A bibliometric analysis from 2004 to 2024 reveals research hotspots and trends in the immunotherapy for vasculitis.

Using bibliometric analysis, this study attempted to provide an overview of the current state of research and key findings regarding the immunotherapy for vasculitis in general. We gathered the literature from the Web of Science (WOS) database covering the last 20 years (2004-2024) pertaining to the immunotherapy for vasculitis, and we used Citespace to evaluate the mapping of knowledge. The findings demonstrated that there were 572 articles concerning the immunotherapy for vasculitis, with a faster growth after 2018. The USA, Assistance Publique Hopitaux Paris, and Cornelia M are the nation, organization, and writer with the highest number of publications. Daxini A (2018) is the most frequently mentioned reference as well (26). Prominent universities and developed nations form the finest alliances for research on immunotherapy for vasculitis researches. Immune checkpoint inhibitors, Wegener's granulomatosis, and systemic lupus erythematosus are Three research hotspots in this field.

Reproductive Health in Scleroderma, Vasculitis, and Sjögren Syndrome.

ABSTRACT: Women with systemic chronic inflammatory disease, such as those with scleroderma, systemic vasculitis, and Sjögren syndrome, need preconception evaluation by a multidisciplinary team. Counseling and pregnancy management should be tailored to patients' needs, considering specific disease features, organ involvement, treatment options, and risk factors to minimize risks of maternal-fetal complications during pregnancy.Additionally, considerations regarding fertility, assisted reproductive techniques, and contraception also need to be addressed for these women.In this narrative review, we integrate the current published literature with our expert opinion to address the issues faced by patients with the aforementioned inflammatory conditions.

[Peripheral neuroimmunological diseases - Neuropathological insights and clinical perspectives]. / Periphere neuroimmunologische Erkrankungen ­ neuropathologische Einsichten und klinische Perspektiven.

This article deals with peripheral neuroimmunological diseases and briefly outlines the currently most important aspects and treatment developments. Idiopathic inflammatory myopathies have different mechanisms of development, manifestations and prognoses. New classification systems and more specific treatment concepts have been developed. The IIMs include different subgroups. These entities can have specific autoantibodies. Diagnostically, a muscle biopsy is generally desirable for a precise diagnosis and is essential in unclear cases. Primary systemic vasculitides can be divided into different groups based on the predominant pattern of involvement, while secondary vasculitides and single organ vasculitides are also differentiated. Vasculitic myopathy cannot be equated with myositis and a reliable distinction is currently only possible by a muscle biopsy. Treatment concepts should be developed on an interdisciplinary basis. Chronic inflammatory demyelinating polyneuropathy is the most frequent immune-mediated neuropathy and is characterized by a predominant demyelination of the motor and sensory nerves. The disease course runs in phases or is progressive and leads to significant disability and reduction in quality of life, despite current standard treatment. Novel treatment approaches are currently undergoing clinical trials. Myasthenia gravis, with the leading symptom of exercise-induced muscle weakness, is caused by autoantibodies against structures of the neuromuscular endplate. Autoantibody testing is the most important pillar in the diagnosis and is now also increasingly guiding treatment decisions. Overall, peripheral neuroimmunological diseases represent a heterogeneous group. Increasing knowledge of the pathophysiology is the key to numerous developments in diagnostics and treatment, which could lead to far-reaching practical changes in the future.

Renal disease in pediatric rheumatology.

PURPOSE OF REVIEW: This review will provide updates in the outcomes in the common rheumatologic diseases with kidney involvement. Covered are also advances in therapeutics for the use of pediatric rheumatologic diseases with kidney involvement, as well as the potential kidney complications from other rheumatologic diseases and their medications. RECENT FINDINGS: Two of the more common rheumatologic diseases with kidney involvement, lupus and vasculitis, continue to show inadequate response to initial therapy of renal disease and practice continues to be driven by results of adult studies. SUMMARY: There is a continued need for pediatric specific studies in rheumatologic diseases with kidney involvement as outcomes continue to be inadequate. Despite recently approved treatments for adults with rheumatic diseases and kidney involvement, therapeutic options in pediatrics remain limited, contributing to the overall morbidity and mortality.

Neuro-ophthalmic challenges and multi-morbidity in vasculitis among the older adults.

INTRODUCTION: Vasculitides are a heterogeneous group of disorders producing inflammation of blood vessels (e.g. arteries or veins). All major vasculitides potentially have ophthalmological symptoms and signs including visual loss. Co-morbidity, multimorbidity, polypharmacy, and geriatric syndromes all play important roles in patient outcomes for these rheumatic conditions in the elderly. This monograph reviews the NCBI PubMed database (Feb 2023) literature on the neuro-ophthalmic and geriatric considerations in vasculitis. AREAS COVERED: Cogan Syndrome, Granulomatosis with Polyangiitis, Giant Cell Arteritis, Polyarteritis Nodosa, Takayasu Arteritis, Vasculitis epidemiology, and neuro-ophthalmological symptoms. EXPERT OPINION: Geriatric patient care for vasculitis with neuro-ophthalmological manifestations can be complicated by the interplay of multiple co-morbidities, polypharmacy, and specific geriatric syndromes. The valuation and treatment of vasculitis and the complications associated with the disease can negatively impact patient care. Advances in noninvasive imaging and updates in diagnostic criteria have enabled increased identification of patients at earlier stages with less severe disease burden. Novel therapeutic agents can be glucocorticoid sparing and might reduce the adverse effects of chronic steroid use. Holistic care models like the 5 M geriatric care model (mind, mobility, medications, multicomplexity, and matters most) allow patients' needs to be in the forefront with biopsychosocial aspects of a patient being addressed.

Efficacy of Therapeutic Apheresis for Cryoglobulinemic Vasculitis Patients with Renal Involvement: A Systematic Review.

INTRODUCTION: Therapeutic apheresis (TA) is commonly used for cryoglobulinemic vasculitis (CV) patients, but its efficacy remains uncertain. This systematic review aimed to assess the efficacy of different TA modalities, such as plasma exchange (PE), plasmapheresis (PP), and cryofiltration (CF), in treating CV patients with renal involvement. METHODS: Literature search of MEDLINE, EMBASE, and Cochrane Databases was conducted up to December 2022. Studies that reported the outcomes of TA in adult CV patients with renal involvement were assessed. The protocol for this systematic review has been registered with PROSPERO (No. CRD42023417727). The quality of each study was evaluated by the investigators using the validated methodological index for non-randomized studies (minors) quality score. RESULTS: 154 patients who encountered 170 episodes of serious events necessitating TA were evaluated across 76 studies. Among them, 51% were males, with a mean age ranging from 49 to 58 years. The CV types included 15 type I, 97 type II, and 13 type III, while the remaining patients exhibited mixed (n = 17) or undetermined CV types (n = 12). Among the treatment modalities, PE, PP, and CF were performed in 85 (56%), 52 (34%), and 17 patients (11%), respectively, with no identical protocol for TA treatment. The overall response rate for TA was 78%, with response rates of 84%, 77%, and 75% observed in type I, II, and III patients respectively. Most patients received steroids, immunosuppressants, and treatment targeting the underlying causative disease. The overall long-term renal outcome rate was 77%, with type I, II, and III patients experiencing response rates of 89%, 76%, and 90%, respectively. The renal outcomes in patients receiving PE, PP, and CF were comparable, with rates of 78%, 76%, and 81%, respectively. CONCLUSIONS: This study presents compelling evidence that combination of TA with other treatments, especially immunosuppressive therapy, is a successful strategy for effectively managing severe renal involvement in CV patients. Among the TA modalities studied, including PE, PP, and CF, all demonstrated efficacy, with PE being the most frequently employed approach.

Autoimmune Axonal Neuropathies.

OBJECTIVE: This article reviews autoimmune axonal neuropathies, their characteristic clinical features, disease and antibody associations, appropriate ancillary testing, treatment, and prognosis. LATEST DEVELOPMENTS: In 2021, the American College of Rheumatology and the Vasculitis Foundation released new summary guidelines for the treatment of antineutrophil cytoplasmic autoantibody-associated vasculitides. In addition, novel autoantibodies have been recently identified; they are often paraneoplastic and associated with axonal neuropathies. ESSENTIAL POINTS: Recognition of autoimmune axonal neuropathies is important because of the potential for effective treatment to either reverse deficits or slow the progression of disease. It is necessary to properly assess for associations with other systemic disorders (eg, systemic vasculitis, connective tissue disease, neoplasm) so that adequate treatment for both neurologic and non-neurologic aspects of the disease can be initiated.

A rare complication of IgA vasculitis: renal and intestinal ischemia successfully treated with plasmapheresis.

BACKGROUND: IgA vasculitis (IgAV) is a multisystemic small vessel vasculitis and is the most common vasculitis in childhood. The characteristic findings of IgAV are palpable purpuric rash, abdominal pain, arthralgia or arthritis, and hematuria. Ischemic complications are very rare in IgAV. Thrombotic complications can be observed after a COVID-19 infection. Also in the presence of familial Mediterranean fever, IgAV may have an atypical or more severe course. CASE: We present a case of IgAV complicated with renal infarction and intestinal ischemia. There was no recent or distant history of COVID-19 in the patient or family members, but the patient`s COVID-19 antibody was positive. In addition, MEFV gene analysis of the patient showed homozygous M694V mutation. The patient did not respond to enoxaparin, pulse methylprednisolone, intravenous immunoglobulin (IVIG), iloprost, and cyclophosphamide treatments. She was successfully treated with six sessions of plasmapheresis. CONCLUSIONS: Plasmapheresis seems to be an effective treatment option in IgAV-related ischemic findings that do not respond to intensive immunosuppressive therapy.

Systemic vasculitis and headache.

PURPOSE OF REVIEW: Vasculitis refers to heterogeneous clinicopathologic disorders that share the histopathology of inflammation of blood vessels. Unrecognized and therefore untreated, vasculitis of the nervous system or so called neurovasculitides, lead to pervasive injury and disability making these disorder of paramount importance to clinicians. RECENT FINDINGS: Headache is an important clue to vasculitic involvement of central nervous system (CNS) vessels. CNS vasculitis may be primary, in which only intracranial vessels are involved in the inflammatory process, or secondary to another known disorder with overlapping systemic involvement. A suspicion of vasculitis based on the history, clinical examination, or laboratory studies warrants prompt evaluation and treatment to forestall progression and avert cerebral ischemia or infarction. There has been remarkable progress in the pathogenesis, diagnosis, and treatment of primary adult and pediatric CNS vasculitides predicated on achievements in primary systemic forms. SUMMARY: Vasculitis can be diagnosed with certainty after intensive evaluation that includes tissue confirmation whenever possible. Clinicians must choose from among the available immune modulating, suppressive, and targeted immunotherapies to induce and maintain remission status and prevent relapse, tempered by the recognition of anticipated medication side effects.

A case series on recurrent and persisting IgA vasculitis (Henoch Schonlein purpura) in children.

BACKGROUND: IgA vasculitis (IgAV) is a small vessel vasculitis that is more common in childhood. Very limited evidence exists on patients who experience an atypical disease course. The aim of this study was to describe a cohort of children diagnosed with recurrent or persisting IgAV to identify any themes associated with their disease course and areas of unmet needs. METHODS: A single centre retrospective study of children diagnosed with recurrent or persisting IgAV at Alder Hey Children's Hospital (Liverpool, UK). Clinical data, including features at presentation and during follow up, potential triggers, abnormal laboratory and histology results, treatment and outcome at last clinical review were retrospectively collected. Key themes were identified. RESULTS: A total of 13 children met the inclusion criteria (recurrent disease, n = 4; persisting disease, n = 9). Median age at first presentation was 10.2 years [2.6-15.5], female:male ratio 1.2:1. Children in the atypical cohort were significantly older than a larger cohort of children who followed a non-complicated disease course (median age 5.5 years (range [0.6-16.7], p = 0.003)). All children re-presented with a purpuric rash (either recurring or persisting), accompanied by joint involvement in 92% of patients (12/13). Disease-modifying anti-rheumatic drugs (DMARDs) were used in 8/13 (62%) children. The median time from first presentation to diagnosis of atypical disease was 18.4 months [5.3-150.8] and the time from first presentation to treatment was 24.1 months [1.8-95.4]. Use of corticosteroids was significantly higher in children with renal involvement (p = 0.026). During follow up, 8/13 (62%) children were admitted at least once, whilst 10/13 (77%) had re-presented at least once to the emergency department. Five (38%) children were referred to psychology services and 7 (54%) children reported feelings of frustration. CONCLUSIONS: This series describes some characteristics of a small cohort of children with atypical IgAV. It also identifies unmet needs in children with atypical IgAV, which includes delays in diagnosis and lengthy waits for treatment, lack of high-quality evidence regarding treatment choices and a high unrecognised disease burden. Further research is needed to study this subgroup of children as evidence is lacking.

Adult and childhood vasculitis.

Vasculitis refers to heterogeneous clinicopathologic disorders that share the histopathology of inflammation of blood vessels. Unrecognized and therefore untreated, vasculitis of the nervous system leads to pervasive injury and disability, making this a disorder of paramount importance to all clinicians. There has been remarkable progress in the pathogenesis, diagnosis, and treatment of primary CNS and PNS vasculitides, predicated on achievement in primary systemic forms. Primary neurological vasculitides can be diagnosed with assurance after intensive evaluation that incudes tissue confirmation whenever possible. Clinicians must choose from among the available immune modulating, suppressive, and targeted immunotherapies to induce and maintain remission status and prevent relapse, unfortunately without the benefit of RCTs, and tempered by the recognition of anticipated medication side effects. It may be said that efforts to define a disease are attempts to understand the very concept of the disease. This has been especially evident in systemic and neurological disorders associated with vasculitis. For the past 100 years, since the first description of granulomatous angiitis of the brain, the CNS vasculitides have captured the attention of generations of clinical investigators around the globe to reach a better understanding of vasculitides involving the central and peripheral nervous system. Since that time it has become increasingly evident that this will necessitate an international collaborative effort.

Reporte de caso: vasculitis leucocitoclastica como forma de presentación de hepatitis b crónica / Case report: leukocytoclastic vasculitis as presenting manifestation of chronic hepatitis b virus infection

La vasculitis leucocitoclástica, también denominada angeitis cutánea leucocitoclástica, es la forma más común de vasculitis. Si bien la mayoría de los casos son idiopáticos, entre los agentes etiológicos que podemos nombrar se encuentran los agentes infecciosos, las enfermedades del tejido conectivos, las reacciones de hipersensibilidad a medicamentos y las neoplasias solidas o hematológicas. Si bien los procesos infecciosos son una causa conocida de vasculitis leucocitoclástica, la infección por virus de Virus de hepatitis B (VHB) es muy infrecuente. Presentamos una mujer de 47 años, sin antecedentes patológicos previos, que consultó por artralgias en rodillas y tobillos, mialgias en gemelos y rash purpúrico con leve prurito en ambos miembros inferiores, de un mes de evolución. La biopsia cutánea de las lesiones de miembros inferiores fue compatible con vasculitis leucocitoclástica. La serología de hepatitis B fue positiva por lo que inició tratamiento antiviral con Tenofovir y Prednisona con buena evolución de sus lesiones cutáneas

Leukocytoclastic vasculitis, also called leukocytoclastic cutaneous angiitis, is the most common form of vasculitis. Although most cases are idiopathic, etiologic agents include infectious agents, connective tissue diseases, drug hypersensitivity reactions, and solid or hematologic malignancies. Although infectious processes are a known cause of leukocytoclastic vasculitis, hepatitis B virus (HBV) infection is very rare. We present a 47-year-old woman, with no previous pathologic history, who consulted for arthralgias in the knees and ankles, myalgia's and purpuric rash with mild pruritus in both lower limbs, of one month evolution. Skin biopsy of lower extremity lesions was compatible with leukocytoclastic vasculitis. Hepatitis B serology was positive, so she started antiviral treatment with tenofovir and prednisone with good evolution of her skin lesions

Annular vasculitic lesions.

Vasculitic skin findings may present with annular morphologies. This group of conditions consists of capillaritis, such as pigmented purpuric dermatoses, and vasculitis, which is often classified by the affected vessel size. Annular vasculitic lesions may be the presenting sign of systemic disease, thus requiring thorough exploration to reach an accurate diagnosis and guide proper disease management. Herein we review the clinical presentation, histopathology, and treatments for cutaneous vasculitic disease that may present with annular lesions.

Ear, Nose, and Throat Manifestations of Vasculitis and Other Systemic Autoimmune Diseases: Otologic, Sinus, and Airway.

Auricular, nasal, and laryngeal manifestations occur frequently in rheumatic diseases. Inflammatory ear, nose, and throat (ENT) processes often result in organ damage and have profound effects on quality of life. Herein, we review the otologic, nasal, and laryngeal involvement of rheumatic diseases, focusing on their clinical presentation and diagnosis. ENT manifestations generally respond to treatment of the systemic disease, which is outside the scope of this review; however, adjunctive topical and surgical treatment approaches, as well as treatment of idiopathic inflammatory ENT manifestations will be reviewed.

Vasculitis and Pregnancy: Disease State and Management.

Family planning in women with vasculitis requires an interdisciplinary approach. This article summarizes recommendations and guidance for each phase of family planning in persons with vasculitis including preconception counseling, birth control, pregnancy, and breastfeeding. Pregnancy complications are presented by category of vasculitis with accompanying diagnostic and therapeutic recommendations. Birth control and assisted reproductive technology options are reviewed with special considerations for women who are high risk or have a history of blood clots. This article can be used as a clinical reference for reproductive discussions in all patients with vasculitis.