhage. The patient died and an autopsy was performed. The pathological findings showed necrotizing vasculitis of the small arteries in various organs, but not associated with that of arterioles or renal glomerular lesions, indicating PN. Unexpectedly, the segmental arteries of the bilateral kidneys showed vascular lesions of dissecting aneurysms, indicating SAM. This case indicates that SAM is one of the causes of aneurysms in PN and is clinically important when the clinical course of PN patients rapidly advances.

We report on the first association of celiac disease, polyarteritis nodosa and HBV infection in a patient who developed a neuropathy. On admission his general and neurological conditions were severely compromised. Haematological test revealed HBV infection and high levels of antibodies to tissue transglutaminase, endomysium, gliadin. EMG showed sensory-motor asymmetric axonal neuropathy. A sural nerve biopsy revealed fibre loss, axonal degeneration with asymmetrical distribution and fascicular ischaemia. A duodenal biopsy was consistent with celiac disease. The patient was treated with immunosuppressive and antiviral therapy, and gluten-free diet with good result. Celiac disease can be related to a higher risk of autoimmune disorders and may have contributed to the development of multineuropathy in this patient.

Cutaneous polyarteritis nodosa commonly affects the distal lower limbs, presenting as nodules, ulcers, and livedo reticularis. We report five cases to illustrate a new specific presentation of cutaneous polyarteritis nodosa on the trunk or proximal extremities. In the acute stage, lesions were tender erythematous plaques. On palpation, 1- to 2-cm diameter subcutaneous nodules were found along the periphery. The lesions responded to dapsone, aspirin, nonsteroidal antiinflammatory drugs, or systemic steroids and healed with postinflammatory hyperpigmentation. Systemic polyarteritis nodosa did not develop in any patient after a follow-up time ranging from 9 months to 9 years. The presence of small nodules at the periphery of an inflammatory plaque was a useful clinical clue, because this prompted us to suspect cutaneous polyarteritis nodosa when we encountered similar cases later, which could be confirmed histologically. In conclusion, cutaneous polyarteritis nodosa can present as inflammatory plaques on the trunk and proximal extremities, and the presence of peripheral nodules around these plaques constitutes a useful clinical clue to its diagnosis.

Objectives: To assess the extent and pattern of irreversible organ damage in patients with Wegener's granulomatosis (WG), microscopic polyangiitis (MPA), polyarteritis nodosa (PAN), and Churg-Strauss syndrome (CSS) by a cross-sectional point prevalence study within a defined geographical area., Methods: The Vasculitis Damage Index (VDI) was recorded for 86 prevalent cases, classified as 46 patients with WG, 27 with MPA, nine with PAN, and four with CSS from a defined population in southern Sweden, with a median age of 64.8 years and a median disease duration of 9 years. The VDI was determined for all patients at the day of point prevalence (pp), 1 January 2003., Results: The median VDI score was 3 [interquartile range (IQR) 2-5] for all patients: 3 (2-4) for WG, 3 (1.5-4.5) for MPA, 5 (2-6) for PAN, and 1.5 (0.75-2.75) for CSS. Only 9% of patients had not been assigned a single item of damage. The most common damage was cardiovascular, followed by renal, neuropsychiatric, ear nose and throat (ENT), and musculoskeletal. Major vascular and treatment-related damage was associated with advanced age whereas ENT damage was more prevalent in younger patients. There was an almost complete separation between ENT damage and cardiac and renal damage with only two out of the 22 patients assigned ENT damage having experienced renal damage; none had been assigned cardiac damage. Patients with cardiac damage had significantly higher damage rates., Conclusions: Damage remains an important problem for patients with systemic vasculitis despite effective remission-inducing drugs. Only a small fraction of patients are unmarked by their disease.

A comatose 30-month-old, entire male boxer was presented because of an acute history of a cluster of three to four seizures. Neurological examination suggested a diffuse to multifocal intracranial lesion. Magnetic resonance tomography revealed symmetrical multifocal to diffuse changes of the cerebral grey matter and ependymal lining with sediment in the lateral ventricles. Haematological examination revealed leucocytosis with neutrophilia. Cerebrospinal fluid examination revealed high protein concentration and polymorphonuclear pleocytosis. Despite antiepileptic treatment, therapy against increased intracranial pressure and antibiosis, the dog's condition continued to deteriorate and he was euthanased. Pathological examination revealed fibrinosuppurative meningo-ependymitis and necrotising arteritis throughout the brain. In addition, chronic inflammation and arterial stenosis was found in the spinal meninges. No infectious agent was found. A diagnosis of steroid-responsive meningitis arteritis was made. The massive extension into the meninges and ventricular system of the forebrain has not been described previously in dogs with steroid-responsive meningitis arteritis and should be considered in the differential diagnosis when an intracranial suppurative infection is suspected.

We describe a 54-year-old man presenting with cutaneous ulcerations, livedo reticularis, numbness of the legs, and skin histological findings compatible with the diagnosis of polyarteritis nodosa (PAN). Initial treatment with 50 mg/day of prednisolone (PSL) was effective. However, the symptoms and signs recurred, and the patient developed multiple periurethral aseptic abscesses, urethra-cutaneous fistula, and testicular lesions after tapering of PSL therapy. The condition improved with PSL and cyclophosphamide administration. Since penile and testicular vasculitis could be associated with PAN, although rarely, we should carefully distinguish such an involvement from infection and malignancy.

Isolated vasculitis involving the bladder is rare. We describe a case of irritative voiding and hematuria with slightly elevated inflammatory parameters. In order to exclude a neoplastic process we performed cystoscopy and computed tomography. Secondary systemic causes such as lupus erythematosus or rheumatoid arthritis were excluded. Cytopathological examination of the biopsies showed necrotizing vasculitis of the small bladder vessels, which responded to treatment with steroids and cyclophosphamide.

Introduction: Vasculitis is an inflammatory disorder of the vessels, often associates with necrosis. The primary (idiopathic) form caused by distinct immunological mechanisms, the secondary form caused by infection, tumor, drugs or systemic autoimmune disease., Case Report: 57-year-old smoking female patient with a medical history of the left ankle fracture. Four days earlier had sore throat, at admission was febrile and generally weak and had severe pain in her lower extremities. Physical examination revealed numerous livid alterations in both lower extremities, necrotic change of some toes and new onset of diastolic hypertension. Elevated serum transaminase levels, severe thrombopenia, anemia, leukocytosis suggested the possibility of thrombotic thrombocytopenic purpura and vasculitis. Tests ruled out septic embolism, therefore we applied high dose steroid treatment and plasma exchange. Meanwhile the results fulfilled the classification criteria of polyarteritis nodosa (vasculitis, livedo reticularis, polyneuropathia and new onset of diastolic hypertension), accordingly we synchronized the treatment with fractionally administered intravenous cyclophosphamide. The process revealed responsive to therapy and the substantive function of the legs remained after surgical removal of the necrotic areas., Conclusions: Early rapid diagnosis of vasculitis is fundamental and we emphasize the significance of the efficient treatment.

Background: The cutaneous manifestations of microscopic polyangiitis (MPA) and polyarteritis nodosa (PAN) have not been compared since their distinction. Objectives To compare the clinical and pathological cutaneous manifestations in a series of patients with systemic MPA and PAN., Methods: Patients with MPA (n = 162) and PAN (n = 248) from the database of the French Vasculitis Study Group were diagnosed according to the American College of Rheumatology and/or the Chapel Hill Consensus criteria. Purpura, livedo, nodules, urticaria, skin necrosis, oral and genital ulcers were recorded when present. Fifty-five skin biopsies were analysed. Clinical and histological skin data were compared in the following groups: MPA, PAN and two PAN subsets: PAN with and PAN without hepatitis B infection. The prevalence of systemic and biological manifestations were analysed in relation to the presence or absence of skin lesions. The chi(2) test was used for statistical studies., Results: Cutaneous manifestations were present in 44% of MPA and PAN. Purpura was the most frequent manifestation (26% cases of MPA vs. 19% cases of PAN, P = 0.026). Urticaria was more frequent during PAN (6% vs. 1.2%, P = 0.015). Skin lesions were more frequent during PAN in the absence of HBV infection (54% vs. 30%, P < 0.05). No significant difference was detected from the histological data. Patients with skin lesions (either MPA or PAN) presented arthralgias and ocular manifestations more frequently. Mononeuritis multiplex was associated with skin lesions in the MPA group (P < 0.05)., Conclusions: The clinical or histological analysis of cutaneous lesions is not helpful for distinguishing PAN from MPA.

Selective immunoglobulin A deficiency (IgAD) is a primary immunodeficiency disease characterized by low levels (< 7 mg/dl) of serum immunoglobulin (Ig) A and normal serum levels of IgG and IgM. Patients with IgAD have increased risk for recurrent respiratory and gastrointestinal infections, autoimmune disease, asthma and allergy. A 26-year-old woman was admitted with sudden onset of painful cutaneous lesions on her lower extremities, pyrexia and arthromyalgia. Her medical history was remarkable for recurrent respiratory tract infections, self-limited episodes of acute diarrhea, atopy, splenomegaly and a 4-year history of a lung granulomatous lesion. Laboratory and imaging tests ruled out severe life-threatening infection, connective tissue disease and neoplasm. Serum protein electrophoresis showed a low IgA serum level (6.67 mg/dl), with normal serum levels of IgG and IgM, conducting to a diagnosis of selective IgAD. A skin biopsy showed necrotizing vasculitis without any sign of internal organ disease. We report a patient with IgAD and granulomatous involvement of lungs, spleen and medium-sized arteries of the skin. Although IgAD results from a failure of B-cell differentiation, we propose that deregulated immune response with production of cross-reactive antibodies and hyperstimulation of T cells and macrophages could contribute to this widespread granulomatous reaction.

Morphologic changes including formation of vessel wall fibrinoid necrosis in evolution of cutaneous polyarteritis nodosa (C-PAN) have not been described in detail. Therefore, an investigation of 18 skin biopsy specimens from 14 cases of clinicohistologically proven C-PAN was performed. The results indicated that evolution of arteritis can be classified into 4 stages. Coexistence of different stages was common (50%) in the same or different specimens. The initial (acute) stage shows endothelial loss and fibrin thrombi with neutrophil infiltration without obvious internal elastic lamina disruption and medial fibrinoid necrosis. The second (subacute) stage has mixed cell infiltrates showing a unique intimal target-like fibrinoid necrosis with fibrinoid leakage extending through the disrupted sites of the internal elastic lamina to the media. The third (reparative) stage shows intimal fibroblastic proliferation and perivascular neovascularization with predominant infiltrates of histiocytes and lymphocytes. The final (healed) stage has minimal cellular inflammation with occlusive intimal thickening. Overall, our results show that there are 4 stages in the evolution of C-PAN. The initial change occurs in the intima, and the role of internal elastic lamina in preventing intimal fibrinoid necrosis from discharging into the media may account for the development of target-like fibrinoid necrosis in C-PAN.

Posterior reversible encephalopathy is a distinctive syndrome associated with different diseases and drugs. Disease evolution is frequently favorable with an adequate treatment. Damage typically involves parietal-occipital lobes even if a more anterior diffusion has been described. Here, we report the case of a woman affected by Polyarteritis Nodosa, who suddenly complicated with decreased consciousness and seizures, during an acute hypertensive state. MRI imaging showed increased T2 and FLAIR signal in posterior regions. Her neurological evolution was positive, according to arterial pressure correction, although the systemic vasculitis was still ongoing, hence affecting final prognosis.

Localized gastrointestinal vasculitis is a rare condition. It may be observed as an incidental unexpected pathologic finding at the time of biopsy of an abdominal mass or may present as unexplained abdominal pain with or without unexplained lower gastrointestinal bleeding. In this report we describe a new case of localized polyarteritis nodosa with involvement of peripancreatic middle-sized blood vessels. A literature review of cases of localized gastrointestinal vasculitis was also conducted. A major point of concern is whether a single organ vasculitis of the gastrointestinal tract is actually a localized gastrointestinal vasculitis or simply an initial manifestation of a more severe systemic vasculitis. Due to this, in cases of localized gastrointestinal vasculitis a complete evaluation of the patient to exclude the presence of a systemic a potentially threatening systemic vasculitis is required.

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.

Background/aims: Polyarteritis nodosa (PN) has been classified into polyarteritis (PA) and microscopic polyarteritis (MA) histologically. To clarify of the characteristics of upper gastrointestinal bleeding lesions in PN, we investigated the patients of PN with rapidly progressive glomerulonephritis (RPGN) presenting with upper gastrointestinal bleeding., Methodology: The subjects of this study were 21 patients of PN with RPGN (PA: 11, MA: 10) who presented with upper gastrointestinal bleeding. The bleeding lesions and their locations were examined endoscopically in the study subjects, and the relationship of the bleeding to the severity of renal failure, the necessity of hemodialysis (HD), presence/ absence of H. pylori infection and the gender of the patients were analyzed., Results: The bleeding lesions were endoscopically identified as esophageal ulcers in 2 cases, gastric ulcers in 15 cases and duodenal ulcers in 4 cases, respectively. In 10 of the 15 cases with gastric ulcers, the ulcer assumed the form of Dieulafoy's lesions affecting the gastric body, and the underlying disease was PA in all the 10 cases. In the remaining 5 cases of gastric ulcers and 2 cases of esophageal ulcer with underlying MA or 4 cases of duodenal ulcers, in whom assumed the bleeding form of oozing from the marginal zone of ulcers. In all of the 4 cases of duodenal ulcers, and the 1 case with underlying PA and the other cases with MA, no correlation was found between the onset of the upper gastrointestinal bleeding and the severity of renal failure or the necessity for HD, presence of H. pylori infection, or the gender of the patients., Conclusions: Dieulafoy's lesions are the most frequent sources of upper gastrointestinal bleeding in cases of PA.