Specialty drug use for autoimmune conditions varies by race and wage among employees with employer-sponsored health insurance.

BACKGROUND: The relationship between race and ethnicity, wage status, and specialty medication (SpRx) use among employees with autoimmune conditions (AICs) is poorly understood. Insight into sociodemographic variations in use of these medications can inform health equity improvement efforts. OBJECTIVE: To assess the association of race and ethnicity and wage status on SpRx use and adherence patterns among employees with AICs enrolled in employer-sponsored health insurance. METHODS: In this observational, retrospective cohort analysis, data were obtained from the IBM Watson MarketScan database for calendar year 2018. Employees were separated into race and ethnicity subgroups based on employer-provided data. Midyear employee wage data were used to allocate employees into the following annual income quartiles: $47,000 or less, $47,001-$71,000, $71,001-$106,000, and $106,001 or more. The lowest quartile was further divided into 2 groups ($35,000 or less and $35,001-$47,000) to better evaluate subgroup differences. Outcomes included monthly days SpRx-AIC supply, proportion of days covered (PDC), and medication discontinuation rates. Generalized linear regressions were used to assess differences while adjusting for patient and other characteristics. RESULTS: From a sample of more than 2,000,000 enrollees, race and ethnicity data were available for 617,117 (29.8%). Of those, 47,839 (7.8%) were identified as having an AIC of interest, with prevalence rates of AICs differing by race within wage categories. Among those with AICs, 5,358 (11.2%) had filled at least 1 SpRx-AIC prescription. Following adjustment, except for the highest wage category, prevalence of SpRx-AIC use was significantly less among Black and Hispanic subpopulations. Black patients had significantly lower SpRx-AIC use rates than White patients (≤$35,000: 4.9 vs 9.4%, >$35,000-$47,000: 5.5 vs 10.6%, >$47,000-$71,000: 8.5 vs 11.1%, and >$71,000-$106,000: 9.1 vs 12.7%; P <0.001 for all). For Hispanic patients, prevalence rates were significantly lower than White patients in 3 different wage categories (≤$35,000: 4.5 vs 9.4%, >$35,000-$47,000: 6.1 vs 10.6%, and >$71,000-$106,000: 8.6 vs 12.7%; P < 0.001). PDC and 90-day discontinuation rates did not differ among race and ethnicity groups within the respective wage bands. CONCLUSIONS: Race and ethnicity and wage-related disparities exist in SpRx use, but not PDC or discontinuation rates for treatment of AICs among non-White and low-income populations with employer-sponsored insurance, and may adversely impact clinical outcomes.

Napkin math can change the world.

Mathematical models hold the key to equitable patient care.

Assessment of adherence to aspirin for preeclampsia prophylaxis and reasons for nonadherence.

BACKGROUND: Preeclampsia is a hypertensive disease unique to pregnancy and has a significant impact on maternal and neonatal morbidity and mortality. Daily aspirin has been demonstrated to reduce the risk of preeclampsia. The American College of Obstetricians and Gynecologists recommends daily low-dose aspirin, ideally before 16 weeks' gestation, in at-risk patients for preeclampsia risk reduction. This study examined whether patients at-risk for preeclampsia by the American College of Obstetricians and Gynecologists criteria recalled aspirin recommendation and factors associated with treatment adherence. OBJECTIVE: This study examined whether patients at-risk for preeclampsia by the American College of Obstetricians and Gynecologists criteria recalled aspirin recommendation and factors associated with treatment adherence. STUDY DESIGN: This study used an anonymous written survey. Pregnant patients were asked to record self-reported risk factors and to recall recommendation to take aspirin for preeclampsia prophylaxis. Participants were then determined to be high-, moderate-, or low-risk on the basis of the American College of Obstetricians and Gynecologists guidelines. Self-reported adherence to recommendations and factors contributing to the patients' decisions to take or decline aspirin were assessed. Secondary outcomes included demographic characteristics of adherent patients and patients who did not recall aspirin recommendation. RESULTS: A total of 544 surveys were distributed and 500 were returned (91.9% response rate). Of the 104 high-risk pregnancies identified, aspirin was recommended in 60 (57.7%; 95% confidence interval, 0.48-0.67). Of the 269 patients with 2 or more moderate-risk factors, aspirin was recommended for 13 (4.8%; 95% confidence interval, 0.03-0.08). Among the participants who recalled aspirin recommendation, adherence was similar between high-risk (81.7%) and moderate-risk (76.9%) groups (P=.69). Patients with chronic hypertension, a history of preeclampsia or gestational hypertension in a previous pregnancy, and pregestational diabetes mellitus were most likely to report receiving aspirin recommendation (78.8%, 76.5%, 63.8%, and 53.3%, respectively). No high-risk factor was associated with a decreased likelihood of adherence. Nonadherent patients rarely discussed their decision with their medical provider (5.9%). In the 42.3% of high-risk participants who did not recall aspirin recommendation, autoimmune disease, multiple gestation, and kidney disease were the most prevalent risk factors (42.9%, 35.7%, and 25.0%, respectively). CONCLUSION: In the population studied, many at-risk patients, as defined by the American College of Obstetricians and Gynecologists criteria, did not recall recommendations to take aspirin for preeclampsia prophylaxis. This raises concerns for absent or ineffective counseling. Of the patients who recalled aspirin recommendation, most reported adherence, and a history of hypertensive disorders or preeclampsia, autoimmune disease, and pregestational diabetes mellitus were most often associated with adherence. There was no single factor most strongly associated with intentional nonadherence.

Preclinical Immunopharmacologic Assessment of KPL-404, a Novel, Humanized, Non-Depleting Antagonistic Anti-CD40 Monoclonal Antibody.

The CD40/CD40L pathway plays a major role in multiple inflammatory processes involving different immune and stromal cells. Abnormal activation of this pathway has been implicated in pathogenesis of complex autoimmune diseases including systemic lupus erythematosus, rheumatoid arthritis, Graves' disease, and Sjogren's Syndrome. We completed in vitro and in vivo preclinical characterization of KPL-404, a novel humanized anti-CD40 IgG4 monoclonal antibody, to demonstrate its potency, efficacy, and pharmacokinetic profile; safety was also assessed. In vitro, KPL-404 bound recombinant human and cynomolgus monkey CD40 with comparable affinity in the nanomolar range. KPL-404 binding to cell surface CD40 did not induce antibody- or complement-mediated cytotoxicity of CD40-expressing cells. Pharmacological antagonistic activity of KPL-404 was demonstrated in vitro by inhibition of CD40-mediated downstream NF-kB activation. In the in vivo study with cynomolgus monkeys, KPL-404, administered intravenously as a single dose (10 mg/kg) or two monthly doses of 1 or 5 mg/kg, did not elicit observable safety findings, including thrombocytopenia over 8 weeks. KPL-404 engaged CD40 expressed on peripheral B cells for 2 and 4 weeks after a single administration of 5 or 10 mg/kg IV, respectively, without depletion of peripheral B cells. At 5 mg/kg IV, KPL-404 blocked both primary and secondary responses to T-cell dependent antibody responses to test antigens, KLH, and tetanus toxoid. These data illustrated the relationship between KPL-404 serum concentration and pharmacodynamic effects of CD40-targeting in circulation and in lymphoid tissues. These data support clinical development of KPL-404 in autoimmune diseases. SIGNIFICANCE STATEMENT: We aimed to develop a potent and efficacious CD40 antagonist. In vitro and in vivo findings show that KPL-404 blocks the anti-CD40 antibody that potently inhibits primary and secondary antibody responses at pharmacologically relevant concentrations, has a favorable pharmacokinetic profile, and does not deplete B cells by antibody-dependent cellular cytotoxicity or apoptosis ("nondepleting"). These findings support clinical development of KPL-404 as a potential therapeutic in autoimmune diseases.

COVID-19: Overview of rheumatology fellows.

SARS-COV-2 infection has spread worldwide since it originated in December 2019, in Wuhan, China. The pandemic has largely demonstrated the resilience of the world's health systems and is the greatest health emergency since World War II. There is no single therapeutic approach to the treatment of COVID-19 and the associated immune disorder. The lack of randomised clinical trials (RCTs) has led different countries to tackle the disease based on case series, or from results of observational studies with off-label drugs. We as rheumatologists in general, and specifically rheumatology fellows, have been on the front line of the pandemic, modifying our activities and altering our training itinerary. We have attended patients, we have learned about the management of the disease and from our previous experience with drugs for arthritis and giant cell arteritis, we have used these drugs to treat COVID-19.

Subepithelial autoimmune bullous dermatoses disease activity assessment and therapy.

Subepidermal (subepithelial) autoimmune blistering dermatoses are a group of rare skin disorders characterized by the disruption of the dermal-epidermal junction through the action of autoantibodies. The fourth article in this continuing medical education series presents the current validated disease activity scoring systems, serologic parameters, treatments, and clinical trials for bullous pemphigoid, mucous membrane pemphigoid, epidermolysis bullosa acquisita, bullous systemic lupus erythematosus, anti-p200 pemphigoid, linear IgA bullous dermatosis, and dermatitis herpetiformis.

Assessment of Treatment Safety and Quality of Life in Patients Receiving Etanercept Biosimilar for Autoimmune Arthritis (ASQA): A Multicenter Post-marketing Surveillance Study.

INTRODUCTION: Phase IV post-marketing surveillance studies are needed to evaluate the real-world safety and effectiveness of drug products. This study aimed to evaluate the safety and effectiveness of biosimilar etanercept (Altebrel, AryoGen Co., Iran) in patients with rheumatoid arthritis (RA), ankylosing spondylitis (AS), and psoriatic arthritis (PsA). METHODS: In this open-label, multicenter, prospective, observational, post-marketing surveillance study, 583 patients received biosimilar etanercept 25 mg twice weekly or 50 mg once weekly and were followed up to 12 months. The primary objective was to evaluate the safety of biosimilar etanercept by documenting all the adverse events in the case report forms throughout the study period. The secondary objective was to evaluate the effectiveness of biosimilar etanercept in study patients, where longitudinal changes in health assessment questionnaire (HAQ), pain, and disease activity scores were assessed. RESULTS: A total of 583 patients (44.80 ± 13.09 years of age) were included and followed for an average of 8.12 ± 3.96 months. Among all patients, 172 (29.50%) experienced at least one adverse event, and injection site reaction, abdominal pain, and upper respiratory tract infection were the most common. HAQ scores decreased from 1.32 ± 0.77 at baseline to 0.81 ± 0.61 at 12 months in patients with RA/PsA (p < 0.01) and from 0.82 ± 0.58 at baseline to 0.66 ± 0.63 at 12 months in patients with AS (p = 0.18). Pain scores decreased from 6.49 ± 2.41 at baseline to 3.51 ± 2.39 at 12 months (p < 0.01). CONCLUSION: The results demonstrated the real-world safety and effectiveness of biosimilar etanercept in patients with RA, PsA, and AS. TRIAL REGISTRATION: ClinicalTrials.gov identifier NCT04582084.

Impact of pharmacist care in the management of autoimmune disorders: A systematic review of randomized control trials and non-randomized studies.

INTRODUCTION: Autoimmune disorders are chronic, self-mediated, misdirected immune responses against their own immune system. It required intensive, complex and costly drug treatment regimen increased the risk of pharmacotherapy error and adversely affects patients. Hence, pharmacist care will have vital roles in autoimmune disorders to achieve health related outcomes. OBJECTIVES: This review aimed to gather evidence on the impact of pharmacist care on clinical, humanistic and economic outcomes, adherence to medications, and drug related problems in the management of autoimmune disorders among the usual care group. METHODOLOGY: A comprehensive review conducted in compliance with the PRISMA statement and systematic search was performed across five databases included PubMed Central, Web of Sciences, Scopus, Cochrane Library andgoogle scholar from inception until August 2020. This research included full-text articles of randomized and non-randomized studies that evaluated impact of pharmacist care in autoimmune disorders. RESULTS: A total of nine studies were included (seven RCTs and two non-RCTs), including 829 patients with autoimmune disorders. A total of four studies (80%) show an enhancement in at least one clinical parameter due to pharmacist care. A substantial improvement in at least one humanistic parameter observed in all five studies (100%). While four out of five studies (80%) clearly displayed a remarkable improvement in medication adherence in the pharmacist care group from baseline to the completion of follow-ups. One study quantified a 99.08% resolution of DRPs in the pharmacist care group. Another study estimated the cost of medical resources uses per patient and found no difference in cost-effectiveness over six months between groups. CONCLUSION: This review reinforces the vital contribution of pharmacists to achieve clinical outcomes, humanistic outcomes, adherence to medications and DRPs in the efficient management of autoimmune disorders. However, no notable changes in economic outcomes were observed in this review.

Assessment of Placental Disposition of Infliximab and Etanercept in Women With Autoimmune Diseases and in the Ex Vivo Perfused Placenta.

Tumor necrosis factor (TNF) inhibitors are increasingly applied during pregnancy without clear knowledge of the impact on placenta and fetus. We assessed placental transfer and exposure to infliximab (n = 3) and etanercept (n = 3) in women with autoimmune diseases. Furthermore, we perfused healthy term placentas for 6 hours with 100 µg/mL infliximab (n = 4) or etanercept (n = 5). In pregnant women, infliximab transferred into cord blood but also entered the placenta (cord-to-maternal ratio of 1.6 ± 0.4, placenta-to-maternal ratio of 0.3 ± 0.1, n = 3). For etanercept, a cord-to-maternal ratio of 0.04 and placenta-to-maternal ratio of 0.03 was observed in one patient only. In ex vivo placenta perfusions, the extent of placental transfer did not differ between the drugs. Final concentrations in the fetal compartment for infliximab and etanercept were 0.3 ± 0.3 and 0.2 ± 0.2 µg/mL, respectively. However, in placental tissue, infliximab levels exceeded those of etanercept (19 ± 6 vs. 1 ± 3 µg/g, P < 0.001). In conclusion, tissue exposure to infliximab is higher than that of etanercept both in vivo as well as in ex vivo perfused placentas. However, initial placental transfer, as observed ex vivo, does not differ between infliximab and etanercept when administered in equal amounts. The difference in placental tissue exposure to infliximab and etanercept may be of clinical relevance and warrants further investigation. More specifically, we suggest that future studies should look into the occurrence of placental TNF inhibition and possible consequences thereof.

JAK inhibitors in 2019, synthetic review in 10 points.

JAK inhibitors are recent treatments. Many publications have appeared in recent years, exposing treatment efficiencies in phases 2 and 3 studies, or their tolerance profile in various rheumatological diseases. We propose here a systematic review of JAK inhibitors, from their mechanism of physiological action up to the estimation of their current risk benefit balance, and their possible future applications. In order to better synthesize the data, we organized this review into 10 essential points. 1- What is the role of JAK/Stat pathway? 2- How can a single signaling pathway regulate as many different signals? 3- What are the commercialized JAK inhibitors and their validated indications in humans today? 4- What is the level of efficiency of JAK inhibitors in inflammatory diseases? 5- What is the delay of efficiency of JAK inhibitors? 6- Where is the place of JAK inhibitors in the therapeutic strategy today? 7- What is the infectious tolerance profile of JAK inhibitors? 8- What is the non-infectious safety profile of JAK inhibitors? 9- What is the cost of JAK inhibitors compared to other DMARDs? 10- What future prospects for JAK inhibitors?

Pre-existing autoimmune disease and the risk of immune-related adverse events among patients receiving checkpoint inhibitors for cancer.

INTRODUCTION: Patients with pre-existing autoimmune diseases have been excluded from clinical trials of immune checkpoint inhibitors (ICIs) for cancer. Real-world evidence is necessary to understand ICI safety in this population. METHODS: Patients treated with ICIs from 2011 to 2017 were identified using data from a large health insurer. Outcomes included time to (1) any hospitalization; (2) any hospitalization with an irAE diagnosis; and (3) outpatient corticosteroid treatment. The key exposure was pre-existing autoimmune disease, ascertained within 12 months before starting ICI treatment, and defined either by strict criteria (one inpatient or two outpatient claims at least 30 days apart) or relaxed criteria only (any claim, without meeting strict criteria). RESULTS: Of 4438 ICI-treated patients, pre-existing autoimmune disease was present among 179 (4%) by strict criteria, and another 283 (6%) by relaxed criteria only. In multivariable models, pre-existing autoimmune disease by strict criteria was not associated with all-cause hospitalization (HR 1.27, 95% CI 0.998-1.62), but it was associated with hospitalization with an irAE diagnosis (HR 1.81, 95% CI 1.21-2.71) and with corticosteroid treatment (HR 1.93, 95% CI 1.35-2.76). Similarly, pre-existing autoimmune disease by relaxed criteria only was not associated with all-cause hospitalization (HR 1.11, 95% CI 0.91-1.34), but was associated with hospitalization with an irAE diagnosis (HR 1.46, 95% CI 1.06-2.01) and corticosteroid treatment (HR 1.46, 95% CI 1.13-1.88). CONCLUSION: Pre-existing autoimmune disease was not associated with time to any hospitalization after initiating ICI therapy, but it was associated with a modest increase in hospitalizations with irAE diagnoses and with corticosteroid treatment.

Challenges of fertility preservation in non-oncological diseases.

Clinicians should provide fertility counseling to all patients receiving gonadotoxic treatment. International scientific societies have mainly focused on oncological patients, and fewer efforts have been made to apply these recommendations to women diagnosed with benign disease (eg benign hematological diseases, autoimmune diseases, and gynecological or genetic disorders). However, these indications account for 8%-13% of the demand for fertility preservation. The risk of premature ovarian failure due to treatment, or to the disease itself, can be considered fairly high for many young women. Counseling and adequate management of these women require particular attention due to the severe health conditions that are associated with some of these diseases. In this review, we address specific issues related to providing adequate fertility counseling and management for women who have been diagnosed with the major non-oncological indications, based on the literature and on our clinical experience.

Short-term costs associated with non-medical switching in autoimmune conditions.

OBJECTIVES: To estimate short-term costs associated with non-medical switch (NMS) from originator biologics to biosimilars among stable patients with autoimmune conditions in rheumatology, gastroenterology, and dermatology from a US provider's and third-party payer's perspective. METHODS: An economic model was constructed to estimate switching costs related to physician time and healthcare resource utilisation (HRU) at the initial NMS visit and over 3 months. The proportion of patients with relevant conditions treated with originators and expected NMS rate, physician time, HRU, and payer reimbursement were derived from a physician survey. Switching costs were estimated for a practice of 1,000 patients with relevant conditions by therapeutic area and for an insurance plan with 1 million individuals by therapeutic area and all areas combined. Switching cost drivers were assessed with one-way sensitivity analyses. RESULTS: Physicians expected extra 6 minutes for the NMS visit and 22 minutes over 3 months; NMS rates of 14.4%, 15.5%, and 17.7%; and 11.3%, 16.2%, and 33.2% of time not reimbursed for gastroenterology, rheumatology, and dermatology, respectively. The total switching costs for payer's were $771,460 (for n = 3,609 patients with an NMS rate of 16.6%), mostly due to follow-up visits and additional laboratory tests/procedures. In sensitivity analyses, the NMS rate was the main cost driver. Increasing the NMS rate to 25% and 50% increased payer's total switching costs to $1.19 and $2.39 million, respectively. CONCLUSIONS: Originator-to-biosimilar NMS in stable patients with autoimmune conditions could result in considerable switching costs for both providers and payers.

Biosimilars: An Approach to some Current Worldwide Regulation Frameworks.

Developing new biologics has led to regulations and norms aimed at guaranteeing their safety, quality and effectiveness, in terms of marketing, prescription, use, interchangeability and switching. Biologics are of great importance in treating patients suffering from rheumatic, autoimmune, inflammatory and neoplastic diseases. The expiry/lapse of reference biologics or originators' patents has meant that developing biosimilars involves accompanying legal requirements for their approval in countries worldwide. This paper has thus approached the situation of biosimilar regulation worldwide, the pertinent technical concepts and regulatory differences in some countries of interest.

A Targeted Literature Review Examining Biologic Therapy Compliance and Persistence in Chronic Inflammatory Diseases to Identify the Associated Unmet Needs, Driving Factors, and Consequences.

Chronic inflammatory diseases (CIDs) represent a substantial clinical and economic burden to patients, providers, payers and society overall. Biologics, such as tumor necrosis factor inhibitors (TNFi), have emerged as effective treatment options for patients with CIDs. However, the therapeutic potential of biologics is not always achieved in clinical practice, with results from studies examining the use of biologics in real-world settings suggesting lower levels of treatment effectiveness compared with clinical trial results. Using a targeted approach, this literature review demonstrates that compliance and persistence with biologic therapy is suboptimal and that this has implications for both clinical outcomes and treatment costs. The review identified a variety of predictors of treatment compliance and persistence, including increased age, female gender, presence of comorbidities, increased disease activity, longer disease duration, smoking, increased body mass index, higher biologic treatment dose, higher treatment cost and lower health-related quality-of-life scores. Patients often cited factors associated with medication delivery as a reason for non-compliance and non-persistence, and device-related improvements to treatment delivery were associated with higher rates of compliance and persistence. The articles identified in this review provide insights that have the potential to help guide the development of new solutions to improve disease management and optimize treatment regimens. This has the potential to benefit patients' health by improving clinical outcomes and to reduce the burden to society by limiting the economic impact of patients' disease. FUNDING: UCB Pharma.

Assessment and Management of Anti-Insulin Autoantibodies in Varying Presentations of Insulin Autoimmune Syndrome.

Context: Insulin autoimmune syndrome (IAS), spontaneous hyperinsulinemic hypoglycemia due to insulin-binding autoantibodies, may be difficult to distinguish from tumoral or other forms of hyperinsulinemic hypoglycemia, including surreptitious insulin administration. No standardized treatment regimen exists. Objectives: To evaluate an analytic approach to IAS and responses to different treatments. Design and Setting: Observational study in the UK Severe Insulin Resistance Service. Patients: Six patients with hyperinsulinemic hypoglycemia and detectable circulating anti-insulin antibody (IA). Main Outcome Measures: Glycemia, plasma insulin, and C-peptide concentrations by immunoassay or mass spectrometry (MS). Immunoreactive insulin was determined in the context of polyethylene glycol (PEG) precipitation and gel filtration chromatography (GFC). IA quantification using ELISA and RIA, and IA were further characterized using radioligand binding studies. Results: All patients were diagnosed with IAS (five IgG, one IgA) based on a high insulin/C-peptide ratio, low insulin recovery after PEG precipitation, and GFC evidence of antibody-bound insulin. Neither ELISA nor RIA result proved diagnostic for every case. MS provided a more robust quantification of insulin in the context of IA. One patient was managed conservatively, four were treated with diazoxide without sustained benefit, and four were treated with immunosuppression with highly variable responses. IA affinity did not appear to influence presentation or prognosis. Conclusions: IAS should be considered in patients with hyperinsulinemic hypoglycemia and a high insulin/C-peptide ratio. Low insulin recovery on PEG precipitation supports the presence of insulin-binding antibodies, with GFC providing definitive confirmation. Immunomodulatory therapy should be customized according to individual needs and clinical response.

Immunosuppressants in Brazil: underlying drivers of spending trends, 2010-2015.

BACKGROUND: Immunosuppressants are recommended for treatment of autoimmune diseases, and in transplant therapy. The high cost of these drugs has been causing an important impact on global pharmaceutical spending. OBJECTIVE: Analyzing immunosuppressant expenditure in Brazil, using data from the Federal Procurement System database (SIASG), between 2010 and 2015. METHODS: The pharmaceutical products were classified in accordance with the Anatomical, Therapeutic and Chemical (ATC) classification system recommended by World Health Organization (WHO) and aggregated by volume and by expenditure. The expenditure variation was decomposed into three broad categories: price effects, quantity effects, and drug mix effects. RESULTS: During the period, annual expenditure increased by 49%, ranging from USD 494.5 million in 2010 to USD 738.7 million in 2015, while purchased quantities increased by 294%, ranging from 49.8 million in 2010 to 196.5 million in 2015. Two factors drove expenditures: the quantity effect and the drug-mix effect. CONCLUSION: These findings may contribute to understand immunosuppressant spending trends and the factors that influence them in order to formulate effective cost containment strategies and design optimum drug policy. Rigorous evaluations are recommended to reduce the drug-mix effect, including systems to monitor price, effectiveness, safety, therapeutic value and budget impact of pharmaceutical innovations.

New use for an old treatment: Hydroxychloroquine as a potential treatment for systemic vasculitis.

Antimalarials have been an effective and safe treatment for autoimmune rheumatic diseases such as systemic lupus erythematosus for more than a hundred years. There are surprisingly few reports of hydroxychloroquine use in the systemic vasculitides. Hydroxychloroquine has antithrombotic, cardiovascular, antimicrobial and antineoplastic effects, making it a potentially valuable treatment for patients with systemic vasculitis who are at risk of infections, malignancy and thrombotic events. We report the successful use of hydroxychloroquine in patients with ANCA vasculitis, Henoch Schonlein purpura/IgA vasculitis, Takayasu's arteritis and polyarteritis nodosa. We review the immunomodulatory mechanisms of action of hydroxychloroquine and the existing evidence for its use in the treatment of vasculitis, with a particular focus on ANCA subtypes.