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Polyarteritis nodosa (PAN) is a rarely seen vasculitis that often affects small-sized and medium-sized arteries. PAN can present with diverse clinical features depending on the organs involved, including potential cardiac involvement. Typical cardiac manifestations of PAN in the paediatric age group include pericarditis, valvular heart disease and coronary artery aneurysms. In contrast to PAN, Kawasaki disease (KD) is often associated with coronary artery abnormalities. Herein, we report a case of a young boy presented with KD-like features, including a coronary artery aneurysm confirmed by an echocardiogram (ECHO) and eventually diagnosed as PAN. The patient was treated with steroids, methotrexate and tocilizumab with improvement of the symptoms. Repeated ECHO revealed the resolution of the coronary artery aneurysms. Our paper highlights a rare presentation of PAN mimicking KD presentation. Physicians should consider PAN in chronic or severe courses of KD or coronary artery aneurysms., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Introduction and Importance: Spontaneous coronary artery dissection is a rare cause of acute coronary syndromes not related to atherosclerosis. It involves the sudden tearing of the coronary artery wall, separating the inner intimal lining from the outer vessel wall, typically affecting a single coronary vessel. In 20% of cases, the cause of spontaneous coronary artery dissection is unknown. The other cases often occur in pregnant or postpartum women or in individuals with conditions such as connective tissue disorders or vasculitis., Case Presentation: Here, we describe a case of a 69-year-old African female presenting with non-ST-segment elevation myocardial infarction. Coronary angiography revealed an unusual triple-vessel spontaneous coronary artery dissection affecting peripheral segments, with further investigations suggesting polyarteritis nodosa., Conclusion: While triple-vessel spontaneous coronary artery dissection and polyarteritis nodosa (PAN) are individually rare, their coexistence is exceptionally uncommon and presents diagnostic and therapeutic challenges. Clinicians should be alert to vasculitic causes in patients with spontaneous coronary artery dissection, especially with atypical clinical features., (© 2024. The Author(s).)

Abstract: A 61-year-old man with a medical history of human immunodeficiency virus well controlled on antiretroviral therapy presented for distal sensorimotor symptoms, fatigue, and recurrent fevers. Erythrocyte sedimentation rate and C-reactive protein were both elevated. Antineutrophilic cytoplasmic antibody and antinuclear antibodies were negative. Neurologic imaging workup was unremarkable. 18 F-FDG PET/CT, which was crucial for diagnosis, demonstrated pathological tracer activity throughout the medium-sized vessels with sparing of the aorta. In view of presentation, comorbidities, and imaging findings, polyarteritis nodosa was diagnosed. The patient was treated appropriately with steroids and cyclophosphamide with significant symptomatic improvement., Competing Interests: Conflicts of interest and sources of funding: none declared., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Competing Interests: Declaration of conflicting interestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: Photoacoustic microscopy is expected to have clinical applications as a noninvasive and three-dimensional (3D) method of observing intradermal structures., Objective: Investigate the applicability of a photoacoustic microscope equipped with two types of pulsed lasers that can simultaneously recognize hemoglobin and melanin., Methods: 16 skin lesions including erythema, pigmented lesions, vitiligo and purpura, were analyzed to visualize 3D structure of melanin granule distribution and dermal blood vessels. 13 cases of livedo racemosa in cutaneous polyarteritis nodosa (cPN) were further analyzed to visualize the 3D structure of dermal blood vessels in detail. Vascular structure was also analyzed in the biopsy specimens obtained from tender indurated erythema of cPN by CD34 immunostaining., Results: Hemoglobin-recognition signal clearly visualized the 3D structure of dermal blood vessels and melanin-recognition signal was consistently reduced in vitiligo. In livedo racemosa, the hemoglobin-recognition signal revealed a relatively thick and large reticular structure in the deeper layers that became denser and finer toward the upper layers. The numerical analysis revealed that the number of dermal blood vessels was 1.29-fold higher (p<0.05) in the deeper region of the lesion than that of normal skin. The CD34 immunohistochemical analysis in tender indurated erythema revealed an increased number of dermal vessels compared with normal skin in 88.9% (8/9) of the cases, suggesting that vascular network remodeling had occurred in cPN., Conclusion: The photoacoustic system has an advantage in noninvasively detecting dermal blood vessel structures that are difficult to recognize by two-dimensional histopathology specimen examination and is worth evaluating in various skin diseases., Competing Interests: Conflicts of Interest None declared., (Copyright © 2024 The Authors. Published by Elsevier B.V. All rights reserved.)

Background: Polyarteritis Nodosa (PAN) is a systemic vasculitis (SV) historically thought to spare the coronary arteries. Coronary angiography and contemporary imaging reveal coronary stenosis and dilation, which are associated with significant morbidity and mortality. Coronary arteries in PAN are burdened with accelerated atherosclerosis from generalized inflammation adding to an inherent arteritic process. Traditional atherosclerotic risk factors fail to approximate risk. Few reports document coronary pathology and optimal therapy has been guarded., Methods: Database publication query of English literature from 1990-2022., Results: Severity of coronary involvement eludes laboratory monitoring, but coronary disease associates with several clinical symptoms. Framingham risk factors inadequately approximate disease burden. Separating atherosclerosis from arteritis requires advanced angiographic methods. Therapy includes anticoagulation, immunosuppression and revascularization. PCI has been the mainstay, though stenting is confounded by vagarious alteration in luminal diameter and reports of neointimization soon after placement., Conclusions: When graft selection avoids the vascular territory of SV's, CABG offers definitive therapy. We have contributed report of a novel CABG configuration in addition to reviewing, updating and discussing the literature. Accumulating evidence suggests discrete clinical symptoms warrant suspicion for coronary involvement., (© 2024. The Author(s).)

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Objective: 18F-fluorodeoxyglucose PET/CT (FDG-PET/CT) is widely used in patients with large vessel vasculitis. The benefits of FDG-PET/CT in PAN has only ever been assessed in three case reports. Our aim was to describe FDG-PET/CT findings in 10 patients with newly diagnosed PAN., Methods: This was a retrospective study of patients with PAN who underwent FDG-PET/CT at diagnosis between 2017 and 2020. The FDG-PET/CT data were analysed retrospectively., Results: Ten patients were included: nine men and one woman with a median age of 67 years (range 43-78). PAN was diagnosed according to ACR criteria in nine patients and histologically in one. All patients had high CRP levels (median 223 mg/l). The main FDG-PET/CT abnormality was increased tracer uptake in the muscles, particularly in the connective tissue (perimysium, epimysium) (n = 7), and in linear (n = 5) or focal (n = 2) patterns. Increased FDG uptake in large-diameter vessels was observed in four patients, in the humeral (n = 4), femoral (n = 1) and common interosseous arteries (n = 1). Nine patients had bone marrow FDG uptake and six had splenic FDG uptake. Three had synovitis and three had lymph node uptake. One patient had subcutaneous FDG uptake with a 'leopard skin' appearance., Conclusions: FDG-PET/CT seems to be a useful non-invasive imaging technique for diagnosing PAN, particularly in patients with non-specific systemic features. Tracer uptake in muscular connective tissue seems to be a recurrent sign in patients with PAN and may be pathognomonic., (© The Author(s) 2021. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Hypertrophic pachymeningitis (HP) is a rare neurological disorder with focal or diffuse thickening of the dura mater, which usually causes headache, cranial neuropathies, seizures, and motor or sensory impairments. The development of HP is attributed to an immune-mediated mechanism, and some autoimmune diseases have been implicated in the development of HP. Herein, we describe the case of a 73-year-old woman with persistent headache ascribable to HP, which developed approximately 3 years after a diagnosis of polyarteritis nodosa (PAN). She was treated with high-dose corticosteroid and cyclophosphamide that resulted in immediate disappearance of headache and improved radiological findings of thickened dura mater. In addition, she was subsequently administered methotrexate, ultimately resulting in maintenance of remission and regular reduction of prednisolone. In our review of published English articles, only two cases of HP in patients with PAN have been reported to date, suggesting that HP is a rare complication in PAN. However, it should be recognized that HP may develop as a neurological involvement related to PAN., (© 2021. International League of Associations for Rheumatology (ILAR).)

Purpose: To perform a descriptive analysis of individuals with suspected Polyarteritis nodosa (PAN) referred for second opinion imaging consultation., Methods: A retrospective observational cohort study was performed at a single institution. A consecutive sample was performed of individuals who underwent a second opinion CT or MR angiography subspecialty radiologist consultation between January 2008 and September 2019 for suspected abdominal medium vessel vasculitis. Demographic, clinical, and imaging data were collected. Clinical and imaging findings were reported for PAN, small vessel vasculitis, and "non-vasculitis" groups. Agreement and diagnostic accuracy between final clinical and second opinion imaging diagnoses for PAN were determined. Two-tailed t-tests with a significant p-value < 0.05 were utilized., Results: Of the 58 participants, 9 were clinically diagnosed with PAN, 11 with small vessel vasculitis (including lupus, IgA, and ANCA-associated vasculitis), and 38 with non-vasculitis diagnoses. The non-vasculitis group included 15 SAM, 3 FMD, and 1 SAM-FMD spectrum diagnoses. Higher C-reactive protein level (51 vs 17, p = 0.04) and superior mesenteric artery involvement (56% vs 21%, p = 0.04) were more common in PAN than non-vasculitis diagnoses, while arterial dissection (40% vs 0%, p = 0.02) and celiac vasculature involvement (53% vs 0%, p = 0.003) were more common in the non-vasculitis group. There was 88% agreement (51/58; Cohen's kappa 0.56); sensitivity was 67% [95%-confidence interval (CI) 30-93%] and specificity was 92% (95%-CI 80-98%)., Conclusion: Isolated celiac artery involvement and arterial dissection were more common in non-inflammatory vasculopathies than PAN. Our findings highlight the need for multidisciplinary collaboration and awareness of the diverse findings of abdominal vasculopathies., (© 2021. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Objective: To provide evidence-based recommendations and expert guidance for the management of systemic polyarteritis nodosa (PAN)., Methods: Twenty-one clinical questions regarding diagnostic testing, treatment, and management were developed in the population, intervention, comparator, and outcome (PICO) format for systemic, non-hepatitis B-related PAN. Systematic literature reviews were conducted for each PICO question. The Grading of Recommendations Assessment, Development and Evaluation methodology was used to assess the quality of evidence and formulate recommendations. Each recommendation required ≥70% consensus among the Voting Panel., Results: We present 16 recommendations and 1 ungraded position statement for PAN. Most recommendations were graded as conditional due to the paucity of evidence. These recommendations support early treatment of severe PAN with cyclophosphamide and glucocorticoids, limiting toxicity through minimizing long-term exposure to both treatments, and the use of imaging and tissue biopsy for disease diagnosis. These recommendations endorse minimizing risk to the patient by using established therapy at disease onset and identify new areas where adjunctive therapy may be warranted., Conclusion: These recommendations provide guidance regarding diagnostic strategies, use of pharmacologic agents, and imaging for patients with PAN., (© 2021 American College of Rheumatology. This article has been contributed to by US Government employees and their work is in the public domain in the USA.)

Systemic vasculitides are multisystem blood vessel disorders, which are defined by the size of the vessel predominantly affected, namely small, medium, or large vessels. The term "large vessel" relates to the aorta and its major branches; "medium vessel" refers to the main visceral arteries and veins and their initial branches. The most common causes of large-vessel vasculitis are giant cell arteritis and Takayasu arteritis, and those of medium-vessel arteritis are polyarteritis nodosa and Kawasaki disease. However, there is some overlap, and arteries of any size can potentially be involved in any of the 3 main categories of dominant vessel involvement. In addition to multisystem vasculitides, other forms of vasculitis have been defined, including single-organ vasculitis (eg, isolated aortitis). Prompt identification of vasculitides is important because they are associated with an increased risk of mortality. Left undiagnosed or mismanaged, these conditions may result in serious adverse outcomes that might otherwise have been avoided or minimized. The ethnic and regional differences in the incidence, prevalence, and clinical characteristics of patients with vasculitis should be recognized. Because the clinical presentation of vasculitis is highly variable, the cardiovascular clinician must have a high index of suspicion to establish a reliable and prompt diagnosis. This article reviews the pathophysiology, epidemiology, diagnostic strategies, and management of vasculitis.

Cardiac vasculitis is recognized as a heterogeneous disease process with a wide spectrum of manifestations including pericarditis, myocarditis, valvular heart disease and less frequently, coronary artery vasculitis (CAV). CAV encompasses an emerging field of diseases which differ from conventional atherosclerotic disease and have a proclivity for the younger population groups. CAV portends multiple complications including the development of coronary artery aneurysms, coronary stenotic lesions, and thrombosis, all which may result in acute coronary syndromes. There are several aetiologies for CAV; with Kawasaki's disease, Takayasu's arteritis, Polyarteritis Nodosa, and Giant-Cell Arteritis more frequently described clinically, and in literature. There is a growing role for multi-modality imaging in assisting the diagnostic process; including transthoracic echocardiography, cardiac magnetic resonance imaging, computed tomography coronary angiography, fluorodeoxyglucose-positron emission tomography and conventional coronary angiogram with intravascular ultrasound. Whilst the treatment paradigms fundamentally vary between different aetiologies, there are overlaps with pharmacological regimes in immunosuppressive agents and anti-platelet therapies. Interventional and surgical management are is a consideration in select populations groups, within a multi-disciplinary context. Further large-scale studies are required to better appropriately outline management protocols in this niche population.

Vasculitides are a complex group of diseases sharing the defining feature of inflamed vessel walls. Vasculitides can be classified depending on the size of the predominantly affected vessels. Modern cross-sectional imaging methods have become a cornerstone in the diagnosis of vasculitis and may help in narrowing down differential diagnoses. This review presents the most important imaging modalities and typical findings in large and medium size vasculitis, implementing current imaging recommendations., Competing Interests: Disclosure The authors have nothing to disclose., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Background/purpose: To assess EDI-OCT (enhanced depth imaging optical coherence tomography) of choroid for inflammatory signs in children with polyarteritis nodosa (PAN) and adenosine deaminase-2 deficiency (DADA-2)., Methods: In this cross-sectional study conducted between June 2017 and September 2018, we evaluated children diagnosed with PAN (n = 11) and DADA-2 (n = 4) and an age- and sex-matched control group (n = 15). Demographic and laboratory data were retrospectively analyzed from patient charts. Disease activity was assessed using the pediatric vasculitis activity score (PVAS). Choroidal images were obtained with spectral domain-OCT to measure choroidal thickness (ChT) at 5 points (750 and 1500 μm from the foveal center in the temporal and nasal quadrants and beneath the fovea), and to calculate the total subfoveal choroidal area (TCA), luminal area (LA), stromal area (SA), and the choroidal vascularity index (CVI)., Results: The median (min-max) age was 8 (4-16) years in PAN patients, 6 (5-16) years in DADA-2 patients and 8 (8-10) years in control group at the OCT visit (p = 0.214). The ChT at 3 points and the TCA, LA, and SA were higher in children with both PAN and DADA-2 patients compared to those of the control group (p < 0.0001, p = 0.049, p = 0.007, p = 0.007, p = 0.006, p = 0.033, respectively). The CVI was similar in both groups. No association was observed between the OCT findings, PVAS, and the erythrocyte sedimentation rate, and serum leukocyte and C-reactive protein levels., Conclusion: Similar CVI scores were obtained from PAN and DADA2 patients under treatment and from healthy controls. Increased subfoveal ChT without any other signs of ocular involvement may suggest choroidal thickening as a sign of mild subclinical inflammation.

A 59-year-old man presented with fever and lower extremity myalgia. Laboratory studies revealed elevated C-reactive protein. F-FDG PET/CT demonstrated FDG uptake not only in the brachial arteries, femoral arteries, and their main ramifications, which were equivalent to small- to medium-sized arteries but also in the kidneys. Angiography revealed a renal aneurysm, confirming the diagnosis of polyarteritis nodosa. The increased FDG uptake in the vessels and kidneys resolved after 6 months of glucocorticoid treatment.

Vasculitis is a systemic disease characterized by immune-mediated injury of blood vessels. Current treatments for vasculitis, such as glucocorticoids and alkylating agents, are associated with significant side effects. Furthermore, the management of both small and large vessel vasculitis is challenging because of a lack of robust markers of disease activity. Recent research has advanced our understanding of the pathogenesis of both small and large vessel vasculitis, and this has led to the development of novel biologic therapies capable of targeting key cytokine and cellular effectors of the inflammatory cascade. In parallel, a diverse range of imaging modalities with the potential to monitor vessel inflammation are emerging. Continued expansion of combined structural and molecular imaging using positron emission tomography with computed tomography or magnetic resonance imaging may soon provide reliable longitudinal tracking of vascular inflammation. In addition, the emergence of radiotracers able to assess macrophage activation and immune checkpoint activity represents an exciting new frontier in imaging vascular inflammation. In the near future, these advances will allow more precise imaging of disease activity enabling clinicians to offer more targeted and individualized patient management.

Polyarteritis nodosa is characterized by presence of aneurysms in the renal, hepatic and mesenteric vasculature, and less often by vascular abnormalities in the intracranial compartment. Spinal subarachnoid hemorrhage is a rare phenomenon that can be associated with inflammatory vasculopathies such as polyarteritis nodosa, but the link between aneurysm formation and spinal subarachnoid hemorrhage in polyarteritis nodosa is unclear. We describe a case of a patient with polyarteritis nodosa and spinal subarachnoid hemorrhage following rupture of an aneurysm of the anterior spinal artery. Following operative washout and decompression of the subarachnoid hemorrhage, spinal digital subtraction angiography was performed and revealed intimal contour irregularities, stenotic changes, and multiple small aneurysms in renal, hepatic, and bronchial arteries and some proximal spinal arteries, and, most notably, a pseudoaneurysm of the anterior spinal artery supplied directly by the artery of Adamkiewicz. Polyarteritis nodosa was subsequently diagnosed in light of these findings. Though previous cases have noted spinal subarachnoid hemorrhage in of the context of polyarteritis nodosa, we found no previously documented case of a definitive aneurysm of the anterior spinal artery in a case of polyarteritis nodosa documented on angiography. This case highlights the potential importance of monitoring for aneurysms of the spinal vasculature in cases of polyarteritis nodosa and in screening for vasculitides in cases of spinal subarachnoid hemorrhage. Future studies are needed to describe patterns of the specific anatomic localization and incidence of spinal artery aneurysms in polyarteritis nodosa., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: Polyarteritis nodosa is a disease that presents with necrotizing vasculitis in small and medium-sized arteries. It may occur in various organs, but approximately half of cases have gastrointestinal involvement. Prognosis is not favorable once organ dysfunction begins as evidenced by gastrointestinal symptoms; thus, treatment with steroids should be promptly initiated. We report the case of a patient who presented with necrosis of the small intestine, which was pathologically diagnosed as polyarteritis nodosa and treated successfully with steroids., Case Presentation: An 18-year-old Japanese woman reported a sudden onset of abdominal pain and vomiting that led her to visit our emergency department, where she was evaluated by a physician. On physical examination, tenderness to palpation in the upper umbilical region was noted, and diagnostic imaging with computed tomography showed emphysema of the wall of her small intestine. She was diagnosed as having necrosis of the small intestine requiring urgent surgery. No strangulations were noted intraoperatively but approximately 20 cm of her small intestine was necrotized. The surrounding arteries were examined and no palpable pulse was observed; therefore, segmentectomy of the necrotized regions was performed. Pathological findings revealed active vasculitis with fibrinoid necrosis, as well as destruction, fibrogenesis, and luminal stenosis of the elastic lamina found in the muscular arteries. A diagnosis of polyarteritis nodosa was confirmed as the cause of the necrosis of her small intestine. No recurrence of polyarteritis nodosa symptoms was observed when she was administered 40 mg of prednisolone daily., Conclusion: In cases of idiopathic intestinal necrosis or perforation, systemic diseases such as polyarteritis nodosa should be considered in the differential diagnosis.

Competing Interests: Disclosure The authors report no conflicts of interest in this work., (© Georg Thieme Verlag KG Stuttgart · New York.)

Background: Myalgia is a common but unspecific set of symptoms that may be caused by orthopedic, neurological and internal medical conditions, often resulting in a diagnostic challenge. Muscular polyarteritis nodosa (PAN) is a rare differential diagnosis of myalgia with elevated serological inflammatory markers., Objective: Based on three clinical cases and the literature this review describes the essential clinical and diagnostic features of muscular PAN., Results: Muscular PAN typically presents with immobilizing myalgia confined to the lower limbs and elevated serological inflammatory markers but often normal creatine kinase (CK) levels. Contrast-enhanced magnetic resonance imaging of the affected muscles, which can often mimic myositis, and muscle biopsy provide the relevant histological findings that lead to the diagnosis of a vasculitis., Conclusion: With respect to own experiences and the reviewed literature, muscular PAN should be considered as a possible diagnosis in cases of myalgia with elevated inflammatory markers but normal CK levels and a lack of further symptoms typical for vasculitis.

Polyarteritis nodosa (PAN) is a rare systemic vasculitis affecting multiple organs. Current standard treatment includes the use of glucocorticoids and cyclophosphamide. Unfortunately, some patients do not respond to this treatment and other therapeutic options are needed. We present a case of a young male with refractory PAN and ongoing biopsy evidence of active vasculitis despite optimal standard therapies, who was successfully treated with interleukin-6 antagonist, tocilizumab. A 24-year-old male presented with severe immobilizing polyneuropathy and myalgias. Clinical features included fasciitis, tenosynovitis, early signs of polyneuropathy, and panniculitis, which were largely refractory to the standard therapies. The previous unsuccessful treatments included high-dose glucocorticoids, methotrexate, cyclophosphamide, rituximab, anakinra, and intravenous immunoglobulins. Magnetic resonance imaging showed signs of myositis, with muscle biopsy confirming the diagnosis of PAN. Rapid clinical improvement and sustained remission occurred after interleukin-6 inhibition with tocilizumab at increased dose of 800 mg every 4 weeks. The used search strategy identified 20 publications of which four articles were included for the further analysis. In total, we report the clinical outcome of five PAN cases from the literature and the present one. The present case and the systematic review of literature suggest that tocilizumab is a possible treatment option for, otherwise, refractory hepatitis B virus negative PAN. Randomized-controlled trials are required to evaluate the safety and efficacy of tocilizumab in PAN.

Spontaneous retroperitoneal hematoma is a rare clinical entity that is most commonly caused by renal tumors and vascular disease. In this article, we present a case of spontaneous retroperitoneal hemorrhage caused by polyarteritis nodosa in a patient who presented with severe left flank pain. He underwent computed tomography angiography of his abdomen that showed left retroperitoneal hematoma, which was followed by arteriogram that showed multiple bilateral renal artery aneurysms with active extravasation, findings consistent with polyarteritis nodosa. The patient underwent successful coiling of the bleeding vessel that secured the bleeding and was started on high-dose prednisone, which resulted in resolution of his symptoms.

A possible association between strongyloidiasis and systemic vasculitis is rarely reported in the literature. We report the case of a patient with severe strongyloidiasis and an angiographic finding consistent with polyarteritis nodosa. Diagnosis of strongyloidiasis was made by finding of larvae and adult parasites in samples of the upper gastrointestinal tract mucosa and stool. The patient was treated with albendazole, ivermectin and corticosteroid withdrawal. This therapy led to the resolution of symptoms, with repeated stool samples negative for S. stercoralis. However, the clinical course was complicated with pulmonary tuberculosis. Despite tuberculostatic therapy and supportive measures, a lethal outcome occurred. The report is followed by a focused review of the available literature on the association of strongyloidiasis and systemic vasculitis.

Vasculitis, characterized by inflammation of vessel walls, is comprised of heterogeneous clinicopathological entities, and thus poses a diagnostic challenge. The most widely used approach for classifying vasculitides is based on the International Chapel Hill Consensus Conference (CHCC) nomenclature system. Based on the recently revised CHCC 2012, we propose computed tomography (CT) features of vasculitides and a differential diagnosis based on location and morphological characteristics. Finally, vasculitis mimics should be differentiated, because erroneous application of immunosuppressive drugs on vasculitis mimics may be ineffective, even deteriorating. This article presents the utility of CT in the diagnosis and differential diagnosis of vasculitides.

Polyarteritis nodosa (PAN) is a necrotising vasculitis that involves medium and small vessels. PAN generally presents with constitutional, cutaneous, neurological, renal and gastrointestinal manifestations. However, PAN initially involving a single organ/system is uncommon. Here, we present a 42-year-old man who was hospitalised because of severe right upper quadrant abdominal pain that started 2 months before. Physical examination was remarkable for right upper quadrant abdominal tenderness. Abdominopelvic CT showed lymphadenopathy but no hepatic, gallbladder, pancreatic, intestinal or renal abnormalities. Abdominal angiography showed multiple small aneurysms located in the jejunal and hepatic arteries characteristic of PAN. He had a prompt and remarkable response to high-dose corticosteroids and oral cyclophosphamide. Our case, together with other reports, suggests that PAN should be considered in patients presenting with right upper abdominal pain. Timely diagnosis and treatment reduce the overall morbidity and mortality of the disease., (2017 BMJ Publishing Group Ltd.)

There is limited number of publications about the use of non-invasive imaging modalities in the diagnosis of childhood polyarteritis nodosa (cPAN). The aim of this study was to present the clinical and imaging findings of the patients with cPAN who were diagnosed with non-invasive imaging techniques. Files of patients who had been diagnosed as cPAN in our department from 2005 to 2015 were reviewed, retrospectively. Demographic, clinical, laboratory, and imaging findings of the patients were evaluated. Nine patients (8M, 1F; age at disease onset: 12.5 years (7-16)) had been diagnosed as cPAN in our clinic with non-invasive imaging techniques within the last 10 years. Abdominal pain, fever, fatigue, and myalgia were the most frequent complaints. Doppler ultrasonography (US) was used in the diagnosis of seven patients and computed tomography (CT) angiography was done in four patients. Duration between admission to our center and diagnosis was median of 5 days (8 h-10 days), including four patients who were diagnosed within 24 h of admission. Approximately 80 % of our patients with cPAN had MEFV gene mutations and 90 % had elevated anti-streptolysin O levels. All of them had the involvement of the gastrointestinal tract. Hepatic and cystic arterial involvements were detected in seven and six patients, respectively. This report included the largest cPAN series that were diagnosed with non-invasive imaging modalities. We suggest that non-invasive modalities, especially Doppler US, should be considered in first line approach in the diagnosis of these patients, particularly in children.