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Neuropathy vasculitis

Alexandre Karras
Cryoglobulins are immunoglobulins that undergo reversible precipitation at low temperatures. They can induce systemic vasculitis, characterized by purpuric cutaneous lesions, arthritis, peripheral neuropathy, hypocomplementemia and glomerular disease. Renal pathology reveals membranoproliferative glomerulonephritis, with particularly intense mesangial cell proliferation and infiltration by macrophages, associated with intracapillary thrombi. This renal disease presents as a nephritic syndrome, with heavy proteinuria, haematuria severe hypertension and rapidly progressive kidney failure that can lead to end-stage renal disease...
March 12, 2018: Néphrologie & Thérapeutique
Sean C Maguire, Christina A Fleming, Gavin O'Brien, Gerald McGreal
BACKGROUND: Lumbar sympthectomy (LS) was traditionally performed for intermittent claudication but is now eclipsed by revascularisation for that indication. However, it retains a role in the management of critical limb ischaemia and other conditions causing lower limb pain with or without ischaemia. We report the role of LS in modern surgical practice when revascularisation and pain management options have been exhausted. METHODS: A medical chart review was performed on all patients who underwent LS in our unit from 2005 to 2016 (inclusive)...
March 7, 2018: Irish Journal of Medical Science
Razek Georges Coussa, Andre Ali-Ridha, Natalia Vila, Rayan Alshareef, John Chen
Purpose: To describe a case of Crohn disease presenting as occlusive vasculitis resulting in a central retinal artery occlusion (CRAO) in one eye and transient ischemic optic neuropathy in the fellow eye. Observations: An 18-year-old patient recently diagnosed with biopsy-proven Crohn disease presented with CRAO OD after a previous episode of transient visual loss OS. Extensive workup was negative for other autoimmune or infectious etiologies. The patient was started on intravenous methylprednisolone for 72 h followed by maintenance dose of azathioprine and oral prednisone...
April 2017: American Journal of Ophthalmology Case Reports
Min K Kang, Rajesh K Gupta, Jayashri Srinivasan
We describe 2 patients presenting with multiplex mononeuritis, associated with skin manifestation, secondary to minocycline-induced vasculitis. One of the cases is associated neither with lupus nor polyarteritis nodosa. An extensive laboratory workup ruled out any possible underlying immunologic disorder. Electrodiagnostic studies were conducted to show axonal neuropathy in patchy and multifocal distribution consistent with multiplex mononeuritis. This diagnosis was confirmed with nerve biopsy. Withdrawing from the offending medication, minocycline, improved the patients' clinical condition and the quantitative serological measures...
March 2018: Journal of Clinical Neuromuscular Disease
Cesare Mazzaro, Luigino Dal Maso, Luca Quartuccio, Michela Ghersetti, Marco Lenzi, Endri Mauro, Milena Bond, Pietro Casarin, Valter Gattei, Ivo Maria Crosato, Salvatore De Vita, Gabriele Pozzato
OBJECTIVES: To investigate the long-term effects and safety of new direct anti-viral agents (DAAs) in patients with hepatitis C virus (HCV)-related mixed cryoglobulinaemia (MC) without renal involvement. METHODS: The study enrolled 22 consecutive patients, 19 received sofosbuvir-based regimen and three patients received other DAAs, individually tailored according to latest guidelines. As of December 2016, the median length of follow-up was 17 months (range 13-21)...
February 13, 2018: Clinical and Experimental Rheumatology
Paul C Yell, Dennis K Burns, Evan G Dittmar, Charles L White, Chunyu Cai
Terminal complement complex deposition in endomysial capillaries detected by a C5b-9 immunostain is considered a diagnostic feature for dermatomyositis. However, we found widespread microvascular C5b-9 reactivity in a substantial subset of muscle biopsies with denervation changes, and in nerve biopsies of peripheral neuropathies, particularly in patients with diabetes. It is unclear whether the presence of C5b-9 deposition signifies active immune-mediated vascular injury that requires immune suppression therapy...
February 20, 2018: Acta Neuropathologica Communications
Sadanandavalli Retnaswami Chandra, Venkata Raviteja Karru, M A Mukheem Mudabbir, Subashree Ramakrishnan, Anitha Mahadevan
Introduction: Immune-mediated peripheral neuropathy is the term applied to a spectrum of peripheral nerve disorders where immune dysregulation plays a role. Therefore, they are treatable. We analyzed the cases seen in the past 3 years by us and evaluated the clinical, laboratory, and outcome parameters in these patients. Patients and Methods: Consecutive patients seen by the authors and diagnosed as immune-mediated neuropathy were analyzed for etiology, pathology, and outcome assessed...
January 2018: Journal of Neurosciences in Rural Practice
Yasunori Minami, Masatoshi Kudo
Cryoglobulins are produced by the over-stimulated immune system of patients with hepatitis C virus (HCV). HCV-induced cryoglobulinemic vasculitis primarily affects small-sized vessels of systemic organs. The most common symptoms are purpura, joint pain, and peripheral neuropathy or Raynaud phenomenon. The blood tests of patients with HCV may exhibit raised erythrocyte sedimentation rate and C-reactive protein levels, positive rheumatoid factor and cryoglobulins, and decreased complement levels. Therapeutic options for HCV-induced cryoglobulinemic vasculitis, include anti-virals, Rituximab, steroids, immunosuppressant drugs, and plasma exchange...
February 2018: Brain and Nerve, Shinkei Kenkyū No Shinpo
Masanori Kurihara, Yu Kurata, Izumi Sugimoto, Yuki Hatanaka, Yasuhisa Sakurai
Proteinase 3 anti-neutrophil cytoplasmic antibody (PR3-ANCA) is reported to be highly specific to vasculitis compared to myeloperoxidase (MPO)-ANCA. We report a case of a 19-year-old woman with chronic inflammatory demyelinating polyneuropathy (CIDP) with high PR3-ANCA positivity. The patient responded well to intravenous immunoglobulin plus oral steroid, and showed no signs of systemic vasculitis during the subsequent 10 months of follow-up. Our present case suggests that CIDP may accompany high PR3-ANCA levels, which should be differentiated from axonal neuropathy due to vasculitis...
March 2017: ENeurologicalSci
Andrea Hinojosa-Azaola, Ariadna Jiménez-González, Natasha Alcocer-Castillejos
The objective of this study is to describe aspects of health-related quality of life (HRQOL) in Mexican patients with antineutrophil cytoplasmic-associated vasculitis (AAV). Cross-sectional comparison study includes patients with established AAV and a comparison group with rheumatoid arthritis (RA), chronic kidney disease (CKD), and healthy subjects. Variables considered were: socio-demographic data, comorbidities, laboratory, disease activity, damage, and Physician's and Patient's Global Assessment (PhGA and PtGA)...
December 15, 2017: Rheumatology International
Rosanna Dammacco
Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease of undefined etiology and with remarkably heterogeneous clinical features. Virtually any organ system can be affected, including the eye. SLE-related eye involvement can be diagnosed in approximately one-third of the patients and is usually indicative of disease activity. An early diagnosis and the adoption of suitable therapeutic measures are necessary to prevent sight-threatening consequences, especially in patients with juvenile SLE. Periocular lesions, such as eyelid involvement and orbital inflammation, are relatively rare and, in case of orbital masses, may require a biopsy control...
December 14, 2017: Clinical and Experimental Medicine
Gülçin Otar Yener, Zahide Ekici Tekin, Neşe Çallı Demirkan, Selçuk Yüksel
Eosinophilic granulomatosis with polyangiitis (EGPA), also known as Churg-Strauss syndrome, is a systemic necrotizing vasculitis of the small and medium vessels. It is primarily associated with respiratory conditions such as asthma and sinusitis as well as eosinophilia, neuropathy, pulmonary infiltrates, and vasculitis. EGPA is extremely rare in the pediatric age group, and respiratory system disorders are usually predominant in EGPA patients. A 14-year-old boy presented with rash and severe extremity pain...
November 30, 2017: Rheumatology International
Michele Iudici, Christian Pagnoux, Pierre Quartier, Matthias Büchler, Ramiro Cevallos, Pascal Cohen, Claire de Moreuil, Philippe Guilpain, Alain Le Quellec, Jacques Serratrice, Benjamin Terrier, Loïc Guillevin, Luc Mouthon, Xavier Puéchal
OBJECTIVE: To investigate differences between childhood-onset ANCA-associated vasculitides (cAAVs) and matched adult-onset controls (aAAVs). METHODS: cAAV clinical pictures at onset and outcomes were compared to a randomly selected sample of aAAV patients from the French Vasculitis Study Group Registry. Cases and controls were matched for AAV (granulomatosis with polyangiitis [GPA], microscopic polyangiitis [MPA] or eosinophilic granulomatosis with polyangiitis [EGPA]), sex and year of enrollment...
February 2018: Autoimmunity Reviews
Harun Arslan, Alpaslan Yavuz, Ayşe Arslan, Abdurrahman Aycan
IgA vasculitis (IgAV) is a leukocytoclastic vasculitis and characterized by involvement of small vessels in skin, gastrointestinal system, joints, kidneys, and less frequently other organs. It is the commonest vasculitis in childhood and etiology is not completely known. Neurological manifestations of IgAV are very rare and usually seen in patients with severe hypertension or as an uncommon feature such as peripheral neuropathy. Posterior reversible encephalopathy syndrome (PRES) is a clinic-radiologic entity characterized with temporary vasogenic edema developing typically in posterior circulation of the brain and has been reported as a rare manifestation of IgAV...
November 14, 2017: Neurologia i Neurochirurgia Polska
Priya S Dhawan, P James B Dyck, Jennifer A Tracy, Elie Naddaf
No abstract text is available yet for this article.
November 14, 2017: Neurology
Mahsa Arzani, Mohammad Ali Sahraian, Hamed Rezaei, Abdorreza Naser Moghadasi
Background: Isolated relapsing optic neuropathy is a recurrent painful optic nerve inflammation without any sign of other demyelinating diseases such as multiple sclerosis (MS) or neuromyelitis optica (NMO) spectrum disorders, and the attacks are purely responsive to steroid therapy. Methods: Recurrent isolated optic neuritis (RION) was diagnosed in patients who presented with at least two disseminating episodes of optic neuritis, and negative clinical, para-clinical, and radiological features of the demyelinating, infiltrative and vasculitis disorders involving optic nerve...
July 6, 2017: Iranian Journal of Neurology
Tanaz A Kermani, Antoine G Sreih, David Cuthbertson, Simon Carette, Gary S Hoffman, Nader A Khalidi, Curry L Koening, Carol A Langford, Carol A McAlear, Paul A Monach, Larry Moreland, Christian Pagnoux, Philip Seo, Kenneth J Warrington, Steven R Ytterberg, Peter A Merkel
Objectives: To evaluate damage and variables associated with damage in GCA. Methods: Patients with GCA enrolled in a prospective, multicentre, longitudinal study were included. Per-protocol assessments were made with the Vasculitis Damage Index and the Large-Vessel Vasculitis Index of Damage. Results: The study included 204 patients: 156 women (76%), mean age at diagnosis 71.3 years (s.d. 8.3), mean follow-up of 3.5 years (s.d. 1.9). One or more damage item was present in 54% at baseline and 79% at the last follow-up on the Vasculitis Damage Index, and 60% at baseline and 82% at the last follow-up on the Large-Vessel Vasculitis Index of Damage...
November 3, 2017: Rheumatology
Sujith Subesinghe, Sander van Leuven, Leena Yalakki, Shirish Sangle, David D'Cruz
OBJECTIVES: We analysed the spectrum of clinical manifestations of cocaine associated pseudovasculitis. METHODS: Clinical, serological, radiological and histological features of 14 patients with cocaine pseudovasculitis syndromes were included. RESULTS: Twelve patients had significant sinus thickening or erosive disease. Other multi-system manifestations included vasculitic rashes, pulmonary lesions and peripheral neuropathy. All patients had positive ANCA titres at presentation...
November 3, 2017: Autoimmunity Reviews
Mie Takahashi, Haruki Koike, Shohei Ikeda, Yuichi Kawagashira, Masahiro Iijima, Atsushi Hashizume, Masahisa Katsuno, Gen Sobue
OBJECTIVE: To investigate the mechanisms of vasculitis in nonsystemic vasculitic neuropathy (NSVN) and microscopic polyangiitis (MPA), focusing on complement- and antineutrophil cytoplasmic antibody (ANCA)-associated pathogenesis. METHODS: Sural nerve biopsy specimens taken from twenty-four patients with NSVN and 37 with MPA-associated neuropathy (MPAN) were examined. Twenty-two patients in the MPAN group tested positive for ANCA. RESULTS: Immunostaining for complement component C3d deposition showed more frequent positive staining of epineurial small vessels in NSVN than in MPAN (p = 0...
November 2017: Neurology® Neuroimmunology & Neuroinflammation
Jiang Zhu, Yuan Jiang, Yewen Shi, Bo Zheng, Zhiguo Xu, Wei Jia
Syphilitic chorioretinitis should be included in differential diagnosis of any form of ocular inflammation. A significantly higher proportion of human immunodeficiency virus (HIV)-positive patients with ocular syphilis as compared to HIV-negative cases have been reported in published studies. However, the clinical signs and symptoms are more insidious in HIV-negative patients who are easily misdiagnosed. We report a series of cases of ocular syphilis and describe the clinical manifestations and treatment outcomes of syphilitic chorioretinitis in HIV-negative patients in China...
October 2017: Medicine (Baltimore)
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