Edoardo Conticini, Jurgen Sota, Paolo Falsetti, Arianna Lamberti, Clelia Miracco, Andrea Guarnieri, Bruno Frediani, Luca Cantarini
Polyarteritis nodosa (PAN) is a medium vessels vasculitis variously involving different organs and systems, sometimes with an aggressive course, leading to death or disability in a significant number of cases. First-line treatment usually relies on steroids and classical immunosuppressants, but a growing number of case reports and small case series shows the potential role of biologic drugs, mostly anti-tumor necrosis factor (TNF)-α agents, in inducing and maintaining remission in patients affected by PAN...
February 17, 2021: Autoimmunity Reviews
Fabiana Assunta Figus, Matteo Piga, Irene Azzolin, Rebecca McConnell, Annamaria Iagnocco
Although synovitis is the pathological hallmark of rheumatoid arthritis (RA), many extra-articular manifestations (EMs) and comorbidities likely occur due to the complex, chronic, inflammatory, and autoimmune features of RA. Cardiovascular (CV) disease is the most common cause of death in patients with RA. Compared to the general population, patients with RA have twice the risk of myocardial infarction and up to 50% increased CV mortality risk. Severe and prolonged disease activity, genetics, and inflammation (e...
February 17, 2021: Autoimmunity Reviews
Luca Quartuccio, Elena Treppo, Marco Binutti, Giulia Del Frate, Salvatore De Vita
No abstract text is available yet for this article.
February 20, 2021: Rheumatology
Zhiqian Yang, Dongpeng Cai, Yesheng Sun, Dequan Zhong, Hui Zhou, Deyuan Zhang, Wei Zhang
BACKGROUND: Microscopic polyangiitis (MPA), an autoimmune disease, is a subtype of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis. The lungs and kidneys are the most common targets, whereas spinal cord involvement is rare. METHODS: We reported the clinical manifestations, diagnosis, and management of a patient with spinal cord MPA. RESULTS: The patient showed spinal compression symptoms and was diagnosed with MPA following magnetic resonance imaging (MRI) and histological examination...
February 19, 2021: Neurological Sciences
Cancan Li, Yiming Du, Huawei Wang, Gaohong Wu, Xueping Zhu
RATIONALE: Kawasaki Disease (KD) is a self-limiting and acute systemic vasculitis of childhood that leads to coronary artery abnormality in about 25% of untreated cases. KD is extremely rare in neonates. The purpose of this paper is to explore the clinical features and diagnosis and treatment of Neonatal Kawasaki Disease for early identification. PATIENT CONCERNS: A 24-day-old male with 3 hours fever and a rash was admitted to our hospital. DIAGNOSES: He had a fever, rash, cracking of lips, lymph node enlargement in the neck, and distal extremity desquamation...
February 19, 2021: Medicine (Baltimore)
Mónica Calderón-Goercke, Santos Castañeda, Vicente Aldasoro, Ignacio Villa, Clara Moriano, Susana Romero-Yuste, Javier Narváez, Catalina Gómez-Arango, Eva Pérez-Pampín, Rafael Melero, Elena Becerra-Fernández, Marcelino Revenga, Noelia Álvarez-Rivas, Carles Galisteo, Francisca Sivera, Eugenio De Miguel, Diana Prieto-Peña, Miguel Á González-Gay, José L Hernández, Ricardo Blanco
OBJECTIVE: To compare the efficacy and safety of TCZ in monotherapy (TCZMONO ) vs. combined with conventional immunosuppressive drugs (TCZCOMBO ) in Giant Cell Arteritis (GCA) in a clinical practice scenario. METHODS: Multicenter study of 134 patients with refractory GCA. Patients on TCZMONO (n = 82) were compared with those on TCZCOMBO (n = 52). Drugs were methotrexate (MTX) (n = 48), azathioprine (n = 3), and leflunomide (n = 1). The main outcomes were: prolonged remission (normalization of clinical and laboratory parameters for at least 6 months) and the number of relapses...
January 27, 2021: Seminars in Arthritis and Rheumatism
Mario Berríos-Hernández, Charigan Abou-Jokh Casas, Laura Sainz-Gaspar, Javier Ginarte-Val, Virginia Fernández Redondo, Diego López-Otero, Carlos Aliste, Jose M Suárez-Peñaranda
Different hydrophilic and hydrophobic polymers are used as lubricious coatings to reduce vascular traumas in minimally invasive percutaneous procedures. Although they are usually very safe, there is still a risk of serious complications in patients undergoing such procedures, mostly derived from the devices' coating detachment and systemic embolization. The lungs are the most common organ involved, followed by the central nervous system. Yet, cutaneous embolization is unusual, and only 19 cases are available in the literature...
February 16, 2021: American Journal of Dermatopathology
Samira Alesaeidi, Seyed Mohammad Piri, Soheil Tavakolpour
BACKGROUND: Granulomatosis with polyangiitis is a systemic anti-neutrophil cytoplasmic antibody-associated vasculitides (AAVs), mainly involving the respiratory tract and renal system. Treatment by Rituximab as a next-generation therapy in ANCA-associated vasculitis was associated with promising outcomes in GPA patients. Despite symptoms improvements, disease recurrence and drug reaction was a challenging topic nowadays. OBJECTIVES: In this study, we examined six GPA patients who were confirmed to have paradoxical reactions to rituximab and then described How to control their symptoms...
February 19, 2021: Current Rheumatology Reviews
Ana Sofia Vaz, Raquel Penteado, Carolina Cordinhã, Carmen Carmo, Clara Gomes
BACKGROUND: Psoriasis is a chronic immune-mediated disorder that primarily affects the skin in both adults and children but can also have systemic involvement, particularly with arthritis and kidney injury. IgA nephropathy is the most frequent kidney disorder associated with psoriasis. Approximately one third of all cases of psoriasis begin in childhood, but association between psoriasis and renal disorders has scarcely been reported in pediatric patients. Henoch-Schönlein purpura (HSP) is a systemic vasculitis characterized by IgA deposits in the vessel walls of affected organs and in the mesangium of the kidney...
February 15, 2021: Jornal Brasileiro de Nefrologia: ʹorgão Oficial de Sociedades Brasileira e Latino-Americana de Nefrologia
Peining Zhou, Jing Ma, Guangfa Wang
Several retrospectivee described the association of interstitial lung disease (ILD) and ANCA-associated vasculitis (AAV). However, the relationship between the ILD and mortality in AAV patients have not been established so far. This study aims to estimate the relevance of AAV-associated-ILD (AAV-ILD) and mortality risk by conducting a systematic review and meta-analysis.A comprehensive systematic review was conducted in accordance with the guidelines of PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-analyses)...
January 2021: Chronic Respiratory Disease
Yoshinori Taniguchi, Takahito Kimata, Shigeto Kobayashi
A 69-year-old woman, whose HTLV-1 associated myelopathy (HAM) had been treated with interferon-α (IFN), presented with a three-week history of high fever, rapidly progressive discoloration and pain in the digits. Physical examination revealed black discoloration of the distal right second and fifth distal fingers (Figure 1A and B), and left second through fifth distal fingers, and purpuric lesions on several fingers.
February 18, 2021: Arthritis & Rheumatology
Burçin Cansu Bozca, Erkan Alpsoy
Behcet's disease (BD) is a chronic systemic vasculitis with inflammation attacks that involve multiple organs. In addition to numerous mucocutaneous symptoms, notably recurrent oral and genital ulcers, ocular, articular, vascular, gastrointestinal, cardiac, and neurological system involvement can be observed. Mucocutaneous lesions are the primary symptom of the disease in most patients, and they usually occur before major organ involvement and other symptoms of the disease. Recognizing the disease's mucocutaneous lesions is very important to diagnose at an early stage, control with appropriate treatment and close follow-up, and prevent major organ involvement...
2021: Journal of Experimental Pharmacology
Max Amos Hoffman, Adel Ekladious
A woman travelling to Australia in her early 70s presents to a regional emergency department with chest pain and associated shortness of breath. Her medical history was that of seasonal affective disorder treated with citalopram, and an allergy to ibuprofen. Subsequent CT imaging revealed aortic wall thickening and associated periaortic fluid, and a moderate pleural effusion. This was successfully treated with oral prednisolone, responding within 1 day. Further blood tests revealed a high CD4/CD8 T-cell ratio, which can be seen in autoimmune disease, sarcoidosis and haematological malignancies...
February 18, 2021: BMJ Case Reports
Michele Iudici, Christian Pagnoux, Delphine S Courvoisier, Pascal Cohen, Mohamed Hamidou, Achille Aouba, François Lifermann, Marc Ruivard, Olivier Aumaître, Bernard Bonnotte, François Maurier, Olivier Decaux, Eric Hachulla, Alexandre Karras, Chahéra Khouatra, Noémie Jourde-Chiche, Jean-François Viallard, Claire Blanchard-Delaunay, Pascal Godmer, Alain Le Quellec, Thomas Quéméneur, Claire de Moreuil, Alexis Régent, Benjamin Terrier, Luc Mouthon, Loïc Guillevin, Xavier Puéchal
OBJECTIVE: To describe the characteristics and long-term outcomes of patients with granulomatosis with polyangiitis (GPA) from the French Vasculitis Study Group database. METHODS: Patients' clinical and laboratory characteristics, Birmingham Vasculitis Activity Score (BVAS)-assessed disease activity, malignancies, opportunistic infections, and vital status were collected at diagnosis and each visit. Estimated probabilities and predictors of overall (OS) and relapse-free survival (RFS) were analyzed by Cox regression...
February 10, 2021: Seminars in Arthritis and Rheumatism
Balamurugan Thirunavukkarasu, Kirti Gupta, Ritambhra Nada, Manish Rathi, Varun Dhir, Chirag Kamal Ahuja, Aman Sharma, Vivek Lal, Bishan Dass Radotra
AIM: Systemic lupus erythematosus (SLE) is a prototype autoimmune disease characterized by circulating autoantibodies and immune complexes involving virtually every organ of the body. However, with respect to central nervous system (CNS), the mechanism of injury is still debated as complement mediated or thrombo-ischemic in nature. We studied the spectrum of neuropathological changes in twelve autopsy cases of SLE and evaluated the role of immune-complexes and complement activation in contributing to the thrombo-ischemic injury and correlated these features with clinical profile...
February 10, 2021: Journal of Neuroimmunology
Ouypom Panamonta, Vasant Sumethkue, Piyanuth Radinahmed, Malinee Laopaiboon, Wichitr Kirdpon
Propylthiouracil (PTU) can induce anti-myeloperoxidase (MPO-ANCA) positive vasculitis. We performed a cross-sectional study to estimate the prevalence of MPO-ANCA in patients with childhood onset Graves' disease (GD) receiving PTU and to assess the relationship between ANCA and clinical manifestations of vasculitis. We studied 60 patients (59 girls and one boy) between 7.3 and 25.0 years of age (mean ± SD, 14.71 ± 4.49). GD, diagnosed at the age of 3.0 to 14.5 years (11.3 ± 2.48), was designated as: newly diagnosed, on PTU therapy, and after PTU discontinuation in 4, 50 and 6 patients, respectively...
October 22, 2020: Journal of Pediatric Endocrinology & Metabolism: JPEM
Tamihiro Kawakami, Yuto Tamura, Yupeng Dong, Miku Yoshinari, Yuka Nishibata, Sakiko Masuda, Utano Tomaru, Akihiro Ishizu
We assessed the IgG and IgM prevalence of anti-phosphatidylserine/prothrombin complex (aPS/PT) antibodies (Abs) in patients with vasculitis using a novel commercial ELISA kit. To examine whether aPS/PT Abs were involved in the pathogenesis of cutaneous vasculitis, inbred wild-type rats were intravenously administered with a rat IgM class aPS/PT monoclonal Ab established previously or with rat immunoglobulins as controls. To express PS on the surface of vascular endothelium, these rats were given a subcutaneous injection of cell-free histones in advance...
February 18, 2021: Journal of Dermatology
Martina Uzzo, Federico Alberici
Anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) are rare autoimmune diseases characterised by medium and small vessels inflammation. Renal vasculitic involvement is one of the most severe manifestations, with high mortality in case of a delayed diagnosis and a significant impact on patients' long-term prognosis. Histological classifications and scores for the definition of renal involvement in AAV exist and correlate with the renal outcome. Current induction regimen consists of a high dose of glucocorticoids and immunosuppressive drugs: cyclophosphamide (CYC), rituximab (RTX) or a combination of both...
February 16, 2021: Giornale Italiano di Nefrologia: Organo Ufficiale Della Società Italiana di Nefrologia
Mikiko Harada, Hirohiko Motoki, Takahiro Sakai, Koichiro Kuwahara
Background: Granulocyte colony stimulating factor (G-CSF) preparations are used for patients with granulocytopenia, especially to prevent febrile neutropenia. Arteritis has been recognized as a side effect of G-CSF treatment; however, there are no clear diagnostic criteria or treatment guidelines because not enough cases have been reported. Present case showed one of the diagnostic and treatment selection methods via multiple imaging modality including vascular echography. Case summary: A 52-year-old woman underwent chemotherapy for ovarian cancer and received G-CSF because of myelosuppression...
February 2021: European Heart Journal. Case Reports
Dean J Choong, Jeremy L Ng, Carl Vinciullo
No abstract text is available yet for this article.
March 2021: JAAD Case Reports
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