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Semin Arthritis Rheum 2021 Apr 28;51(4):839-844. Epub 2021 Apr 28.

Department of Pediatrics, Division of Rheumatology, Hacettepe University School of Medicine, Ankara, Turkey; Pediatric Rheumatology Unit, Translational Medicine Laboratories, Hacettepe University, Ankara, Turkey. Electronic address:

Objectives: The first objective was to assess the role of vascular inflammatory factors in the pathogenesis of deficiency of adenosine deaminase 2 (DADA2) and to compare these markers among DADA2 patients with different phenotypes. We also aimed to investigate differences between DADA2 patients with vasculitic features and classic polyarteritis nodosa (PAN) for the aforementioned markers.

Methods: The study included eighteen DADA2 patients, ten PAN patients, and eight healthy controls. Read More

Autoimmun Rev 2021 Jun 9:102874. Epub 2021 Jun 9.

Department of Internal Medicine, Hôpital Cochin, 27, rue du Faubourg Saint-Jacques, 75679 Paris Cedex 14, France. Electronic address:

Clin Nephrol 2021 Jun 4. Epub 2021 Jun 4.

IgG4-related disease is an immune-mediated systemic inflammatory condition characterized by tissue infiltration of IgG4-positive plasma cells and elevated serum IgG4 concentrations. Peripheral neuropathy is an atypical manifestation of this disease. We describe an unusual case of vasculitic neuropathy in a patient with IgG4-related kidney disease. Read More

Front Pharmacol 2021 14;12:610585. Epub 2021 Apr 14.

Colentina-Research and Development Center, Colentina Clinical Hospital, Bucharest, Romania.

Immune axonal neuropathies are a particular group of immune-mediated neuropathies that occasionally accompany systemic autoimmune rheumatic diseases such as connective tissue dissorders and primary systemic vasculitides. Apart from vasculitis of vasa nervorum, various other mechanisms are involved in their pathogenesis, with possible therapeutic implications. Immune axonal neuropathies have highly heterogeneous clinical presentation and course, ranging from mild chronic distal sensorimotor polyneuropathy to severe subacute mononeuritis multiplex with rapid progression and constitutional symptoms such as fever, malaise, weight loss and night sweats, underpinning a vasculitic process. Read More

In Vivo 2021 May-Jun;35(3):1537-1543

Department of Neurosurgery, Adatip Hospital, Istanbul, Turkey

Background/aim: Neuropathic pain and neuropathy is commonly seen after ischemia-reperfusion injuries. Our aim was to evaluate the effect of lutein on ischemia-reperfusion (I/R)-induced vasculitic neuropathic pain and neuropathy in rats.

Materials And Methods: An hour before anesthesia, 6 Albino Wistar male rats with I/R were orally administered with 1 mg/kg lutein (LIR group). Read More

Clin Geriatr Med 2021 May 23;37(2):327-345. Epub 2021 Mar 23.

Department of Neurology, Virginia Commonwealth University, 1101 East Marshall Street, PO Box 980599, Richmond, VA 23298, USA. Electronic address:

This article discusses the chronic immune-mediated polyneuropathies, a broad category of acquired polyneuropathies that encompasses chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), the most common immune-mediated neuropathy, the CIDP variants, and the vasculitic neuropathies. Polyneuropathies associated with rheumatological diseases and systemic inflammatory diseases, such as sarcoidosis, will also be briefly covered. These patients' history, examination, serum studies, and electrodiagnostic studies, as well as histopathological findings in the case of vasculitis, confirm the diagnosis and differentiate them from the more common length-dependent polyneuropathies. Read More

Cureus 2021 Mar 9;13(3):e13776. Epub 2021 Mar 9.

Internal Medicine, Nepalese Army Institute of Health Sciences, Kathmandu, NPL.

Multiple myeloma (MM) is a clonal proliferation of plasma cells in the bone marrow resulting in the production of paraproteins. It is more common in elderly adults and presents with nonspecific symptoms like bone pain, pathological fracture, fatigue, and signs of hypercalcemia. Peripheral neuropathy is an atypical presentation. Read More

J Neurol Sci 2021 05 27;424:117421. Epub 2021 Mar 27.

University of Virginia, Department of Neurology, Charlottesville, VA, USA.

Rheumatological diseases result in immune-mediated injury to not only connective tissue, but often components of the peripheral nervous system. These overlap conditions can be broadly categorized as peripheral neuropathies and overlap myositis. The peripheral neuropathies are distinctive as many have unusual presentations such as non-length-dependent, small fiber neuropathies and sensory neuronopathies (both due to dorsal root ganglia dysfunction), multiple mononeuropathies (e. Read More

World J Clin Cases 2021 Mar;9(7):1741-1747

Department of Rehabilitation Medicine, Seoul National University College of Medicine, SMG-SNU Boramae Medical Center, Seoul 07061, South Korea.

Background: Vasculitis, a systemic disorder with inflammation of blood vessel walls, can develop broad spectrum of signs and symptoms according to involvement of various organs, and therefore, early diagnosis of vasculitis is challenging. We herein describe a patient who developed a special case of systemic vasculitis with mononeuropathy multiplex, rectal perforation and antiphospholipid syndrome (APS) presented with pulmonary embolism.

Case Summary: A 61-year-old woman visited hospital with complaints of myalgia and occasional fever. Read More

Neurol India 2021 Jan-Feb;69(1):140-146

Department of Neuropathology, National Institute of Mental Health Neurosciences, Bangalore, Karnataka, India.

Background: Perls Prussian blue stain (PPB) for hemosiderin, a marker of vascular injury is often employed as an adjunct in the diagnosis of vasculitic neuropathies. However, inflammation/vascular injury is also seen in leprosy, immune mediated, paraproteinemic, diabetic neuropathies, etc. The frequency of detection of hemosiderin in these neuropathies and its utility in diagnosis of vasculitis has not been explored. Read More

Muscle Nerve 2021 Feb 25. Epub 2021 Feb 25.

Brain and Mind Centre, University of Sydney, Sydney, New South Wales, Australia.

After initial investigation of patients presenting with symptoms suggestive of neuropathy, a clinical decision is made for a minority of patients to undergo further assessment with nerve biopsy. Many nerve biopsies do not demonstrate a definitive pathological diagnosis and there is considerable cost and morbidity associated with the procedure. This highlights the need for appropriate selection of patients, nerves and neuropathology techniques. Read More

Medicina (Kaunas) 2021 Feb 9;57(2). Epub 2021 Feb 9.

Division of Rheumatology, Department of Internal Medicine, School of Medicine, Kyung Hee University, 26 Kyungheedae-ro, Dongdaemun-gu, Seoul 02447, Korea.

Microscopic polyangiitis (MPA) is an anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). The splenic involvement in AAV is known to be rare, and that in MPA has not been reported to date. A 74-year-old woman was admitted owing to left arm numbness and weakness. Read More

Rheumatology (Oxford) 2021 Jan 27. Epub 2021 Jan 27.

Division of Rheumatology, Department of Medicine, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.

Background: Limited evidence exists to guide treatment of refractory vasculitic neuropathy. While rituximab and intravenous immune globulin (IVIG) have both been proposed as individual treatment options for these patients, combination therapy has never been reported.

Methods: Written informed consent was obtained from three patients with refractory vasculitic neuropathy who were treated with combination rituximab and IVIG. Read More

Brain Sci 2021 Jan 20;11(2). Epub 2021 Jan 20.

Neurology Unit, Department of Neuroscience, Biomedicine and Movement Sciences, University of Verona, 37134 Verona, Italy.

Despite the introduction of non-invasive techniques in the study of peripheral neuropathies, sural nerve biopsy remains the gold standard for the diagnosis of several neuropathies, including vasculitic neuropathy and neurolymphomatosis. Besides its diagnostic role, sural nerve biopsy has helped to shed light on the pathogenic mechanisms of different neuropathies. In the present review, we discuss how pathological findings helped understand the mechanisms of polyneuropathies complicating hematological diseases. Read More

Handchir Mikrochir Plast Chir 2021 Jan 18. Epub 2021 Jan 18.

Intern Med 2021 Jun 15;60(12):1941-1947. Epub 2021 Jan 15.

Department of Neurology, Tokyo Metropolitan Neurological Hospital, Japan.

Due to its rarity and the limited literature, the clinicopathological characteristics of peripheral nerve involvement in immunoglobulin G4 (IgG4)-related disease are unknown. We present two cases of IgG4-related disease, accompanied by peripheral neuropathy, presenting as unilateral ptosis (case 1) and sclerosing cholangitis (case 2), respectively. In both cases, sural nerve biopsy indicated vasculitis as the underlying pathophysiology; the peripheral neuropathy was refractory to corticosteroid therapy. Read More

Front Pediatr 2020 7;8:581780. Epub 2020 Dec 7.

Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy.

Kawasaki Disease is a systemic vasculitis, particularly involving coronary arteries. Rare involvement of other vascular districts is described, as central nervous system arteries, leading to a vasculitic neuropathy. Sensorineural hearing loss and alterations of evoked potentials are uncommonly reported complications. Read More

J Neuromuscul Dis 2021 ;8(1):155-161

Neurology department, FLENI, Buenos Aires, Argentina.

Background: Vasculitic peripheral neuropathy (VPN) is caused by vessel inflammation leading to peripheral nerve injury of acute-to-subacute onset. When VPN occurs in the context of systemic disease it is classified as Systemic Vasculitic Neuropathy (SVN) and as Non-Systemic Vasculitic Neuropathy (NSVN) when restricted to the nerves.

Objective: This study aimed to compare the clinical characteristics, biopsy findings and disease outcome in patients with VPN. Read More

Continuum (Minneap Minn) 2020 10;26(5):1257-1279

Purpose Of Review: This article discusses peripheral neuropathies associated with vasculitis (isolated or in the setting of systemic vasculitis) and autoimmune connective tissue disease and provides a brief overview of their diagnostic evaluation and management.

Recent Findings: The classification of systemic vasculitic neuropathy and nonsystemic vasculitic neuropathy continues to evolve. Classification according to the presence of antineutrophil cytoplasmic antibodies and their subtypes facilitates prognostication and management. Read More

eNeurologicalSci 2020 Dec 11;21:100272. Epub 2020 Sep 11.

Neurology Department, Centro Hospitalar de Entre o Douro e Vouga, Santa Maria da Feira, Portugal.

Introduction: Peripheral neuropathies may present in the context of systemic vasculitis and other autoimmune diseases. The etiologic characterization is crucial to define the treatment and prognosis in secondary vasculitis. The purpose of this study is to describe the pathway of etiologic investigation including the role of nerve biopsy. Read More

Ticks Tick Borne Dis 2020 11 25;11(6):101545. Epub 2020 Aug 25.

Southampton General Hospital, Tremona Road, Southampton, SO16 6YD, UK.

Lyme neuroborreliosis (LNB) typically presents as a painful radiculitis or a cranial mononeuropathy with lymphocytic meningitis (Bannwarth's syndrome). Isolated peripheral mononeuropathy or multiple mononeuropathy is less frequently recognised. A 58-year-old female with a background of IgA nephropathy and chronic kidney disease presented with a painful left ulnar neuropathy followed within 3 months by superficial radial neuropathy. Read More

Eur J Rheumatol 2020 Sep 8. Epub 2020 Sep 8.

Department of Rheumatology, Hiroshima City Hiroshima Citizens Hospital, Hiroshima, Japan.

Semin Arthritis Rheum 2020 10 27;50(5):846-853. Epub 2020 Jul 27.

Department of Pathophysiology, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece; Joint Rheumatology Academic Program, University of Athens, Athens, Greece.

Objective: To describe the clinical spectrum of cryoglobulinemic vasculitis (CV) in primary Sjögren's syndrome (pSS), investigate its relation to lymphoma and identify the differences with hepatitis C virus (HCV) related CV.

Methods: From a multicentre study population of consecutive pSS patients, those who had been evaluated for cryoglobulins and fulfilled the 2011 classification criteria for CV were identified retrospectively. pSS-CV patients were matched with pSS patients without cryoglobulins (1:2) and HCV-CV patients (1:1). Read More

Cureus 2020 Aug 30;12(8):e10133. Epub 2020 Aug 30.

Neurology, CHRISTUS Good Shepherd Medical Center, Longview, USA.

Splenic marginal zone lymphoma (SMZL), a rare sub-type of non-Hodgkin lymphoma (NHL) presents with abdominal discomfort, lymphocytosis, cytopenias along with B symptoms including fatigue, night sweats, night fevers, weight loss. NHLs rarely present with paraneoplastic neurological syndromes like Guillain-Barre (GB) syndrome, myelopathy causing paraplegia, chorea, neuromyotonia, vasculitic neuropathy and dermatomyositis. Here, we present a 85-year old caucasian lady presenting with GB syndrome and Syndrome of Inappropriate Antidiuretic Hormone (SIADH) who eventually got diagnosed with SMZL. Read More

J Cataract Refract Surg 2020 12;46(12):e8-e10

From the Renaissance School of Medicine (Rosenberg), Department of Ophthalmology (Weissbart, Sibony), Renaissance School of Medicine, Stony Brook University, Stony Brook, New York, USA.

An 82-year-old woman presented with a failed Descemet-stripping automated endothelial keratoplasty (DSAEK) in her right eye performed a month earlier. The patient underwent uneventful repeat DSAEK. Six days after the procedure, the patient was found to have an afferent pupillary defect and optic nerve swelling in her right eye on fundoscopy and optical coherence tomography. Read More

J Oncol Pharm Pract 2021 Apr 20;27(3):764-765. Epub 2020 Aug 20.

Department of Medicine, Division of Hematology Oncology, University of California, Irvine, Chao Family Comprehensive Cancer Center, Irvine, CA, USA.

Introduction: The incidence of neuropathy with checkpoint inhibitors is 0.3-1%, typically occurring 2-12 weeks after treatment initiation. Common neuropathy phenotypes include inflammatory myopathies, myasthenia gravis, acute and chronic demyelinating polyradiculopathies, vasculitic neuropathies, isolated cranial neuropathies, aseptic meningitis, autoimmune encephalitis, multiple sclerosis and hypophysitis. Read More

Intern Med 2020 Dec 4;59(23):3075-3078. Epub 2020 Aug 4.

Department of Neurology and Stroke Medicine, Yokohama City University Graduate School of Medicine, Japan.

We herein report a 33-year-old woman who was an asymptomatic hepatitis B virus (HBV) carrier and presented with distal muscle weakness in the legs and asymmetrical paresthesia in the distal extremities. A nerve biopsy specimen revealed fibrinoid necrosis associated with inflammatory infiltration in the perineural space, and deposition of hepatitis B core antigen and C4d complement was detected in the vascular endothelial cells as well as around the vessels. She was diagnosed with HBV-related vasculitic neuropathy and treated with intravenous immunoglobulin (IVIG). Read More

Neurol Clin 2020 08 11;38(3):711-735. Epub 2020 Jun 11.

Department of Neurology, Virginia Commonwealth University, 1101 East Marshall Street, PO Box 980599, Richmond, VA 23298-0599, USA.

The immune-mediated neuropathies are a broad category of diseases differentiated by time course, affected nerve fibers, and disease associations. This article spans the common, well-defined inflammatory demyelinating polyradiculoneuropathies (Guillain-Barré syndrome and chronic inflammatory demyelinating polyradiculoneuropathy) to the rarer, acquired demyelinating neuropathy variants (Miller-Fisher syndrome and multifocal motor neuropathy), vasculitic neuropathies, and sensory neuronopathies (dorsal root ganglionopathies). These case studies illustrate the characteristic clinical patterns of the immune-mediated neuropathies encountered in neurologic practice. Read More

Inflammopharmacology 2020 Dec;28(6):1753-1754

Department of Anesthesiology, Taipei Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, 289, Jianguo Rd., Sindian District, New Taipei City, 23142, Taiwan.

Unfortunately, Fig. 5 was incorrectly published in the original publication. The complete corrected Fig. Read More

Ter Arkh 2019 Dec 15;91(12):63-69. Epub 2019 Dec 15.

Clinic of modern rheumatology.

Vasculitis is a clinically diverse group of diseases with histopathological signs of blood vessel inflammation, which contributes to vascular damage and ischemic damage to the affected tissues. Vasculitic neuropathy is a common complication of the primary systemic vasculitides, such as polyartertis nodosa and antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, systemic diseases of the connective tissue - systemic lupus erythematosus and Sjogren syndrome, vasculitis associated with infection, most often viral hepatitis C and B and non - systemic vasculitis neuropathy. Vessels of medium and small caliber are involved in the pathological process in these diseases. Read More