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.

Acute Renal Failure and Generalized Weakness in a 75-Year-Old Male With Pauci-Immune Necrotizing Antineutrophil Cytoplasmic Antibody (ANCA)-Associated Vasculitis: A Case Report.

We describe a 75-year-old male who presented to the emergency department with generalized weakness and was ultimately diagnosed with acute renal failure secondary to pauci-immune necrotizing antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. The patient's clinical course was complicated by a perforated gastric ulcer and severe malnutrition, necessitating involvement from multiple specialists. The case highlights the challenges of this rare vasculitis and the complications that can arise from the disease and its treatment. .

Novel Inflammatory Markers Associated With Cutaneous Leukocytoclastic Vasculitis Etiology.

Objectives: We aimed to compare inflammatory markers and determine their potential role in distinguishing secondary leukocytoclastic vasculitis (SLV) from idiopathic leukocytoclastic vasculitis (ILV). Materials and Methods: We included in this cross-sectional study patients with cutaneous leukocytoclastic vasculitis (CLV) diagnosed on cutaneous biopsy. We assessed clinical and laboratory data and then calculated platelet-to-lymphocyte ratio (PLR), neutrophil-to-lymphocyte ratio (NLR), C-reactive protein (CRP)-to-albumin ratio (CAR), and fibrinogen-to-albumin ratio (FAR). We have also defined the number of positive etiological examination (NPE) as the sum in a unique patient of the positive paraclinical examinations involved in the etiological assessment of CLV. Results: In total 77 patients were included, with 52 SLV group patients and 25 in the ILV group, mean age was 44+/-18 vs 49+/-21, and gender ratio was 29/23 vs 11/14. Comparison of PLR, NLR, CAR, and FAR showed significant differences in mean values between SLV and ILV groups with 199.1 (117.3-309.8) vs 126.8 (79-193) (P = 0.01) for PLR, 3.6 (1.9-5.1) vs 2.3 (1.7-3.4) (P = 0.048) for NLR, 1.9 mg.g-1 (0.4-3.6) vs 0.6 mg g-1 (0.2-1.9) (P = 0.043) for CAR, and 155.8 mg.g-1 (90.7-192.3) vs 108.7 mg.g-1 (82.2-148.1) (P = 0.034) for FAR. PLR, CAR, and FAR were positively correlated to NPE (r = 0.463, P < 0.001; r = 0.434, P < 0.001; and r = 0.411, P < 0.001, respectively), and there was no significant correlation between NLR and NPE (r = 0.165, P = 0.151). Conclusion: This is the first study to investigate PLR, NLR, CAR, and FAR in CLV, and it demonstrates that elevation of these ratios is associated with SLV, which leads us to suggest to exhaustively explore patients with elevated ratios.

Crinkle-Cut Coronary Arteries in a Patient With Granulomatosis With Polyangiitis.

Granulomatosis with polyangiitis (GPA) is a rare type of small to medium vessel necrotizing vasculitis that usually affects vessels of the upper or lower airways and kidneys. Cardiac involvement in GPA is often subclinical and if clinically significant has been rarely reported, even less so as an initial presentation. We describe the case of a 44-year-old man who presented with what appeared to be inferior ST-segment elevation myocardial infarction and was found to have small vessel vasculitis of the coronary arteries with associated myocarditis as a presenting manifestation of GPA, which was ultimately treated with steroids, rituximab, and avacopan.

Normocomplementemic Urticarial Vasculitis Following Influenza Vaccination: A Case Report and Review of the Literature.

Urticarial vasculitis is characterized by persistent urticarial lesions lasting over 24 h. Urticarial vasculitis is often triggered by medications, infections, and autoimmune disorders. However, vaccinations against viral and bacterial pathogens have recently been documented to induce urticarial vasculitis. We describe the case of a 67-year-old woman who was presented with an extensive erythematous and purpuric rash without systemic symptoms 3 days after an influenza vaccination. She was diagnosed with normocomplementemic urticarial vasculitis based on clinical findings, normal complement levels, and histopathological findings of leukocytoclastic vasculitis. After receiving oral histamines, she showed complete resolution 3 months after receiving the influenza vaccination. Although vaccination-associated vasculitis is common, urticarial vasculitis following vaccinations is rare. We reviewed 13 cases of urticarial vasculitis following a wide range of vaccines, including those against Bacillus Calmette-Guérin, serogroup B meningococcus, influenza, and coronavirus disease. We conducted a comprehensive review of various aspects, including age, sex, past medical history, type of vaccination, number of vaccinations, onset time, cutaneous symptoms, place of eruption, systemic symptoms, laboratory disorders, treatment period, and treatment of urticarial vasculitis. Two patients developed hypocomplementemic urticarial vasculitis after vaccination, and both experienced systemic symptoms such as arthralgia and fever. In this review, no significant differences were found in the data, which may be attributed to the small number of cases. The mechanisms underlying the induction of urticarial vasculitis by vaccines remain unknown; however, in addition to immune complex deposition and complement activation due to vaccine components, molecular mimicry may trigger urticarial vasculitis by producing vaccine-derived pathogenic antigen antibodies. This case study emphasizes the need for heightened awareness and further investigation of urticarial vasculitis as a rare adverse effect of vaccination.

Takayasu arteritis and interstitial lung disease: a case report and literature review.

Introduction and importance: Although pulmonary artery involvement is well recognized, the incidence of interstitial lung disease (ILD) with Takayasu arteritis is very rare. The pathophysiology of ILD in Takayasu is still incompletely understood, in contrast to several studies establishing the relationship between ANCA-associated vasculitis and ILD. The management of this patient involved a multidisciplinary approach with long-term follow-up. Case presentation: The authors present a case of HRCT-proven interstitial lung disease in a patient with Takayasu arteritis and heart failure. The patient was on long-term corticosteroids on and off for several years and recently developed progressive dyspnea with a dry cough. After reviewing her history and physical examination, pulmonary function tests (PFTs) and high-resolution computed tomography (HRCT) were performed, and interstitial lung disease was diagnosed. The patient was managed by a team of pulmonologists, rheumatologists, and cardiologists and gradually improved after adjustment of medications, including corticosteroids and mycofenolate, and via long-term oxygen therapy. Clinical discussion: Takayasu arteritis is a rare form of systemic vasculitis that can involve the pulmonary vasculature, such vasculitis with associated parenchymal involvement is rare. ILDs have been demonstrated with ANCA-associated vasculitis; however, whether the pathophysiology applies to Takayasu is unknown. Since Takayasu can be debilitating to the patient, the association of ILDs can have further prognostic implications. Given that no established guidelines exist to address this association, management is based on clinical expertise. Conclusion: The authors report a case of Takayasu arteritis and associated ILD and its pharmacological management. Takayasu arteritis is a very uncommon type of vasculitis, and pulmonary parenchymal involvement further contributes to this case's rarity. As the management of Takayasu arteritis alone is cumbersome, the addition of another significant comorbidity, such as ILD, can pose several threats to the patient. Given the rarity of this association, no established guidelines exist, making clinical expertise crucial for managing such patients. Further research is needed to explore the underlying mechanisms and develop evidence-based treatment strategies for this rare combination.

Efficacy and Safety of Mepolizumab in Patients With Eosinophilic Granulomatosis With Polyangiitis: A Single-Center Experience.

Eosinophilic granulomatosis with polyangiitis (EGPA), previously known as Churg-Strauss syndrome, is a systemic vasculitis characterized by eosinophil-rich, necrotizing granulomatous inflammation primarily affecting the respiratory tract with necrotizing vasculitis of small- to medium-sized arteries. In this case series, we retrospectively evaluated the efficacy and safety of mepolizumab in seven patients diagnosed with EGPA who presented to the Department of Allergy and Clinical Immunology at Cleveland Clinic Abu Dhabi. The variables assessed before and after mepolizumab treatment included Birmingham Vasculitis Activity Score (BVAS), prednisolone dose, Asthma Control Test (ACT) score, and blood eosinophil count (BEC). We found a significant reduction in BVAS and prednisolone dosage with clinical improvements in asthma symptoms after treatment with mepolizumab. Our case series, the first from the Middle East on the use of mepolizumab in EGPA, demonstrates that mepolizumab is a safe and effective treatment for patients with EGPA.

An Unusual Complication of Lisinopril: A Report of Iatrogenic Henoch-Schönlein Purpura.

Henoch-Schönlein purpura (HSP), also known as IgA vasculitis, is a hypersensitivity vasculitis characterized by palpable purpuric lesions associated with polyarthralgia, abdominal discomfort, and renal involvement. We present the case of a 41-year-old man who was admitted to the emergency department due to generalized purpuric lesions and abdominal pain. During the complementary study, there was no evidence of thrombocytopenia or coagulopathy but confirmed microscopic haematuria. The diagnosis of HSP was supported by the presence of leukocytoclastic vasculitis with perivascular IgA deposits in the skin biopsy. After excluding infectious, autoimmune, and neoplastic pathologies, the possibility of HSP associated with taking lisinopril, which had been recently initiated after hospitalization for acute heart failure, was assumed. Angiotensin-converting enzyme (ACE) inhibitor suspension and treatment with systemic corticosteroids lead to significant clinical regression, supporting our suspicion.

The real-world use and effectiveness of avacopan in routine practice for the treatment of ANCA vasculitis. First experiences in Spain.

OBJECTIVES: ANCA-associated vasculitis (AAV) are chronic diseases with relapses that associate organic damage because of the disease and its treatment. Avacopan is a new treatment indicated for AAV. We present the first experiences with avacopan in Spain as part of an Early Access program. METHODS: Patients with AAV who started avacopan between June 2022-September 2023 were included. For comparison, a historical cohort of patients diagnosed with AAV around the same time and treated without avacopan was also included. RESULTS: 29 patients treated with avacopan were analyzed. Twelve patients (41.4%) were male, median age was 56 years. Most of patients were ANCA MPO positive (21/29, 72.4%). The most frequently affected organ was the kidney (23/29, 79.31%), with a mean eGFR of 23.2 ml/minMedian follow-up was 456.8 ± 181.7 days with a remission rate of 86.2%. eGFR increased from 23.2 ± 11.2-38.38 ± 18.55 ml/min after 12 months of diagnosis.2 patients had Adverse Events related to avacopan as severe neutropenia and a gastrointestinal affectation , 13 infections were reported and 1 death.Patients treated with avacopan received a significantly lower cumulative dose of prednisone at 6 and 12 months (p-values of 0.02 and <0.01, respectively) compared with historical controls. The evolution of GFR at 1 year of follow-up and the incidence of relapse were similar in both groups. CONCLUSION: The combination of avacopan in clinical practice presents a good safety profile and provides added value by contributing to the control of AAV activity, increase GFR, and removal of steroids.

[Update on Behçet syndrome]. / Neues zum Behçet-Syndrom.

BACKGROUND: Behçet syndrome (BS) is a vasculitis of variable vessels with multiple organ manifestations. OBJECTIVE: This article gives an overview of innovations in the last 2 years. MATERIAL AND METHODS: A literature search was carried out using the keyword "Behcet" in 2022-2024 in PubMed. The selection of suitable articles was based on the relevance. RESULTS AND CONCLUSION: With respect to the pathophysiology it is now clear that BS occupies an intermediate position between autoinflammatory and autoimmune clinical pictures. It is now classified as MHC-I-opathy, i.e., a disease that has a strong association with HLA class I antigens, which also play a prominent role in the pathogenesis. The diagnostic international criteria for Behcet's disease (ICBD) from 2014 with a score of 4 points or more that makes the diagnosis of BS probable have become established; however, in countries with a low prevalence of BS, the differential diagnosis of BS from other diseases is difficult and a higher point limit in the diagnostic score seems to make sense in order to avoid incorrect diagnoses. Clusters or phenotypes of the disease have now been described in various countries in which different symptom complexes frequently occur together; however, the clusters differ between the different countries of origin and depending on the age of the patients. Sonography of the common femoral vein with specific wall thickening in BS patients has been established as an additional tool for the differential diagnosis. Typical characteristics of oral aphthae in BS were also described and the frequency of positivity in the pathergy test could be significantly increased using pneumococcal antigens as the reagent. The treatment recommendations of the EULAR from 2018 still apply; in treatment-refractory cases, tocilizumab, secukinumab, Janus kinase inhibitors (JAKi) and ustekinumab have now also been successfully used. The new EULAR treatment recommendations are expected in 2025.

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.

Ferulic acid suppresses the inflammation and apoptosis in Kawasaki disease through activating the AMPK/mTOR/NF-κB pathway.

Background: Kawasaki disease (KD) is a self-limiting and acute systemic vasculitis of unknown etiology, mainly affecting children. Ferulic acid (FA), a natural phenolic substance, has multiple pharmacological properties, including anti-inflammatory, anti-apoptosis, and anti-fibrosis, and so on. So far, the protective effects of FA on KD have not been explored. Methods: In this study, we established Candida albicans water soluble fraction (CAWS)-induced mouse coronary artery vasculitis of KD model and the tumor necrosis factor α (TNF-α)-induced human umbilical vein endothelial cells (HUVECs) injury model to investigate the anti-inflammatory and anti-apoptosis effects of FA on KD, and try to elucidate the underlying mechanism. Results: Our in vivo results demonstrated that FA exerted anti-inflammatory effects on KD by inhibiting the infiltration of CD45-positive leukocytes and fibrosis around the coronary artery. Additionally, FA downregulated the levels of inflammatory and chemotactic cytokines, alleviated splenomegaly, and exhibited anti-apoptotic effects on KD by reducing TUNEL-positive cells, downregulating BAX expression, and upregulating BCL-2 expression. In addition, Our in vitro findings showed that FA could effectively inhibit TNF-α-induced HUVEC inflammation like NF-κB inhibitor QNZ by downregulating the expression of pro-inflammatory cytokines as well as attenuated TNF-α-induced HUVEC apoptosis by reducing apoptotic cell numbers and the BAX/BCL-2 ratio, which could be reversed by the AMPK inhibitor compound c (CC). The further mechanistic study demonstrated that FA could restrain vascular endothelial cell inflammation and apoptosis in KD through activating the AMPK/mTOR/NF-κB pathway. However, FA alone is hard to completely restore KD into normal condition. Conclusion: In conclusion, FA has potential protective effects on KD, suggesting its promising role as an adjuvant for KD therapy in the future.

mTOR signalling controls the formation of smooth muscle cell-derived luminal myofibroblasts during vasculitis.

The accumulation of myofibroblasts within the intimal layer of inflamed blood vessels is a potentially catastrophic complication of vasculitis, which can lead to arterial stenosis and ischaemia. In this study, we have investigated how these luminal myofibroblasts develop during Kawasaki disease (KD), a paediatric vasculitis typically involving the coronary arteries. By performing lineage tracing studies in a murine model of KD, we reveal that luminal myofibroblasts develop independently of adventitial fibroblasts and endothelial cells, and instead derive from smooth muscle cells (SMCs). Notably, the emergence of SMC-derived luminal myofibroblasts-in both mice and patients with KD, Takayasu's arteritis and Giant Cell arteritis-coincided with activation of the mechanistic target of rapamycin (mTOR) signalling pathway. Moreover, SMC-specific deletion of mTOR signalling, or pharmacological inhibition, abrogated the emergence of luminal myofibroblasts. Thus, mTOR is an intrinsic and essential regulator of luminal myofibroblast formation that is activated in vasculitis patients and therapeutically tractable. These findings provide molecular insight into the pathogenesis of coronary artery stenosis and identify mTOR as a therapeutic target in vasculitis.

Granulomatosis with polyangiitis: clinical characteristics and updates in diagnosis.

Granulomatosis with polyangiitis (GPA) is a rare systemic disease characterized by granulomatous inflammation of the respiratory tract and necrotizing vasculitis of small and medium vessels often associated with the production of anti-neutrophil cytoplasmic antibodies (ANCA) directed mainly against leukocyte proteinase 3 (PR3). Usually, it involves upper airways, lungs, and kidneys, however any organ may be affected. The diagnosis is based on clinical, radiological, and serological findings. Biopsies, although strongly recommended, are not always feasible and often provides non-specific features. ANCA plays a crucial role in the diagnosis of GPA; nevertheless, ANCA detection is not a substitute for biopsy, which plays an important role in suspected cases, particularly when histological confirmation cannot be obtained. Significant advances have been made in classification criteria and phenotyping of the disease, particularly in determining the nuances between PR3-ANCA and myeloperoxidase (MPO)-ANCA vasculitis. This has led to better characterization of patients and the development of targeted treatment in the future. In addition, better identification of cytokine and immunological profiles may result in immuno-phenotyping becoming a new approach to identify patients with ANCA-associated vasculitis (AAV). Due to the chronic relapsing-remitting nature, strict follow-up of GPA is necessary to provide appropriate management. The search for the accurate marker of disease activity and to predict relapse is still ongoing and no predictor has been found to reliably guide therapeutic decision-making.

Gastrointestinal Vasculitis in Adult Patient With Dermatomyositis Presenting as Hematemesis.

Dermatomyositis (DM) is an inflammatory disease of the muscles and skin. Severe gastrointestinal (GI) involvement, characterized by GI bleeding and perforation secondary to underlying vasculopathy, is rarely seen. We describe a case of newly diagnosed DM in a 75-year-old woman who presented with a rash and muscle weakness. She then had sudden onset of hematemesis and was found to have duodenal ulcers due to leukocytoclastic vasculitis from her DM. Our aim was to highlight the need for recognition of GI involvement in adults with DM.

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.

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.