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polyarteritis nodosa diagnosis

Ashok Kumar, Anshul Goel, Mehul Lapsiwala
Polyarteritis nodosa is a medium artery vasculitis that can cause fatal complications. It commonly follows an acute monophasic course that may remit after treatment or cause serious morbidity or death. A 24-year-old patient described here had many vasculitic episodes in the past 16 years causing infarction of different organs. The last episode was most serious which caused mononeuritis multiplex, digital gangrene, bowel gangrene and subsequent perforations. There was strong clinical suspicion of this disease right from the beginning...
October 2016: Oxford Medical Case Reports
Z Birsin Özçakar, Suat Fitöz, Adalet Elçin Yıldız, Fatoş Yalçınkaya
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...
October 5, 2016: Clinical Rheumatology
Justine Perrin, Julien Carvelli, Bertrand Gondouin, Laurent Daniel, Megan Fraisse, Céline Gaudon, Fouad Bouzana, Henri Vacher-Coponat, Julie Moussi-Francès, Bertrand Dussol, Noémie Jourde-Chiche
Renal involvement of systemic vasculitides is an emergency in nephrology. Although it has become very rare, the diagnosis of polyarteritis nodosa must be considered in some patients. A 70-year-old patient, previously healthy, presented with acute renal failure and malignant hypertension and abundant albuminuria. Subcutaneous nodule, orchitis and mononeuritis appeared subsequently. The search for auto-immunity or viral infection was negative. Markers of thrombotic microangiopathy, present initially, resolved after blood pressure control...
September 24, 2016: Néphrologie & Thérapeutique
Ibrahim Hatemi, Gulen Hatemi, Aykut F Çelik
PURPOSE OF REVIEW: Gastrointestinal system can be involved in primary and secondary vasculitides. The recent data regarding the pathophysiology, clinical findings, diagnosis, management, and outcome of gastrointestinal involvement in different types of vasculitis are reviewed. RECENT FINDINGS: Diagnosis of gastrointestinal vasculitis may be difficult and relies mostly on imaging, because biopsy samples are hard to obtain and superficial mucosal biopsies have a low yield...
September 28, 2016: Current Opinion in Rheumatology
S Lowell Kahn, Jonathan McClain, Jeffrey L Kaufman
A 66-year-old man, with an abnormal porta hepatis, consistent with tumor or inflammation, developed massive bleeding from one of numerous hepatic artery aneurysms, and coil embolization achieved control of bleeding. He died of subsequent multisystem organ failure, and the most likely diagnosis was either polyarteritis nodosa or segmental arterial mediolysis. Although the dual hepatic blood supply allows a degree of arterial embolization, this case demonstrates the risks associated with large territory hepatic arterial embolization in the presence of hemodynamic instability...
October 2016: Vascular and Endovascular Surgery
Fatma Alibaz-Oner, Matthew J Koster, Cynthia S Crowson, Ashima Makol, Steven R Ytterberg, Carlo Salvarani, Eric L Matteson, Kenneth J Warrington
OBJECTIVES: Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis of medium-sized visceral vessels. However, cutaneous arteritis (CA) and gastrointestinal (GI) vasculitis are forms of single organ vasculitis having indistinguishable histopathological findings from PAN. The aim of this study was to evaluate and compare the clinical characteristics, treatment, and outcomes of patients with systemic PAN, CA and GI vasculitis. METHODS: Retrospective cohorts were assembled of patients with PAN, CA and GI vasculitis between 1980 and 2014...
August 26, 2016: Arthritis Care & Research
Christiana Georgiou, Miltiadis Krokidis, Natasha Elworthy, Stavros Dimopoulos
INTRODUCTION: Polyarteritis Nodosa (PAN) is a systemic vasculitis affecting small and medium size arteries resulting in microaneurysms formation. Bilateral renal aneurysm rupture is a rare and life threatening complication. Although uncommon, PAN has been associated with chronic myelomonocytic leukaemia (CMML). PRESENTATION OF CASE: We report a case of a 77-year-old female with a known CMML, presented to hospital with abdominal pain. Left initially and right renal microaneurym ruptures were shown in CT scan within one-week interval...
2016: International Journal of Surgery Case Reports
Yan Rong Yong, Narayan Lath, Foong Koon Cheah, Yuen Li Ng
This pictorial essay presents cases of non-atherosclerotic coronary artery encasement which were encountered in our institution, including malignant lymphoma, Erdheim-Chester disease, immunoglobulin G4 (IgG4)-related disease and Polyarteritis Nodosa. These conditions usually have multisystemic involvement which aid in the diagnosis. Awareness of these uncommon disorders and their ancillary findings can facilitate early, accurate diagnosis and appropriate management.
September 2016: Journal of Cardiovascular Computed Tomography
Negiin Pourafshar, Eric Sobel, Mark Segal
Polyarteritis nodosa (PAN) is a systemic necrotising vasculitis that affects medium-sized and small-sized arteries. The spectrum of disease ranges from involving a single organ to polyvisceral failure. We report a case of a 75-year-old male presented with solely hypertension and mild renal function impairment as a consequence of diffuse renal necrotising vasculitis with occlusive but non-stenotic lesions associated with PAN. The overall prognosis of PAN has been improved in recent decades, primarily reflecting early diagnosis and more effective treatments...
2016: BMJ Case Reports
Eun Jeong Gong, Do Hoon Kim, Joo Hyun Chun, Ji Yong Ahn, Kwi-Sook Choi, Kee Wook Jung, Jeong Hoon Lee, Kee Don Choi, Ho June Song, Gin Hyug Lee, Hwoon-Yong Jung, Jin Ho Kim, In Hye Song, Yong-Gil Kim
BACKGROUND/AIMS: Gastrointestinal involvement in vasculitis may result in life-threatening complications. However, its variable clinical presentations and endoscopic features, and the rarity of the disease, often result in delayed diagnosis. METHODS: Clinical characteristics, endoscopic features, and histopathological findings were reviewed from medical records. RESULTS: Of 6,477 patients with vasculitis, 148 were diagnosed as primary vasculitis with upper gastrointestinal involvement...
July 15, 2016: Gut and Liver
Yunus Ugan, Atalay Doğru, Hüseyin Şencan, Mehmet Şahin, Şevket Ercan Tunç
Familial Mediterranean fever (FMF) is an autoinflammatory disorder with autosomal recessive inheritance, characterized by recurrent fever and episodes of serositis. The condition is known to be caused by mutations in the MEFV (Mediterranean FeVer) gene, located in the short arm of chromosome 16. While more than 310 sequence variants in the MEFV gene have been described to date, the diagnosis is still established clinically. FMF may be accompanied by sacroiliitis and various forms of vasculitis. The most common forms of associated vasculitis are Henoch-Schonlein purpura and polyarteritis nodosa (PAN)...
2016: Case Reports in Medicine
Paresh Jobanputra
A 60-year-old man presented with cutaneous vasculitis, leucopenia and psoriasis. He was treated initially with ciclosporin A. On withdrawal of ciclosporin, due to inadequate improvement of cutaneous vasculitis, he developed psoriatic arthritis. Worsening neutropenia and pancytopenia, believed to be immune mediated, developed. He was treated with prednisolone, methotrexate and adalimumab but developed pneumocystis pneumonia. Leucocyte levels improved markedly with granulocyte colony-stimulating factor (G-CSF)...
April 2016: Oxford Medical Case Reports
C Anquetil, C Stavris, N Chanson, M Lambert, E Hachulla, D Launay, P Y Hatron
INTRODUCTION: The vascular disorders in systemic lupus erythematosus (SLE) result from various mechanisms and presentations (inflammatory disease or vasculitis, atherosclerosis). CASE REPORT: We report on a 34-year-old man with cutaneous, articular, neurological and nephrologic SLE. He presented with catastrophic haemorrhage on microaneurysm rupture of the left hepatic artery. After blood transfusions and immunosuppressive treatments, his condition improves. CONCLUSION: Uncommon complication in SLE patients, digestive vasculitis with microaneurysms may occur as in polyarteritis nodosa...
March 29, 2016: La Revue de Médecine Interne
Youying Mao, Lei Yin, Hui Xia, Hua Huang, Zhengyu Zhou, Tongxin Chen, Wei Zhou
OBJECTIVES: To determine the incidence and clinical features of paediatric primary vasculitis in patients from one centre in Eastern China. METHODS: Medical records of paediatric patients diagnosed with primary vasculitis between January 1999 and December 2013 were retrospectively reviewed. For Henoch-Schönlein purpura (HSP) and Kawasaki disease (KD), patients included in the analyses had data available for the previous 5 years. RESULTS: In total, 1896 patients were identified, of whom 1100 had HSP, 760 had KD, 23 had Takayasu arteritis, five had polyarteritis nodosa, four had cutaneous polyarteritis, three had Behçet's disease and one had microscopic polyangiitis...
June 2016: Journal of International Medical Research
Alessandro Broccoli, Letizia Gandolfi, Cinzia Pellegrini, Claudio Agostinelli, Lisa Argnani, Pier Luigi Zinzani
BACKGROUND: Autoimmune manifestations may occur in patients with hairy cell leukemia (HCL), and some rare cases of polyarteritis nodosa and leukocytoclastic vasculitis have been reported. However, data regarding the treatment of these cutaneous manifestations are lacking, given the rarity of the concomitance of HCL and vasculitic syndromes. CASE PRESENTATION: We present a 37-year-old man with paraneoplastic leukocytoclastic vasculitis complicating newly diagnosed HCL...
March 16, 2016: Tumori
Katsuhisa Ogata
Vasculitic peripheral neuropathy (VPN) occurs due to ischemic changes of peripheral nerves, resulting from a deficit of vascular blood supply due to damaged vasa nervorum leading to vasculitis. VPN usually manifests as sensorimotor or sensory disturbances accompanied by pain, presenting as a type of multiple mononeuropathy, with a scattered distribution in distal limbs. VPN may also present as a mononeuropathy, distal symmetric polyneuropathy, plexopathy, or radiculopathy. The rapidity of VPN is variable, ranging from days to months, with symptoms occasionally changing with the appearance of new lesions...
March 2016: Brain and Nerve, Shinkei Kenkyū No Shinpo
Shalini Thapar Laroia, Suman Lata
Chronic hepatitis caused by hepatitis B virus (HBV) is an endemic disease in India. It is associated with extrahepatic manifestations like polyarteritis nodosa (PAN) which is a vasculitis like disorder, presenting in subacute or chronic phase; involving visceral and systemic vessels. It should always be considered as a possible etiology of hypertension in an underlying setting of hepatitis B. We describe a 56-year-male patient with a history of chronic HBV who presented to the outpatient clinic with history of recent onset hypertension and suspected liver disease...
March 16, 2016: World Journal of Clinical Cases
Armando De Virgilio, Antonio Greco, Giuseppe Magliulo, Andrea Gallo, Giovanni Ruoppolo, Michela Conte, Salvatore Martellucci, Marco de Vincentiis
Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis predominantly targeting medium-sized arteries. PAN is a rare form of vasculitis, and the precise frequency of this disease is difficult to determine. The major environmental factor associated with PAN is HBV infection. The pathogenesis of "idiopathic PAN" remains enigmatic, although the clinical responses to immunosuppressive therapy support the concept that immunological mechanisms play an active pathogenic role. The spectrum of disease ranges from involving a single organ to polyvisceral failure...
June 2016: Autoimmunity Reviews
Yusuhn Kang, Sung Hwan Hong, Hye Jin Yoo, Ja-Young Choi, Jin Kyun Park, Jina Park, Heung Sik Kang
OBJECTIVE: The purpose of this study is to describe MRI findings of muscle involvement in patients with polyarteritis nodosa (PAN), with an emphasis on the contrast enhancement characteristics. MATERIALS AND METHODS: MRI studies of eight patients with PAN were retrospectively reviewed (four male and four female patients; mean age at presentation, 34.3 years; age range, 7-64 years). MR images were evaluated focusing on the following features: affected structures (muscle, fascia, or periosteum), lesion signal intensity and characteristics on T1-and T2-weighted images, and contrast enhancement patterns...
February 2016: AJR. American Journal of Roentgenology
Emma Louise Barlow, Owen Seddon, Brendan Healy
Polyarteritis nodosa (PAN) is a rare, severe form of vasculitis affecting medium-sized vessels. It manifests as a multisystem syndrome, and may be associated with hepatitis B virus-associated PAN (HBV-PAN) although the incidence of this is declining with better vaccination strategies and awareness of bloodborne virus screening. We report a case in which a patient displayed many classical features of the disease, occurring separately over a period of months and leading to contact with various medical specialties...
2016: BMJ Case Reports
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