6,892 results match your criteria Polyarteritis Nodosa


Identification of novel Adenosine Deaminase 2 gene variants and varied clinical phenotype in pediatric vasculitis.

Arthritis Rheumatol 2019 Apr 22. Epub 2019 Apr 22.

BC Children's Hospital Research Institute, BC Children's Hospital, Vancouver, BC, Canada.

Objectives: Individuals with deficiency of adenosine deaminase 2 (DADA2), a recently recognized autosomal recessive disease, present with various systemic vascular and inflammatory manifestations, often with early-onset age, or recurrent strokes. Their clinical features and histological findings overlap with those of childhood onset polyarteritis nodosa (PAN), a primary "idiopathic" systemic vasculitis. Despite similar clinical presentation, individuals with DADA2 may respond better to biologic therapy than traditional immunosuppression. Read More

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http://dx.doi.org/10.1002/art.40913DOI Listing

Infliximab for the treatment of refractory polyarteritis nodosa.

Clin Rheumatol 2019 Apr 10. Epub 2019 Apr 10.

Rheumatology Unit, Bnai-Zion Medical Center, Haifa, Israel.

Polyarteritis nodosa (PAN) is a necrotizing vasculitis predominantly affecting medium and small size arteries. Cyclophosphamide, a drug with narrow therapeutic range and poor safety profile, constitutes the treatment of choice for PAN vasculitis with major organ involvement. To describe our clinical experience in treating refractory PAN with infliximab (a TNF inhibitor), a drug with good tolerability and better safety profile than cyclophosphamide. Read More

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http://dx.doi.org/10.1007/s10067-019-04474-9DOI Listing
April 2019
1 Read

Teaching NeuroImages: Lacunar stroke and polyarteritis nodosa: Consider ADA2 deficiency (DADA2).

Neurology 2019 Apr;92(15):e1801-e1802

From the Department of Neurology, Division of Child Neurology (T.d.S.G., J.A.d.P.), Department of Radiology-INRAD, Division of Neuroradiology (C.A.P.F.A., L.T.L.), and Fleury Group (C.A.P.F.A.), Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, Brazil.

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http://dx.doi.org/10.1212/WNL.0000000000007270DOI Listing

Polyarteritis Nodosa Neurologic Manifestations.

Neurol Clin 2019 May 16;37(2):345-357. Epub 2019 Mar 16.

Vasculitides and Scleroderma, Department of Internal Medicine, Referral Center for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, Université Paris Descartes, 27, rue Fg Saint-Jacques, Paris 75679 Cedex 14, France. Electronic address:

Polyarteritis nodosa (PAN) is a necrotizing vasculitis affecting medium-sized vessels whose main manifestations are weight loss, fever, peripheral neuropathy, renal, musculoskeletal, gastrointestinal tract and/or cutaneous involvement(s), hypertension and/or cardiac failure. Peripheral neuropathy is one of the most frequent and earliest symptoms, affecting 50% to 75% of PAN patients. Central nervous system involvement affects only 2% to 10% of PAN patients, often late during the disease course. Read More

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http://dx.doi.org/10.1016/j.ncl.2019.01.007DOI Listing
May 2019
1 Read

Extracutaneous involvement of pyoderma gangrenosum.

Arch Dermatol Res 2019 Mar 29. Epub 2019 Mar 29.

Department of Dermatology and OHSU Wound and Hyperbaric Medicine, Oregon Health and Science University, 3303 SW Bond Ave Center for Health and Healing Building 1, Suite 16, Portland, OR, 97239, USA.

Pyoderma Gangrenosum (PG) is an inflammatory neutrophilic dermatosis (ND) associated with underlying chronic inflammation and/or malignancy. Diagnosis remains to be challenging as a gold standard diagnostic test is lacking. Initial manifestations may include papules, vesicles, or pustules that subsequently develop into ulceration with features of undermining and violaceous borders. Read More

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http://dx.doi.org/10.1007/s00403-019-01912-1DOI Listing
March 2019
1 Read

Polyarteritis Nodosa in a Cat with Involvement of the Central and Peripheral Nervous Systems.

J Comp Pathol 2019 Feb 18;167:6-11. Epub 2018 Dec 18.

Department of Veterinary Sciences, University of Pisa, Viale delle Piagge 2, Italy.

An 18-month-old neutered male domestic shorthair cat was referred with a history of pyrexia, polyuria and polydypsia, and transient episodes of bilateral hindlimb paralysis. Cardiac evaluation revealed severe systemic hypertension and severe concentric hypertrophy of the left ventricle. One month later the cat had a new episode of hindlimb paralysis with recurrent seizures, and died in status epilepticus. Read More

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http://dx.doi.org/10.1016/j.jcpa.2018.11.003DOI Listing
February 2019
4 Reads

Parotitis coexisting with Kawasaki disease in a child: a rare presentation of Kawasaki disease.

Trop Doct 2019 Mar 21:49475519829604. Epub 2019 Mar 21.

3 Associate Professor, Ege University Faculty of Medicine, Department of Pediatrics, General Pediatrics Unit, Ege University, Bornova, Izmir, Turkey.

Kawasaki disease (KD), also known as mucocutaneous lymph node syndrome and infantile polyarteritis nodosa, is known to present in multiple ways. Although inflammatory changes in several systems have been reported in KD, there are few documented child patients presenting with parotitis. We report such a case in a five-year-old. Read More

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http://dx.doi.org/10.1177/0049475519829604DOI Listing
March 2019
4 Reads

Systemic vasculitis involving the breast: a case report and literature review.

Rheumatol Int 2019 Mar 14. Epub 2019 Mar 14.

Department of Rheumatology and Immunology, Peking University Third Hospital, No. 49, North Garden Road, Beijing, 100191, China.

Systemic vasculitis involving the breast is a rare clinical condition and may mimic breast cancer or mastitis clinically or radiographically. Here, we report a case of polyarteritis nodosa (PAN) with breast involvement and perform a literature review of published cases of systemic vasculitis affecting the breast to better understand this disorder. We report a case of PAN affecting the right breast in a young woman. Read More

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http://link.springer.com/10.1007/s00296-019-04279-8
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http://dx.doi.org/10.1007/s00296-019-04279-8DOI Listing
March 2019
4 Reads

Necrosis of the small intestine leading to a diagnosis of polyarteritis nodosa: a case report.

J Med Case Rep 2019 Mar 9;13(1):55. Epub 2019 Mar 9.

Department of Pathology, Saitama Medical University, 38 Morohongou, Moroyama, Iruma-gun, Saitama, 350-0495, Japan.

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. Read More

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http://dx.doi.org/10.1186/s13256-019-2017-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6408775PMC
March 2019
1 Read

[A Case of Polyarteritis Nodosa Diagnosed from Pathological Findings of Refractory Epididymitis].

Hinyokika Kiyo 2018 Dec;64(12):515-518

The Department of Urology, Higashiosaka City Medical Center.

A 54-year-old man was admitted to internal medicine due to unidentified fever persisting for 3 months, and was examined. Then, he was referred to our department for suspected pyelonephritis. Although he was initially being treated for pyelonephritis, right epididymitis occurred during the course of treatment. Read More

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http://dx.doi.org/10.14989/ActaUrolJap_64_12_515DOI Listing
December 2018

Ustekinumab for skin reactions associated with anti-tumor necrosis factor-α agents in patients with inflammatory bowel diseases: A single-center retrospective study.

J Dermatol 2019 Apr 28;46(4):322-327. Epub 2019 Feb 28.

Department of Dermatology, Courlancy Clinic, Rheims, France.

Anti-tumor necrosis factor (TNF)-α agents may induce skin reactions, in particular in patients with inflammatory bowel diseases (IBD). The objective of this study was to determine the efficacy of ustekinumab in these patients. IBD patients facing therapeutic issues because of cutaneous reactions or tolerance issues, consequently treated with ustekinumab in our department, were included. Read More

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http://dx.doi.org/10.1111/1346-8138.14816DOI Listing
April 2019
1 Read

Sudden unexpected death due to coronary thrombosis associated with isolated necrotizing vasculitis in the coronary arteries of a young adult.

Forensic Sci Med Pathol 2019 Feb 27. Epub 2019 Feb 27.

Tokyo Medical Examiner's Office, Tokyo Metropolitan Government, 4-21-18 Otsuka, Bunkyo-ku, Tokyo, 112-0012, Japan.

Coronary arteritis is an uncommon cause of sudden death in non-atherosclerotic coronary diseases, and is mostly associated with systemic vasculitis or systemic autoimmune diseases; therefore, sudden death due to isolated coronary arteritis rarely occurs. The case described in this report is that of a 34-year-old man with no significant personal medical history who died suddenly after presenting with nausea. Postmortem examination revealed a significant infiltration of lymphocytes predominantly on the adventitia and periadventitial tissues of the coronary arteries in the epicardium. Read More

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http://dx.doi.org/10.1007/s12024-019-00099-1DOI Listing
February 2019
1 Read

[Acute kidney injury related to acute vascular disorders].

Rev Prat 2018 Feb;68(2):166-169

Service de néphrologie et transplantation, CHU Henri-Mondor, université Paris-Est Créteil, Créteil, France.

Acute kidney injury related to acute vascular disorders. A broad spectrum of vascular disease characterized by primary vessel involvement may be associated with acute renal failure. These diseases are usually classified according to the size of the injured vessel that include the small vessels (thrombotic microangiopathy, kidney injury due to malignant hypertension, scleroderma renal crisis, and cholesterol crystal embolism disease), the medium vessels (polyarteritis nodosa) or the large size vessels (acute renal infarction). Read More

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February 2018

Cutaneous Polyarteritis Nodosa Treated with Pentoxifylline and Clobetasol Propionate: A Case Report.

Saudi J Med Med Sci 2018 May-Aug;6(2):104-107. Epub 2018 Apr 16.

Department of Dermatology, King Fahd Hospital of the University, Imam Abdulrahman Bin Faisal University, Dammam, Saudi Arabia.

Cutaneous polyarteritis nodosa is a rare type of cutaneous vasculitis. It affects the small- and medium-sized arteries of the dermis and subcutaneous tissue without extracutaneous involvement. The diagnosis is by skin biopsy, and the characteristic finding is a leukocytoclastic vasculitis. Read More

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http://dx.doi.org/10.4103/sjmms.sjmms_148_16DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6196709PMC
April 2018
1 Read

Papillophlebitis as manifestation of polyarteritis nodosa.

Arch Soc Esp Oftalmol 2019 Feb 4. Epub 2019 Feb 4.

Servicio de Cardiología, Complejo Hospitalario Universitario Insular Materno Infantil, Las Palmas de Gran Canaria, Las Palmas, España.

A 35 year-old-man diagnosed with polyarteritis nodosa developed papillophlebitis on his left eye. Throughout the evolution of the disease, he had a macular oedema treated with intravitreal injections of aflibercept, with adequate recovery of visual acuity. He also had episcleritis on the other eye. Read More

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http://dx.doi.org/10.1016/j.oftal.2018.12.007DOI Listing
February 2019

Association of serum high mobility group box 1 levels with disease activity and renal involvement in patients with systemic vasculitis.

Medicine (Baltimore) 2019 Feb;98(6):e14493

Center for Hypertension of People's Hospital of Xinjiang Uygur Autonomous Region, Hypertension Institute of Xinjiang, Urumqi, Xinjiang, China.

High mobility group box 1 (HMGB1) is a kind of proinflammatory mediator that acts as an alarmin when released by dying, injured or activated cells. Previous studies have reported that HMGB1 are closely linked to antineutrophil cytoplasmic antibody-associated vasculitis (AAV). The present study aimed to evaluate whether serum HMGB1 levels were associated with systemic vasculitis (VAs). Read More

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http://dx.doi.org/10.1097/MD.0000000000014493DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6380849PMC
February 2019
10 Reads

Polyarteritis nodosa presenting atypically as appendicitis and pyelonephritis in a single patient.

BMJ Case Rep 2019 Feb 1;12(2). Epub 2019 Feb 1.

Griffith University School of Medicine, Gold Coast, Queensland, Australia.

A 17-year-old man presented to the emergency department 10 days postlaparoscopic appendicectomy with ongoing lower abdominal pain. The pain was associated with fevers, chills, sweating and constipation. There were no other associated symptoms. Read More

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http://dx.doi.org/10.1136/bcr-2018-228072DOI Listing
February 2019
7 Reads

Imaging in small and medium vessel vasculitis.

Int J Rheum Dis 2019 Jan;22 Suppl 1:78-85

Department of Internal Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

Vasculitis includes a group of disorders characterized by inflammation of the vessel wall and classified based on the diameter of the predominantly involved vessels. Granulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis and Henoch-Schonlein purpura are the important entities in the small vessel vasculitis group, while polyarteritis nodosa and Kawasaki disease represent the medium vessel vasculitis group. The clinical manifestations may be non-specific and there may be considerable overlap with the other disorders. Read More

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http://dx.doi.org/10.1111/1756-185X.13390DOI Listing
January 2019
3 Reads

[Muscular polyarteritis nodosa-a case-based review].

Z Rheumatol 2019 Mar;78(2):173-179

Rheumatologie, Klinische Immunologie, Nephrologie, Asklepios Klinik Altona, Paul-Ehrlich-Str. 1, 22763, Hamburg, Deutschland.

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. Read More

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http://dx.doi.org/10.1007/s00393-019-0595-xDOI Listing
March 2019
2 Reads

Androgen deprivation therapy for prostate cancer and the risk of autoimmune diseases.

Prostate Cancer Prostatic Dis 2019 Jan 28. Epub 2019 Jan 28.

Graduate Institute of Life Sciences, National Defense Medical Center, Taipei, Taiwan.

Background: Androgen deprivation therapy (ADT) has been a mainstay of treatment for advanced prostate cancer (PCa), but limited studies have been performed to investigate the association between ADT and autoimmune diseases.

Methods: We conducted a population-based nationwide cohort study of 17,168 patients newly diagnosed with PCa between 1996 and 2013 using the National Health Insurance Research Database (NHIRD) of Taiwan. Cox proportional hazards models with 1:1 propensity score-matched analysis were used to investigate the association between ADT use and the risk of autoimmune diseases. Read More

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http://www.nature.com/articles/s41391-019-0130-9
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http://dx.doi.org/10.1038/s41391-019-0130-9DOI Listing
January 2019
19 Reads

Four distinct clinical phenotypes of vasculitis affecting medium-sized arteries.

Scand J Rheumatol 2019 Jan 23:1-7. Epub 2019 Jan 23.

a Department of Hematology and Rheumatology , Tohoku University Graduate School of Medicine , Sendai , Japan.

Objective: Within the spectrum of polyarteritis nodosa (PAN), cutaneous PAN (cPAN) is further classified into mild cPAN and severe cPAN which presents with ulcers, necrosis, or neuritis. As distinguishing between severe cPAN and systemic PAN can be difficult, this study evaluated the clinical characteristics of patients with necrotizing arteritis of medium-sized arteries.

Methods: Forty-one patients diagnosed with necrotizing arteritis of medium-sized arteries between 2008 and 2017 at our institution were enrolled in this study. Read More

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http://dx.doi.org/10.1080/03009742.2018.1551965DOI Listing
January 2019
2 Reads

Hepatitis C Virus-associated Membranoproliferative Glomerulonephritis Treated with Directly Acting Antiviral Therapy.

Indian J Nephrol 2018 Nov-Dec;28(6):462-464

Department of Nephrology, Institute of Liver and Biliary Sciences, New Delhi, India.

Hepatitis C virus (HCV) infection has been shown to affect kidneys with various histopathological pattern on the kidney biopsy. These commonly include a membranoproliferative glomerulonephritis (MPGN) pattern with mixed cryoglobulinemia (CG), thrombotic microangiopathy, membranous nephropathy, and vasculitis affecting medium and small vessels of the kidneys causing polyarteritis nodosa. It has been rarely associated with MPGN without detectable CG. Read More

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http://dx.doi.org/10.4103/ijn.IJN_235_17DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6309397PMC
January 2019
2 Reads

A 9.5-year-old boy with recurrent neurological manifestations and severe hypertension, treated initially for polyarteritis nodosa, was subsequently diagnosed with adenosine deaminase type 2 deficiency (DADA2) which responded to anti-TNF-α.

Paediatr Int Child Health 2019 Jan 15:1-4. Epub 2019 Jan 15.

a Department of Paediatric Rheumatology, Cerrahpasa Medical School , Istanbul University , Istanbul , Turkey.

A 9.5-year-old boy was referred with a 2-year history of recurrent fever, myalgia, abdominal pain and various neurological manifestations associated with increased acute phase reactants and IgG level. During the recent episode, severe hypertension and right-sided hemiparesis developed and angiography demonstrated irregularities and stenosis in renal and mesenteric artery branches. Read More

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http://dx.doi.org/10.1080/20469047.2018.1559495DOI Listing
January 2019
3 Reads

MPO-ANCA-positive cutaneous polyarteritis nodosa with livedo racemosa arising from immune-deposit-positive necrotizing vasculitis.

G Ital Dermatol Venereol 2019 Jan 9. Epub 2019 Jan 9.

Departments of Rheumatology, Tokyo Metropolitan Geriatric Hospital and Institute of Gerontology, Tokyo, Japan.

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https://www.minervamedica.it/index2.php?show=R23Y9999N00A190
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http://dx.doi.org/10.23736/S0392-0488.18.05956-4DOI Listing
January 2019
14 Reads

Serum lysosomal-associated membrane protein-2 levels are increased in small and medium vessel vasculitis, especially in polyarteritis nodosa.

Clin Exp Rheumatol 2019 Jan 4. Epub 2019 Jan 4.

Centre for Hypertension of People's Hospital of Xinjiang Uygur Autonomous Region; Hypertension Institute of Xinjiang, Urumqi, Xinjiang, China.

Objectives: Lysosomal-associated membrane protein-2 (LAMP-2) is a highly glycosylated type I glycoprotein ex- pressed on the membranes of neutrophils, endothelial cells and other cells, which are closely linked to subsets of systematic vasculitis. The aim of this study was to investigate whether serum LAMP-2 can be used as a biomarker in small and medium vessel vasculitis (SMVV).

Methods: Serum samples from 39 patients with SMVV (including ANCA-associated vasculitis (AAV) and polyarteritis nodosa (PAN)) confirmed by angiography and/or biopsy and 78 healthy controls (HC) were collected. Read More

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January 2019
14 Reads

PET-CT and polyarteritis nodosa-associated artery aneurysms.

QJM 2019 Mar;112(3):219-220

Department of General Internal Medicine, Asahi General Hospital, Asahi, Chiba, Japan.

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http://dx.doi.org/10.1093/qjmed/hcy308DOI Listing
March 2019
4 Reads

Human adenosine deaminase 2 deficiency: A multi-faceted inborn error of immunity.

Immunol Rev 2019 Jan;287(1):62-72

Department of Microbiology and Immunology, Laboratory for Childhood Immunology, KU Leuven, Leuven, Belgium.

Human adenosine deaminase 1 deficiency was described in the 1970s to cause severe combined immunodeficiency. The residual adenosine deaminase activity in these patients was attributed to adenosine deaminase 2. Human adenosine deaminase type 2 deficiency (DADA2), due to biallelic deleterious mutations in the ADA2 gene, is the first described monogenic type of small- and medium-size vessel vasculitis. Read More

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http://dx.doi.org/10.1111/imr.12722DOI Listing
January 2019
12 Reads

Vasculitis in Systemic Autoinflammatory Diseases.

Front Pediatr 2018 3;6:377. Epub 2018 Dec 3.

Division of Pediatric Rheumatology, Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey.

Autoinflammatory diseases (AID) are diseases of the innate immune system, characterized by recurrent episodes of localized or systemic inflammation. Vasculitis may accompany AID. The causes of the association of vasculitis with monogenic AID are still debated. Read More

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http://dx.doi.org/10.3389/fped.2018.00377DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6287042PMC
December 2018
19 Reads

What's new in autoinflammation?

Authors:
Seza Ozen

Pediatr Nephrol 2018 Dec 14. Epub 2018 Dec 14.

Department of Pediatrics, Hacettepe University, Ankara, Turkey.

The pathogenesis of autoinflammatory diseases has shed light on the concept of inflammation in general and on our understanding of the role of the innate immune system. The autoinflammatory diseases have a large spectrum with varying features of inflammation. The most common autoinflammatory diseases are those associated with periodic fevers. Read More

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http://dx.doi.org/10.1007/s00467-018-4155-4DOI Listing
December 2018
9 Reads

"Emergency" Cochlear Implantation in Labyrinthitis Ossificans Secondary to Polyarteritis Nodosa: How to Face a Rare Entity.

J Int Adv Otol 2018 Dec 12. Epub 2018 Dec 12.

Department of Otolaryngology, University of Pavia, IRCCS Policlinico "S. Matteo" Foundation, Pavia, Italy.

Polyarteritis nodosa (PAN) is a systemic vasculitis affecting the small- and medium-sized arteries that may present with hearing impairment. In rare cases, PAN may be associated with progressive labyrinthitis ossificans (LO), an otologic emergency requiring early cochlear implantation (CI) to restore hearing before the complete, irreversible cochlear ossification. We report the first case in the literature of a patient affected by PAN with bilateral sudden sensorineural hearing loss and rapid LO who underwent "emergency" bilateral simultaneous CI. Read More

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http://dx.doi.org/10.5152/iao.2018.5463DOI Listing
December 2018
6 Reads

Case Report: Unexpected Benefit of Echocardiography in Childhood Polyarteritis Nodosa.

Klin Padiatr 2019 Mar 11;231(2):96-98. Epub 2018 Dec 11.

General University Hospital, Paediatric Rheumatology and Autoinflammatory Diseases Unit, Praha, Czech Republic.

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http://www.thieme-connect.de/DOI/DOI?10.1055/a-0802-8950
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http://dx.doi.org/10.1055/a-0802-8950DOI Listing
March 2019
17 Reads

European consensus-based recommendations for the diagnosis and treatment of rare paediatric vasculitides - the SHARE initiative.

Rheumatology (Oxford) 2019 Apr;58(4):656-671

Department of Paediatric Rheumatology, Alder Hey Children's Hospital, Liverpool.

Objectives: The European initiative Single Hub and Access point for paediatric Rheumatology in Europe (SHARE) aimed to optimize care for children with rheumatic diseases. Systemic vasculitides are very rare in children. Consequently, despite recent advances, paediatric-specific information is sparse. Read More

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https://academic.oup.com/rheumatology/advance-article/doi/10
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http://dx.doi.org/10.1093/rheumatology/key322DOI Listing
April 2019
30 Reads

Painful leg rash.

Indian J Dermatol Venereol Leprol 2019 Jan-Feb;85(1):129

Department of Dermatology, Changi General Hospital, Singapore.

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http://www.ijdvl.com/preprintarticle.asp?id=246743
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http://dx.doi.org/10.4103/ijdvl.IJDVL_388_18DOI Listing
April 2019
3 Reads

Polyarteritis nodosa in case of familial Mediterranean fever.

Turk J Pediatr 2018 ;60(3):326-330

Division of Pediatric Nephrology, Department of Pediatrics, Marmara University Faculty of Medicine, İstanbul, Turkey.

Gökçe İ, Altuntaş Ü, Filinte D, Alpay H. Polyarteritis nodosa in case of familial Mediterranean fever. Turk J Pediatr 2018; 60: 326-330. Read More

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http://dx.doi.org/10.24953/turkjped.2018.03.016DOI Listing
February 2019
4 Reads

Pulmonary manifestations of large, medium, and variable vessel vasculitis.

Respir Med 2018 Dec 10;145:182-191. Epub 2018 Nov 10.

University of Texas Southwestern Medical Center, Department of Pulmonary and Critical Care Medicine, United States.

The hallmark of vasculitis is autoimmune inflammation of blood vessels and surrounding tissues, resulting in an array of constitutional symptoms and organ damage. The lung is commonly targeted in the more familiar ANCA-associated small vessel vasculitidies, but large and medium vessel vasculitides, including Takayasu arteritis, giant cell arteritis, polyarteritis nodosa, Behcet's disease, and necrotizing sarcoid granulomatosis, may also feature prominent pulmonary involvement. Pulmonary manifestations of these conditions include pulmonary arterial aneurysms, pulmonary hypertension, diffuse alveolar hemorrhage, pulmonary nodules, and parenchymal infiltrates. Read More

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http://dx.doi.org/10.1016/j.rmed.2018.11.003DOI Listing
December 2018
3 Reads

Conditions and Factors Associated With Spontaneous Coronary Artery Dissection (from a National Population-Based Cohort Study).

Am J Cardiol 2019 Jan 30;123(2):249-253. Epub 2018 Oct 30.

Brigham and Women's Hospital Heart & Vascular Center, Harvard Medical School, Boston, Massachusetts. Electronic address:

The pathophysiology of spontaneous coronary artery dissection (SCAD) is heterogeneous, associated with systemic arteriopathies and inflammatory diseases, and often compounded by environmental precipitants, genetics, or stressors. However, the frequency of these associated conditions with SCAD on a population level remains unknown. Therefore, the objective of this analysis was to evaluate heterogeneous phenotypes of SCAD in the United States using data from the Nationwide Inpatient Sample collected from January 1, 2004, to September 31, 2015. Read More

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http://dx.doi.org/10.1016/j.amjcard.2018.10.012DOI Listing
January 2019
16 Reads
3.280 Impact Factor

Tocilizumab treatment in refractory polyarteritis nodosa: a case report and review of the literature.

Rheumatol Int 2019 Feb 21;39(2):337-344. Epub 2018 Nov 21.

Asklepios Klinik Altona, Rheumatologie, Klinische Immunologie, Nephrologie, Paul-Ehrlich-Straße 1, 22763, Hamburg, Germany.

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. Read More

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http://dx.doi.org/10.1007/s00296-018-4210-2DOI Listing
February 2019
22 Reads

Polyarteritis nodosa: lessons from 25 years of experience.

Clin Exp Rheumatol 2018 Oct 30. Epub 2018 Oct 30.

Department of Paediatric Rheumatology, Hacettepe University Faculty of Medicine, Ankara, and Hacettepe University Vasculitis Center, Ankara, Turkey.

Objectives: Polyarteritis nodosa is a necrotising vasculitis of predominantly medium size vessels. The present study aimed to summarise the characteristics of PAN patients, and also analyse the trend of decreasing PAN frequency in the last 25 years.

Methods: PAN patients followed up between 1990 and 2015 were included. Read More

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October 2018
17 Reads

Immunoglobulin G4-related disease with recurrent uveitis and kidney tumor mimicking childhood polyarteritis nodosa: a rare case report.

Acta Reumatol Port 2018 Jul-Sep;43(3):226-229

Instituto da Criança, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brasil.

Introduction: Immunoglobulin G4-related disease (IgG4-RD) is an condition rarely reported in children. Additionally, IgG4-RD may rarely mimic vasculitis in adults and may infrequently present with uveitis. In our service, 6,198 patients were followed-up and only one (0. Read More

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November 2018
5 Reads

Diffuse Alveolar Hemorrhage and Pulmonary Vasculitides: Histopathologic Findings.

Semin Respir Crit Care Med 2018 08 7;39(4):425-433. Epub 2018 Nov 7.

Department of Pathology and Laboratory Medicine, David Geffen School of Medicine at UCLA, Los Angeles, California.

Vasculitides are a heterogeneous group of disorders in which inflammation of blood vessel walls is present at least some time during the course of the disease. Vasculitides can affect any caliber or type of vessel in many anatomic sites; however, the disease can alter more than just vasculature. Given the diversity of vasculitides, in 2012, a revised classification system was proposed to categorize vasculitides by the type of vessel involved including size, function, and structural attributes. Read More

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http://www.thieme-connect.de/DOI/DOI?10.1055/s-0038-1669412
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http://dx.doi.org/10.1055/s-0038-1669412DOI Listing
August 2018
19 Reads

An update on the nomenclature for cutaneous vasculitis.

Curr Opin Rheumatol 2019 Jan;31(1):46-52

Section of Dermatology, Department of Surgery and Translational Medicine, University of Florence, Florence, Italy.

Purpose Of Review: Cutaneous vasculitis reflects a spectrum ranging from skin limited to severe systemic forms. To date, there is still no generally acknowledged nomenclature for cutaneous vasculitis. This review aims to summarize the recent advances in the nomenclature of cutaneous vasculitis. Read More

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http://dx.doi.org/10.1097/BOR.0000000000000563DOI Listing
January 2019
10 Reads

Immune-Mediated Systemic Vasculitis as the Proposed Cause of Sudden-Onset Sensorineural Hearing Loss following Lassa Virus Exposure in Cynomolgus Macaques.

MBio 2018 10 30;9(5). Epub 2018 Oct 30.

Office of the Chief Scientists, U.S. Army Medical Research Institute of Infectious Diseases, Fort Detrick, Frederick, Maryland, USA

Lassa virus (LASV) causes a severe, often fatal hemorrhagic disease in regions in Africa where the disease is endemic, and approximately 30% of patients develop sudden-onset sensorineural hearing loss after recovering from acute disease. The causal mechanism of hearing loss in LASV-infected patients remains elusive. Here, we report findings after closely examining the chronic disease experienced by surviving macaques assigned to LASV exposure control groups in two different studies. Read More

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http://dx.doi.org/10.1128/mBio.01896-18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6212830PMC
October 2018
8 Reads

Renal Amyloidosis in Deficiency of Adenosine Deaminase 2: Successful Experience With Canakinumab.

Pediatrics 2018 Nov;142(5)

Departments of Pediatric Rheumatology.

Deficiency of adenosine deaminase 2 (DADA2) is a rare autoinflammatory disease that was firstly described in patients with early-onset strokes, livedo reticularis, and periodic fever resembling polyarteritis nodosa. In reported case series, researchers described highly variable manifestations, including autoimmunity, immunodeficiency, hepatosplenomegaly, pancytopenia, ichthyosiform rash, and arthritis, in patients with DADA2. A thirteen-year-old female patient who was born to consanguineous parents was admitted to our hospital with generalized edema and leg pain. Read More

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http://pediatrics.aappublications.org/lookup/doi/10.1542/ped
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http://dx.doi.org/10.1542/peds.2018-0948DOI Listing
November 2018
17 Reads

Severe strongyloidiasis and systemic vasculitis: comorbidity, association or both? Case-based review.

Rheumatol Int 2018 Dec 23;38(12):2315-2321. Epub 2018 Oct 23.

Clinic of Allergology and Immunology, Clinical Center of Serbia, Belgrade, Serbia.

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. Read More

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http://link.springer.com/10.1007/s00296-018-4178-y
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http://dx.doi.org/10.1007/s00296-018-4178-yDOI Listing
December 2018
18 Reads

Orchitis: An unusual presentation of polyarteritis nodosa.

Indian J Pathol Microbiol 2018 Oct-Dec;61(4):600-603

Department of Pathology, IPGME and R, Kolkata, West Bengal, India.

Polyarteritis nodosa (PAN), a systemic necrotizing vasculitis with multiorgan development, is generally restricted to the medium-sized muscular arteries. The varied initial clinical presentations of PAN can lead to a delayed diagnosis. We present the case of a middle-aged male patient who presented with an acute onset right-sided testicular pain as the initial clinical symptom with ischemic changes on ultrasonogram, thereby requiring orchiectomy. Read More

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http://www.ijpmonline.org/text.asp?2018/61/4/600/242994
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http://dx.doi.org/10.4103/IJPM.IJPM_569_17DOI Listing
February 2019
5 Reads

Autoimmune Kidney Diseases Associated with Chronic Viral Infections.

Rheum Dis Clin North Am 2018 11 7;44(4):675-698. Epub 2018 Sep 7.

Department of Medicine, Division of Nephrology, Massachusetts General Hospital, 55 Fruit Street, GRB 7, Boston, MA 02114, USA. Electronic address:

Autoimmune kidney diseases triggered by viruses are an important cause of kidney disease in patients affected by chronic viral infection. Hepatitis B virus (HBV) infection is associated with membranous nephropathy and polyarteritis nodosa. Hepatitis C virus (HCV) infection is a major cause of cryoglobulinemic glomerulonephritis. Read More

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http://dx.doi.org/10.1016/j.rdc.2018.06.006DOI Listing
November 2018
4 Reads

Renal complications of liver diseases.

Expert Rev Gastroenterol Hepatol 2018 Nov 11;12(11):1135-1142. Epub 2018 Oct 11.

a Service de Néphrologie, Hémodialyse , Aphérèses et Transplantation rénale , Grenoble-Alpes , France.

Introduction: Hepatitis B virus (HBV) and hepatitis C virus (HCV) infections are the major causes of chronic liver disease. HBV and HCV affect nearly 7% of the world's population. Extra-hepatic complications and particularly renal failure have different mechanisms and manifestations. Read More

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http://dx.doi.org/10.1080/17474124.2018.1530984DOI Listing
November 2018
24 Reads