Vasculitis reumatoidea: una manifestación poco habitual. Presentación de tres casos clínicos y revisión de la literatura

La vasculitis reumatoidea es una complicación sistémica y poco frecuente de la Artritis Reumatoidea. Si bien su incidencia ha descendido en los últimos años con el advenimiento de las nuevas terapias inmunosupresoras y biológicas, continua teniendo una alta morbimortalidad. Predomina en el sexo masculino, en pacientes seropositivos y con un largo período de la enfermedad establecida. Requiere de alta presunción diagnostica, siendo el compromiso cutáneo y nervioso periférico el más frecuente. La biopsia de nervio o piel es requerida habitualmente para su diagnóstico. El tratamiento se basa en corticoides e inmunosupresores. Presentamos tres casos clínicos y realizamos una revisión de la literatura.

Rheumatoid vasculitis is a rare systemic complication of rheumatoid arthritis. Although its incidence has decreased in recent years with the advent of new immunosuppressive and biological therapies, it continues to have a high morbidity and mortality. It predominates in males, in seropositive patients and with a long period of established disease. It requires high diagnostic presumption, with skin and peripheral nervous involvement being the most affected. Nerve or skin biopsy is usually required for diagnosis. Treatment is based on corticosteroids and immunosuppressants. We present three clinical cases and carry out a review of the literature.

A vasculite reumatóide é uma complicação sistêmica rara da artrite reumatóide. Embora sua incidência tenha diminuído nos últimos anos com o advento de novas terapias imunossupressoras e biológicas, continua apresentando elevada morbidade e mortalidade. Predomina no sexo masculino, em pacientes soropositivos e com longo período de doença estabelecida. Exige alta presunção diagnóstica, sendo o envolvimento cutâneo e nervoso periférico os mais afetados. A biópsia de nervo ou pele geralmente é necessária para o diagnóstico. O tratamento é baseado em corticosteroides e imunossupressores. Apresentamos três casos clínicos e realizamos uma revisão da literatura.

Late onset Kawasaki disease with multiple coronary arterial aneurysms: A case report.

Kawasaki disease is an acute, self-limiting, systemic vasculitis of small and medium-sized arteries. It predominantly occurs in children under 4 years of age, though rarely older children can also be affected. This disease is the leading cause of acquired heart disease in children, with coronary aneurysms being a hallmark finding. The risk of coronary complications necessitates regular monitoring and possible preventative treatment with thromboprophylaxis. Here we discuss a rare case of a 10-year-old boy who exhibited typical symptoms of Kawasaki disease and was found to have multiple coronary artery aneurysms through diagnostic imaging.

An unusual cause of headache: Expanding your differential.

Giant cell arteritis (GCA) is an inflammatory vasculitis that affects larger blood vessels, like the temporal arteries and the aorta. It is systemic and tends to affect individuals over 50. Common symptoms include headache, jaw pain provoked by chewing, and fever. We present an interesting case of a GCA variant.

SARS-CoV-2 as a trigger of IgA vasculitis: a clinical case and literature review.

Coronavirus Disease 2019 (COVID-19), caused by SARS-CoV-2, has negatively affected global health. COVID-19 has been associated with a variety of autoimmune and inflammatory disorders, complicating its respiratory manifestations. SARS-CoV-2 triggers inflammatory reactions which may involve multiple organs and systems. The proof for IgA involvement in the immune reactions to coronavirus infection is growing, particularly in the case of IgA immune complex deposition diseases such as IgA vasculitis (IgAV) and IgA nephropathy.This report presents a case of IgAV caused by SARS-CoV-2 in a 53-year-old man. His symptoms included papillomatous, bright red rashes, urticaria throughout the body, aphthous stomatitis, pain in all joints and muscles, weakness, malaise, abdominal pain, face swelling, and arterial hypertension (160/100 mmHg). He received intravenous methylprednisolone (250 mg) and then oral methylprednisolone (16 mg) treatment, which improved his condition. This improvement included the disappearance of abdominal and joint pain and skin rashes.This article also provides an overview of published cases of IgAV after SARS-CoV-2. It may alert rheumatologists and allied specialists of clinical features of IgAV and guide them how to diagnose and treat this disease.

ANCA-associated vasculitis and lung cancer: an immunological perspective.

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a severe autoimmune disease that often involves the upper and lower respiratory tracts. In recent years, numerous studies have found a significant increase in the incidence of cancer among AAV patients, but the association between lung cancer and AAV remains inconclusive, with relatively low clinical attention. This review summarizes the current literature on the risk of lung cancer in patients with ANCA-associated vasculitis (AAV), detailing the potential mechanisms by which AAV may contribute to lung cancer, and further elucidates the inherent carcinogenic risks of immunosuppressants.There is a correlation between AAV and lung cancer, which is related to T cell senescence and damage, as well as the abnormal expression of cytokines such as IL-6 and IL-10. In AAV patients, the use of cyclophosphamide and azathioprine (AZA) alone has a clear carcinogenic risk, with frequent use of CYC potentially posing a high risk for lung cancer. Although TNF inhibitors (TNFi) combined with CYC have carcinogenic risks, there is insufficient evidence to link them directly to an increased risk of lung cancer. For patients at high risk for lung cancer, the judicious use of immunosuppressants, timely computed tomography (CT), and lung cancer screening can reduce the risk of lung cancer in AAV patients.

Metallosis Following Non-Metal-on-Metal Hip Arthroplasty: A Case Report and Review.

Introduction: Metallosis which is traditionally associated with Metal-on-Metal (MoM) hip arthroplasty can occur with other bearing surfaces too, posing diagnostic challenges. They can be asymptomatic or present with local and systemic symptoms. This article reports a case of metallosis in a total hip replacement (THR) with metal on polyethylene (PE) articulation who presented with dislocation. It also reviews the pathology and various presentations of metallosis following hip arthroplasty. Case Report: A 35-year-old female patient presented 4 years after a left THR with recurrent dislocation. It was an uncemented prosthesis with metal on PE articulation. Serology and radiological investigations were done to evaluate for infection, implant loosening, implant malposition, etc. The femoral stem appeared to be in varus malposition. She was posted for revision surgery with a pre-operative plan to change the femoral stem and head if necessary. Intraoperative signs of local metallosis were noticed. Debridement was done along with the change of the femoral stem and bearing surface to ceramic on PE. Metallosis was also later confirmed by the histopathological report. The patient has been symptom-free during the 2-year follow-up period. Conclusion: Metallosis can occur even in non-MoM articulations and a high degree of clinical suspicion is required to detect the same preoperatively. Classical signs of metallosis can often be absent in the early disease and subtle signs of instability must be looked out for even in the absence of obvious misalignment in radiographic assessment. Metallosis when combined with malposition or malalignment can be more detrimental. If detected early before osteolysis and periarticular soft tissue damage sets in, a complete revision of all the implant components and abductor damage can be avoided. In suspected cases, a lower threshold should be adopted for sending blood and joint aspirates for cobalt-chromium levels.

Clinical Characteristics and Treatment Outcomes of Patients With Newly Diagnosed Takayasu Arteritis in Japan During the First 2 Years of Treatment　- A Nationwide Retrospective Cohort Study.

BACKGROUND: This study aimed to clarify recent clinical features and treatment outcomes in Japanese patients with newly diagnosed Takayasu arteritis (TAK) during the first 2 years of treatment. METHODS AND RESULTS: A nationwide multicenter retrospective cohort study for TAK was implemented to collect data between 2007 and 2014. The primary outcome of the study was clinical remission at Week 24. Of the 184 participants registered, 129 patients with newly diagnosed TAK were analyzed: 84% were female and the mean age at onset was 35 years. Clinical symptoms at diagnosis were mostly associated with large-vessel lesions. Frequent sites of vascular involvement included the carotid artery, subclavian artery, aortic arch, and descending aorta. The mean initial dose of prednisolone administered was 0.68 mg/kg/day, and 59% and 17% of patients received immunosuppressive drugs and biologics, respectively, by Week 104. Clinical remission at Week 24 and sustained clinical remission with daily prednisolone at ≤10 mg at Week 52 were achieved in 107 (82.9%) and 51 (39.5%) patients, respectively. The presence of signs and symptoms linked to large-vessel lesions was associated with failure to achieve sustained clinical remission at Week 52. CONCLUSIONS: We elucidated the clinical characteristics, treatment outcomes, and factors associated with failure to achieve sustained clinical remission in patients with newly diagnosed TAK in Japan during the first 2 years of treatment.

Clinical spectrum and outcome of children with deficiency of adenosine deaminase 2 (DADA2): multicentric experience from India.

OBJECTIVES: Deficiency of adenosine deaminase-2 (DADA2) is a monogenic disorder closely resembling polyarteritis nodosa (PAN) and can present to physicians across various specialties. Through this case series, we aim to describe the clinical spectrum and outcome of Indian children with DADA2. We aimed to study the clinical spectrum and outcome of Indian children with DADA-2. METHODS: The deidentified data from all participating centres were entered in an excel sheet, and the coordinating centre (All India Institute of Medical Sciences, New Delhi) screened the data for accuracy and completeness. RESULTS: We enrolled 16 children (11 females) in the study; the mean (SD) age at the time of onset of symptoms for males and females was 46.2 (47) and 73.6 (50.4) months, respectively. The most common clinical feature in this cohort was fever and rash in 80% of patients. More than half of children n, (%) [8, (53%)] had a CNS stroke. The other clinical features were hypertension [5(33%)], anaemia [3 (20%)] and arthralgia/arthritis in 4 (26%). These children were managed with various immunomodulators: steroids [13, (86%)], anti-TNF agents [(12, (80%)], cyclophosphamide [2 (13%)] and mycophenolate mofetil [3 (20%)]. The median (IQR) duration of follow-up for this cohort was 17 (10, 29) months. Fourteen children achieved remission and none had recurrent strokes after the initiation of anti-TNF drugs. CONCLUSION: DADA-2 closely resembles PAN; early age of onset and CNS stroke are striking differentiating features from classic PAN. Most children respond well to anti-TNF agents without serious adverse events during short-term follow-up.

Serum syndecan1 has the potential to reflect activity at diagnosis and predict death during follow-up in patients with ANCA-associated vasculitis.

OBJECTIVES: This study investigated whether serum syndecan1 at diagnosis reflects activity at diagnosis and predicts poor outcomes during follow-up in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). METHODS: The study included 79 patients with AAV from the cohort of Korean patients diagnosed with AAV. AAV-specific indices, including the Birmingham vasculitis activity score (BVAS), five-factor score (FFS), 36-item short-form survey (SF-36) physical and mental component summary (PCS and MCS), and vasculitis damage index (VDI), were assessed. Laboratory data including erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) levels were also collected. The highest tertile and upper half of the BVAS were tentatively defined as having high AAV activity. Serum syndecan1 levels were measured in sera stored at diagnosis. RESULTS: Serum syndecan1 at diagnosis was significantly correlated with AAV activity and functional status, as assessed by BVAS, FFS, SF-36 PCS, MCS, and acute-phase reactants, including ESR and CRP. Patients with serum syndecan1 ≥ 76.1 ng/mL at diagnosis, and those with serum syndecan1 ≥ 60.0 ng/mL at diagnosis showed significantly higher risks for the highest tertile and the upper half of BVAS at diagnosis than those without, respectively. Patients with serum syndecan1 ≥ 120.1 ng/mL at diagnosis had a significantly higher risk for all-cause mortality during follow-up than those without, and further, exhibited a significantly lower cumulative patients' survival rate than those without. CONCLUSION: Serum syndecan1 at diagnosis may not only reflect AAV activity at diagnosis but may also be associated with all-cause mortality during follow-up.

Identification and Quantification of Precursory Changes of Rheumatoid Vasculitis in the Dorsalis Pedis Artery.

OBJECTIVE: Rheumatoid arthritis (RA) is a systemic connective tissue autoimmune disease that can infiltrate arterial walls. The delay in diagnosis and treatment of rheumatoid vasculitis (RV) in patients with RA may lead to irreversible damage to the arterial walls of small-to-medium vessels, which has serious and devastating consequences, most notably lung and cardiac damage. In this work an ultrasound image-based biomarker was developed to detect precursory changes in RV. METHODS: The ground truth was initiated from a medical diagnosis of RA, with arterial wall thickening of the proximal dorsalis pedis artery (DPA) indicating precursory changes of RV identified with ultrasound scanning. Ultrasound images of the DPA from 49 healthy subjects in the control group and 46 patients in the RA group were obtained. In total, 187 texture features were extracted from the images, followed by principal component analysis and linear discriminant analysis. RESULTS: The proposed biomarker detected a significant difference between the two groups (p = 5.74 × 10-18) with an area under the receiver operating characteristic curve of 0.85. Ten major textural features contributing most heavily to the biomarker were identified, with these textures being consistent with clinical observations of RV identified in previous studies. Interscan reproducibility was assessed by computing the biomarker twice based on repeated scans of each ankle. High interscan reproducibility was demonstrated by a strong and significant Pearson's coefficient (r = 0.85, p < 0.01) between the two repeated measurements of the proposed biomarker. CONCLUSION: The proposed biomarker can discriminate image textural differences seen in images acquired from RA patients, demonstrating precursory changes in RV compared with healthy controls. The major discriminative features identified in this study may facilitate the early identification and treatment of RV.

Update on Takayasu arteritis: Year in review 2024.

Takayasu arteritis is an uncommon systemic inflammatory large vessel vasculitis affecting women in their third and fourth decades frequently. The disease poses considerable morbidity and mortality owing to the involvement of the aorta and its major branches. Treatment comprises medical and vascular interventions, tailored to each patient. We review the high-impact publications of the year 2023 up to April 2024, which provide great insight into clinical, biomarker, imaging, pathogenetic, and therapeutic updates.

COVID-19 vaccine-associated vasculitis: A systematic review.

Objectives: Post-COVID-19 vaccine-associated vasculitis stands as one of the most serious side effects attributed to COVID-19 vaccines. This complication encompasses diverse manifestations which vary in presentation and severity. Moreover, it can impact patients across all age groups, with a notably elevated incidence in the elderly. This systematic review seeks to review and evaluate the spectrum of vasculitis manifestations linked to COVID-19 vaccination. Methods: A systematic review of the literature was done by searching through PubMed, Google Scholar, and Scopus up to October 2022. Articles including data about sex, age at diagnosis, vasculitis clinical manifestations, type of vaccination, most commonly used investigations, comorbid medical conditions, treatments, and clinical outcomes were included in the final analysis. Furthermore, vasculitis flare-ups post-vaccination were considered part of this review. Results: A total number of 117 studies describing 158 patients developing vasculitis following COVID-19 vaccination were included in the final analysis. Among the patients who developed vasculitis, the most administered type of vaccination was the mRNA vaccine subtype (n = 103), followed by the viral vector vaccines (n = 42) and inactivated viral vaccines (n = 10). On the other hand, about 38% of vasculitis-related symptoms occurred after the administration of the first dose of the vaccine and 37% occurred after taking the second dose. The skin (60.7%) and the kidneys (27.8%) were the most affected organs and complete remission was achieved in 111 patients (70%), while partial remission occurred in 11% of the patient population. Conclusion: COVID-19 vaccine-induced vasculitis is a rare occurrence associated with COVID-19 vaccines. It generally presents a favorable prognosis and outcomes for the vast majority of patients, ultimately leading to full remission within days. This review emphasizes the notion that the advantages of COVID-19 vaccines outweigh the potential risks, particularly for individuals with compromised immune systems.

Progressive Acute Lower Extremity Ischemia Resulting From Cardiac Myxoma Embolization: A Case Report.

Cardiac myxomas are the most common primary benign tumors of the heart. The occlusion of peripheral arteries and complete obstruction of the abdominal aorta by a tumor embolus presents with distinct clinical manifestations. Herein, we present the case of a 38-year-old male with acute paresthesia, muscle weakness, erythematous, and violaceous changes in skin color localized to the dorsum of the left forefoot initially treated as cutaneous vasculitis. Further studies revealed the total occlusion of the terminal abdominal aorta by a saddle embolus from a cardiac myxoma. A multidisciplinary team consisting of cardiothoracic and vascular surgeons were involved in treating the patient, which resulted in full resolution of the case. This paper details the progression of acute bilateral limb ischemia to chronic limb threatening ischemia resulting from the total occlusion of the terminal abdominal aorta by a saddle embolus.

The coincidence of multiple sclerosis and primary vasculitis; from the bench of pathology to the bedside of treatment: a systematic review of case reports.

INTRODUCTION: Multiple sclerosis (MS) is a chronic, disabling neurodegenerative disease, leads to reduced quality of life. The increasing prevalence of MS around the world and its comorbidities increase its burden. Primary vasculitis subtypes, one of autoimmune diseases with different prevalence in different ages and genders, should be considered one of the important differential diagnosis in patients with MS. This study aims to verify the relationship between MS and primary vasculitis by conducting a systematic review. METHOD: We searched PubMed, Scopus, EMBASE, Web of Science, and Google Scholar, from January 1974 to July 2023. We included original articles that reported characteristics of patients involved with any type of Primary Vasculitis with MS. RESULT: From an initial 816 publications, 18 studies consisting of 18 individual patients from 14 countries with confirmed MS and one of different subtypes of primary vasculitis met the inclusion criteria. The female/male ratio was 0.38:1, the mean (SD) age was 40.44 (14.37) years with the range of 16 to 70 years old, and the relapsing/progressive ratio was 1.57:1. Most of them, 14 (77%) experienced MS before vasculitis, and mostly received Corticosteroids, interferon, cyclophosphamide, Glatiramer acetate as MS treatment. The concurrence of Takayasu Arteritis (2 cases), Polyarteritis Nodosa (2 cases), Churg-Strauss Syndrome (1 case), Wegener's Granulomatosis (2 cases), Microscopic Polyangiitis (1 case), Cutaneous leukocytoclastic vasculitis (5 cases), Good pasture's disease (5 cases) were reported with MS. CONCLUSION: Our study suggested that different primary vasculitis can be an important comorbidity of MS and can mimic its symptoms and MRI. Any atypical syndrome for PwMS, whether clinical or radiological, must be evaluated in terms of other differential diagnoses including vasculitis.

Unexpected Strikes: Sjögren Syndrome Triggering Multiple Evolving Cerebral Strokes in a Young Patient.

Neurological complications are observed less frequently with primary Sjögren syndrome (SS). The central nervous system (CNS) has seldom been shown to exhibit symptoms of SS, making the diagnosis of SS with neurological involvement difficult. We present a rare case scenario in which a young 23-year-old male presenting with an acute history of fever, headache, vomiting, altered sensorium, and seizures was admitted and diagnosed as a sub-acute infarct in the right frontal-parietal-temporal lobes on a computed tomography (CT) scan. Upon further examination, laboratory investigations were suggestive of viral encephalitis. The patient was treated accordingly with antiviral drugs, and the patient improved. The patient took "discharge against medical advice" after 12 days, only to return to the hospital with similar complaints within 15 days. Magnetic resonance imaging (MRI) was done, which suggested an acute evolving infarct in the right frontal and parietal lobe, and further evaluation yielded a diagnosis of SS. The patient was treated with high-dose steroids for seven days. A repeat MRI showed new acute infarcts with dilatation of the ventricular system with periventricular ooze. The patient could not be revived and succumbed after one week of steroid therapy.

Amelioration of immunoglobulin A vasculitis by suppression of the pathological expansion of T follicular helper 17 cells.

The main pathogenic features of immunoglobulin A vasculitis (IgAV) are overactive B cells and elevated production of IgA, which requires help from T follicular helper 17 (Tfh17) cells. To evaluate the pathological role of Tfh17 cells in IgAV, we investigated the mechanism responsible for Tfh17 differentiation and explored how to ameliorate IgAV by modulating Tfh17 generation. Peripheral blood mononuclear cells from IgAV patients were analyzed by flow cytometry. In vitro culture was performed to assess the modulation of cytokine-induced phenotypes. IgAV rats were used to explore the therapeutic effects of IL-6 blockade and the regulatory functions of IL-6 in Tfh17 cells. Serum cytokine and IgA levels were measured by ELISA while histopathological changes were evaluated by H&E,PAS or immunofluorescence staining. Frequency of CD4+CXCR5+CCR6+ Tfh17 cells were increased in IgAV patients and associated with disease severity. There was also a significant infiltration of Tfh17 cells in the kidney of human IgAV nephritis patients. IL-6 promoted the dendritic cell production of TGF-ß and Tfh17 differentiation. In IgAV rats, the in vivo blockade of IL-6 signaling inhibited Tfh17 differentiation, resulting in reduction of the germinal center and IgA production. Suppression of Tfh17 cells using IL-6 blockade greatly ameliorated clinical symptoms such as hemorrhagic rash and bloody stool and decreased IgA deposition and mesangial proliferation in the kidney in IgAV rats. Our findings suggest that suppression of Tfh17 differentiation can alleviate IgA-mediated vasculitis and may permit the development of tailored medicines for treating IgAV.