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IgA vasculitis

Tim B van der Houwen, P Martin van Hagen, Wilhemina M C Timmermans, Sophinus J W Bartol, King H Lam, Jasper H Kappen, Menno C van Zelm, Jan A M van Laar
OBJECTIVES: Behçet's disease (BD), an auto-inflammatory vasculitis with oro-genital ulcerations, skin lesions and uveitis, is regarded as T cell mediated. A successful trial with rituximab suggests an additive role for B cells in the pathogenesis. Therefore, we studied B cell abnormalities in BD patients and the effect of TNF-blocking therapy. METHODS: B cells in blood (n = 36) and tissue (n = 6) of BD patients were analysed with flow cytometry and/or immunohistochemistry and compared with healthy controls (n = 22)...
October 15, 2016: Rheumatology
C Cossart, G Le Moal, M Garcia, E Frouin, E Hainaut-Wierzbicka, F Roblot
BACKGROUND: Visceral leishmaniasis is not normally expressed in skin. Herein, we describe the case of an HIV-positive patient who developed two unusual skin manifestations during an episode of visceral leishmaniasis. PATIENTS AND METHODS: A 48-year-old female patient consulted initially for infiltrated purpura of all four limbs. Skin biopsy revealed leukocytoclastic vasculitis with Leishman-Donovan bodies. Laboratory tests showed medullary, splenic, gastric and colic involvement, suggesting systemic disease, and enabling visceral leishmaniasis to be diagnosed...
October 11, 2016: Annales de Dermatologie et de Vénéréologie
Zaw Min, Raquel R Garcia, Monika Murillo, Jeffrey M Uchin, Nitin Bhanot
Intravenous vancomycin is a widely used antibiotics, but it causes different types of cutaneous hypersensitivity reactions, ranging from maculopapular rash, red-man syndrome, drug rash with eosinophilia and systemic symptoms, IgA bullous dermatosis, leukocytoclastic vasculitis, Stevens-Johnsons syndrome, to IgE-mediated anaphylaxis. We report an elderly patient with the end-stage renal disease presented with diffuse palpable purpura while receiving IV vancomycin therapy for methicillin-resistant Staphylococcus aureus septicemia...
September 25, 2016: Journal of Infection and Chemotherapy: Official Journal of the Japan Society of Chemotherapy
Mayuko Kawabe, Izumi Yamamoto, Yo Komatsuzaki, Takafumi Yamakawa, Haruki Katsumata, Ai Katsuma, Aki Mafune, Yasuyuki Nakada, Akimitsu Kobayashi, Yudo Tanno, Ichiro Ohkido, Nobuo Tsuboi, Keitaro Yokoyama, Shigeru Horita, Masayoshi Okumi, Hideki Ishida, Hiroyasu Yamamoto, Takashi Yokoo, Kazunari Tanabe
BACKGROUND: IgA vasculitis, a rare condition resulting in end-stage renal disease, is a small-vessel vasculitis that affects the kidney in 49-83 % of adults. The reported recurrence rate of IgA vasculitis in renal transplant recipients is 11.5-60 %, leading to graft loss in 0-50 % of these patients. However, limited data are available on recurrence and graft loss after renal transplantation. METHODS: We evaluated renal transplant recipients seen from 1987 to 2015 at the Jikei University School of Medicine and the Department of Urology, Tokyo Women's Medical University...
September 27, 2016: Clinical and Experimental Nephrology
Neil Gaskill, Bruce Guido, Cynthia Mago
Henoch-Schonlein purpura is an immunoglobulin A (IgA)-immune complex mediated leukocytoclastic vasculitis that classically manifests with palpable purpura, abdominal pain, arthritis, and hematuria or proteinuria. The condition is much more predominant in children (90% of cases) and commonly follows an upper respiratory infection. We present a case of recurrent Henoch-Schonlein purpura (HSP) complicated by nephritis in an adult female initially categorized as IgA nephropathy (IgAN). We review the pathophysiologic basis of HSP nephritis as the variant of HSP accompanied by renal involvement and its pathogenetic commonality with IgA nephropathy...
2016: Dermatology Online Journal
Yusuke Okubo, Kotaro Nochioka, Hiroshi Sakakibara, Hiroshi Hataya, Toshiro Terakawa, Marcia Testa, Robert P Sundel
At the national level, IgA vasculitis-related hospitalizations among children in the USA are scarce. Furthermore, nationwide epidemiology and hospital course of children with IgA vasculitis have not been fully described in the USA, and disparities by race/ethnicity remain unknown. Hospital discharge records of patients aged 19 years or younger were obtained from the 2003, 2006, 2009, and 2012 Kids' Inpatient Database, and they were weighted to estimate the annual hospitalization rates with respect to age, gender, and race/ethnicity in the USA...
November 2016: Clinical Rheumatology
E F Johnson, D A Wetter, J S Lehman, J L Hand, D M R Davis, M M Tollefson
BACKGROUND: Leukocytoclastic vasculitis (LCV) in children is a complex group of conditions. OBJECTIVES: This study presents the demographics, clinical features, direct immunofluorescence (DIF) results and suspected aetiologies of 56 biopsy-confirmed cases of leukocytoclastic vasculitis in children. METHODS: Retrospective review of 56 children seen at Mayo Clinic in Rochester, Minnesota, from 1993 to 2013 with clinical features and cutaneous biopsy consistent with LCV...
August 31, 2016: Journal of the European Academy of Dermatology and Venereology: JEADV
María C De Paoli, Dino Moretti, Carlos M Scolari Pasinato, Martín G Buncuga
The Henoch-Schönlein purpura (HSP) is a small vessel vasculitis with IgA immune complex deposition. The presentation in adults is rare and severe. Reported cases of HSP in patients infected with HIV are scarce. Neutrophil cytoplasmic antibodies (ANCA) are commonly found in other systemic vasculitis, but rarely in HSP and even more unusual the perinuclear pattern. Beside small vessel vasculitis, positivity of ANCA can be detected in a number of different pathological conditions in association with infectious processes, including HIV, or cocaine use, and especially the pattern of ANCA-p, associated with drugs, inflammatory bowel or autoimmune diseases...
2016: Medicina
Guru Prasad Patnala, Anila P Sunandini, Rama Rayavarapu, Padmasri Somala Yandapalli
Erythema elevatum diutinum (EED) is a rare form of vasculitis characterized clinically by red-violet brown papules, plaques, and nodules mainly involving the extensor surfaces; histologically by leukocytoclastic vasculitis in early lesions, and fibrosis and cholesterolosis in late lesions. EED has been associated with many systemic disorders including infections, autoimmune disorders, and both benign and malignant hematological disorders. As it is a rare form of vasculitis and only 250 cases reported till date, we report a case of EED in association with IgA monoclonal gammopathy with partial response to dapsone treatment...
July 2016: Indian Dermatology Online Journal
Caroline Maris Takatu, Antonio Pedro Ribeiro Heringer, Valéria Aoki, Neusa Yuriko Sakai Valente, Paula Cristina de Faria Sanchez, Jozélio Freire de Carvalho, Paulo Ricardo Criado
This is the largest direct immunofluorescence (DIF) analysis of patients with histology-proven cutaneous leukocytoclastic vasculitis (LCV). To establish the correlation of deposition of immune complexes at the blood vessel walls with underlying causes and prognosis of LCV, we performed a retrospective study from January 2007 to December 2014. The patients are followed at the Department of Dermatology, Hospital Das Clínicas da Faculdade de Medicina da Universidade de São Paulo, a tertiary hospital at São Paulo, Brazil...
August 16, 2016: Immunologic Research
Tiina Lipitsä, Anita Naukkarinen, Joel Laitala, Ilkka T Harvima
The complement factor C3 and chymase released from tryptase(+), chymase(+) mast cells may be involved in the pathogenesis of cutaneous leukocytoclastic vasculitis. To study whether mast cells contain C3 in vasculitis and whether chymase interacts with C3, cryosections from vasculitis biopsies were double-stained histochemically for C3c in tryptase(+) mast cells, as well as for chymase and vessel wall C3c, or they were treated with 5 µg/ml rh-chymase for 24 h followed by immunofluorescence (IF) analysis of C3c, IgG, IgM and IgA...
October 2016: Archives of Dermatological Research
Izabela Grabska-Kobylecka, Dariusz Nowak, Anna Wlodarczyk, Piotr Bialasiewicz
Henoch-Schonlein purpura (HSP) is a generalized form of IgA-mediated vasculitis that usually spares pulmonary circulation. Nevertheless, it is conceivable that subclinical changes at the HSP onset may lead to lung impairment in the long term. Therefore, we decided to follow a group of HSP patients for 4 years to monitor changes in pulmonary function. A group of 11 children and adolescents diagnosed with HSP without apparent pulmonary involvement was subjected to pulmonary function tests (PFTs), i.e., spirometry, body plethysmography, and diffusing capacity for CO (DLCO); these tests were repeated after 48 months...
November 2016: Clinical Rheumatology
Roberta Fenoglio, Carla Naretto, Bruno Basolo, Giacomo Quattrocchio, Michela Ferro, Paola Mesiano, Giulietta Beltrame, Dario Roccatello
Henoch-Schonlein purpura, also called IgA-vasculitis, is a systemic small vessels vasculitis with immunoglobulin A1-dominant immune deposits. The optimal treatment remains controversial. Because IgA-vasculitis is characterized by leukocyte infiltration of the blood vessel walls along with immunoglobulin A deposition, and because glucocorticosteroids inhibit inflammatory processes, early administration of glucocorticosteroids has been postulated to be effective, but this indication remains controversial. Immunosuppressive agents (azathioprine, cyclophosphamide, cyclosporine, mycophenolate) have been used in combination with glucocorticosteroids without definitive evidence of effectiveness...
July 23, 2016: Immunologic Research
Michelle M O'Shaughnessy, Sai Liu, Maria E Montez-Rath, Colin R Lenihan, Richard A Lafayette, Wolfgang C Winkelmayer
Differences in kidney transplantation outcomes across GN subtypes have rarely been studied. From the US Renal Data System, we identified all adult (≥18 years) first kidney transplant recipients (1996-2011) with ESRD attributed to one of six GN subtypes or two comparator kidney diseases. We computed hazard ratios (HRs) for death, all-cause allograft failure, and allograft failure excluding death as a cause (competing risks framework) using Cox proportional hazards regression. Among the 32,131 patients with GN studied, patients with IgA nephropathy (IgAN) had the lowest mortality rates and patients with IgAN or vasculitis had the lowest allograft failure rates...
July 18, 2016: Journal of the American Society of Nephrology: JASN
Kibriya Fidan, Ipek Isik Gonul, Bahar Büyükkaragöz, Emel Isiyel, Turgay Arinsoy, Oguz Soylemezoglu
Renal biopsy is the gold standard method for determining the diagnosis, treatment, and prognosis in children with renal disease. This study aims to evaluate the histopathological features of pediatric renal biopsies obtained from the national nephrology registry in the last two decades. Data recorded in the Turkish Society of Nephrology Registry System (TSNRS) in 1991 as well as in between 2001 and 2010 were analyzed. A total of 3892 biopsies were recorded; with the least number in 1991 (total 103 biopsies from 17 centers) and the highest number in 2008 (total 654 biopsies from 23 centers)...
September 2016: Renal Failure
Sveta Janković, Marijana Nikolić, Aleksandra Simović, Ana Vujić
INTRODUCTION: Henoch-Schönlein purpura (HSP) is a small blood vessel vasculitis, which usually manifests during childhood. The exact cause of the disease is unknown. CASE REPORT: We reported a 14-year-old girl who had been admitted to our clinic due to the appearance of red macules on her extremities and face, vomiting, and pain in the abdomen and joints. The patient was initially diagnosed with Henoch-Schönlein purpura. At the end of the fourth week of illness, larvae of Strongyloides stercoralis were detected in stool samples...
May 2016: Vojnosanitetski Pregled. Military-medical and Pharmaceutical Review
Chaowapong Jarasvaraparn, Chatmanee Lertudomphonwanit, Kwanchai Pirojsakul, Suchin Worawichawong, Napat Angkathunyakul, Suporn Treepongkaruna
Henoch-Schönlein purpura (HSP) is a multi-organ vasculitis involving skin, joints, gastrointestinal tract, and kidneys. The present study reported a 5-year-old boy presenting with intense abdominal pain, bloody diarrhea, and protein-losing enteropathy. Investigations for infectious enteritis were negative. Esophagogastroduodenoscopy showed swelling and erythematous mucosa with hemorrhagic spots at duodenal bulb to the third part of duodenum. Histopathology of endoscopic biopsies revealed non-specific duodenitis...
April 2016: Journal of the Medical Association of Thailand, Chotmaihet Thangphaet
Mary H Foster
Animal models are indispensable for the study of glomerulonephritis, a group of diseases that destroy kidneys but for which specific therapies do not yet exist. Novel interventions are urgently needed, but their rational design requires suitable in vivo platforms to identify and test new candidates. Animal models can recreate the complex immunologic microenvironments that foster human autoimmunity and nephritis and provide access to tissue compartments not readily examined in patients. Study of rat Heymann nephritis identified fundamental disease mechanisms that ultimately revolutionized our understanding of human membranous nephropathy...
September 1, 2016: American Journal of Physiology. Renal Physiology
Camelia Cojocariu, Carol Stanciu, Codrina Ancuta, Mihai Danciu, Stefan Chiriac, Anca Trifan
Immunoglobulin A (IgA) vasculitis, formerly called Henoch-Schönlein purpura, is a leukocytoclastic type of vasculitis affecting small vessels with a deposition of immune IgA complexes, clinically characterized by the classic tetrad of nonthrombocytopenic palpable purpura, arthralgia (or arthritis), and gastrointestinal and renal involvement. Although the cause of the disease remains unknown, immune complexes of IgA and unidentified antigens seem to play a central pathogenic role. The diagnosis is easily established in the presence of purpura, but may be challenging in its absence, especially when colicky abdominal pain precedes the cutaneous lesions...
June 2016: Journal of Gastrointestinal and Liver Diseases: JGLD
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
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