Jean-Paul Makhzoum

• MD, MSc, FRCPC, FACP
• Associate Professor at Université de Montréal

Objectives This study describes the spectrum and initial impact of pulmonary manifestations in the primary systemic vasculitides. Methods Description and comparison of pulmonary manifestations in adults with Takayasu’s arteritis (TAK), giant cell arteritis (GCA), granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic...

Objective The objective of this study was to compare the efficacy and safety of two rituximab (RTX) regimens for the induction of remission in severe antineutrophil cytoplasm antibody–associated vasculitis (AAV): the four-dose (375 mg/m² intravenously weekly) versus the two-dose (1000 mg intravenously biweekly) regimen. Methods A systematic review...

Antineutrophil cytoplasm antibody (ANCA)-associated vasculitides (AAV) are small-vessel vasculitides that include granulomatosis with polyangiitis (formerly Wegener's granulomatosis), microscopic poly-angiitis, and eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome). Renal-limited AAV can be considered a fourth entity. Despite th...

Objectives To compare accuracy of colour doppler ultrasonography (CDUS) and temporal artery biopsy (TAB) to establish the final diagnosis of GCA and to determine how the giant cell arteritis probability score (GCAPS) performs as a risk stratification tool. Methods Descriptive statistics were performed on a retrospective cohort of patients referred...

Introduction/Objectives Optic nerve sheath (ONS) enhancement using magnetic resonance imaging of the orbits was observed in patients with giant cell arteritis (GCA). We previously showed that ONS diameter (ONSD) by bedside ultrasound is increased in patient with active GCA. This study aims to assess whether ONSD decreases with clinical remission in...

Objective Optic nerve sheath enhancement on magnetic resonance imaging has been reported in patients with giant cell arteritis (GCA), with or without visual manifestations. Whether similar findings can be documented on ultrasound is unknown. Optic nerve ultrasound is a point‐of‐care, easy to learn, rapid, and noninvasive technique. This study aims...

Giant cell arteritis (GCA) is the most common primary vasculitis, and permanent vision loss is its most feared complication. GCA is both a medical emergency and a diagnostic challenge. Unfortunately, timely access to temporal artery biopsy remains challenging in many areas, and false-negative results are common. This review article discusses new cu...

Background Giant cell arteritis (GCA) is the most common systemic vasculitis in adults. Presenting features include new-onset headaches, constitutional symptoms, jaw claudication, polymyalgia rheumatica, and visual symptoms. Arterial inflammation with subsequent stenosis and occlusion may cause tissue ischemia, leading to blindness, strokes, and my...

Objective Positive antineutrophil cytoplasmic antibodies (ANCAs) may occur in the setting of interstitial lung disease (ILD), with or without ANCA‐associated vasculitis (AAV). We aim to compare the characteristics and clinical course of patients with ILD and positive ANCA (ANCA‐ILD) to those with negative ANCA. Methods We performed a single‐center...

Introduction/objectives To assess and compare the performance of the giant cell arteritis probability score (GCAPS), Ing score, Bhavsar-Khalidi score (BK score), color Doppler ultrasound (CDUS) halo count, and halo score, to predict a final diagnosis of giant cell arteritis (GCA). Method A prospective cohort study was conducted from April to Decemb...

Background: Giant cell arteritis (GCA) is the most common systemic vasculitis in adults. Presenting features include new-onset headaches, constitutional symptoms, jaw claudication, polymyalgia rheumatica, and visual symptoms. Arterial inflammation with subsequent stenosis and occlusion may cause tissue ischemia, leading to blindness, strokes, and m...

Background In 2020, the Canadian Vasculitis Research Network (CanVasc) published their updated recommendations for the management of antineutrophil cytoplasm antibody (ANCA)-associated vasculitides (AAV). Since then, clinical data on the complement C5a receptor inhibitor avacopan (formerly, CCX168) has continued to expand. Objectives The current a...

Objective: In 2020, the Canadian Vasculitis Research Network (CanVasc) published their updated recommendations for the management of antineutrophil cytoplasm antibody (ANCA)-associated vasculitides (AAV). The current addendum provides further recommendations regarding the use of avacopan in AAV based on a review of newly available evidence. Methods...

Objective In 2020, the Canadian Vasculitis Research Network (CanVasc) published their updated recommendations for the management of antineutrophil cytoplasm antibody (ANCA)-associated vasculitides (AAV). The current addendum provides further recommendations regarding the use of avacopan in AAV based on a review of newly available evidence. Methods...

Background Primary angiitis of the central nervous system (PACNS) is a rare disease, for which no validated guidelines exist. We report the findings of a survey on the clinical practice of physicians who manage adults with PACNS. Methods An online survey was distributed through neurology, internal medicine, and rheumatology societies in Canada and...

The objective of this study is to report the clinical, serological and pathological features of patients with autoimmune myositis other than dermatomyositis, who displayed both muscle weakness on physical examination and prominent B cell aggregates on muscle pathology, defined as ≥ 30 CD20+ cells/aggregate. Specifically, the presence of a brachio-c...

Introduction À ce jour, la prise en charge diagnostique et thérapeutique des vascularites primitives du système nerveux central (VPSNC) ne repose sur aucune recommandation consensuelle. L’objectif de ce sondage était d’évaluer les habitudes pratiques des médecins prenant en charge des adultes avec une VPSNC, en évaluant les éléments concordants et...

Background/Purpose: Giant cell arteritis (GCA) is the most common primary vasculitis but remains challenging to diagnose. In the past years, many probability tools have been developed to predict the presence of GCA. Clinical prediction tools include the GCA probability score (GCAPS), the GCA prediction model (Ing Score) and the Bhavsar-Khalidi (BK)...

Background/Purpose: Various pulmonary manifestations may occur in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). ANCA-related interstitial lung disease (ILD) has recently been described in patients with or without overt AAV. The significance and evolution of this association remains unclear and has been raisin...

Case series Patients: Female, 75-year-old • Female, 76-year-old • Female, 72-year-old Final Diagnosis: Giant cell arteritis Symptoms: Ischemic complications of giant cell arteritis Medication: — Clinical Procedure: — Specialty: General and Internal Medicine • Rheumatology Objective Challenging differential diagnosis Background Some ischemic compl...

Background Shrinking lung syndrome (SLS) is a rare manifestation of systemic lupus erythematosus (SLE) characterized by decreased lung volumes and diaphragmatic weakness in a dyspneic patient. Chest wall dysfunction secondary to pleuritis is the most commonly proposed cause. In this case report, we highlight a new potential mechanism of SLS in SLE,...

VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome was recently discovered in 25 men with late-onset severe and refractory inflammatory syndromes and associated hematologic abnormalities. Various diseases have been described, such as relapsing polychondritis, myelodysplastic syndrome, polyarteritis nodosa, and giant cell arte...

Primary membranous nephropathy (MN) and mucous membrane pemphigoid (MMP) are two autoimmune conditions with well-defined diagnostic and treatment guidelines. MN has been linked to bullous pemphigoid (BP) in certain case reports, though little is known regarding the association of MN and other bullous diseases. The association of MN and MMP has rare...

Prompt treatment of autoimmune encephalitis can prevent long-term neurological sequelae. However, the data and evidence for the treatment of autoantibody negative encephalitis are limited, particularly so for rhombencephalitis. We report the case of a seronegative autoimmune rhombencephalitis successfully treated with rituximab. A 21-year-old woman...

Significant progress has been made in the treatment of ANCA-associated vasculitides (AAV), notably in granulomatosis with polyangiitis and microscopic polyangiitis. Over the past few years, many innovative studies have changed the way we now induce and maintain remission in AAV; achieving remission while limiting treatment toxicity is the key. This...

Background/purposePermanent vision loss (PVL) is a feared complication and a leading cause of morbidity in giant cell arteritis (GCA). The objective of this study is to describe visual manifestations and identify risk factors of ocular involvement in GCA.MethodsA retrospective database from a single vasculitis referral center was used. Descriptive...

Objective: In 2015, the Canadian Vasculitis Research Network (CanVasc) created recommendations for the management of antineutrophil cytoplasm antibody (ANCA)-associated vasculitides (AAV) in Canada. The current update aimed to revise existing recommendations and create additional recommendations, as needed, based on a review of new available evide...

Objective: To describe systemic sclerosis (SSc) with myopathy in patients without classic SSc-specific and SSc-overlap autoantibodies (aAbs), referred to as seronegative scleromyositis. Methods: Twenty patients with seronegative scleromyositis diagnosed by expert opinion were analysed retrospectively for SSc features at myositis diagnosis and fo...

Primary systemic vasculitides are rare diseases that may manifest similarly to more commonly encountered conditions. Depending on the size of the vessel affected (large vessel, medium vessel, or small vessel), different vasculitis mimics must be considered. Establishing the right diagnosis of a vasculitis mimic will prevent unnecessary immunosuppre...

Objective: To describe successful therapeutic strategies in statin-induced anti-HMGCR myopathy. Methods: Retrospective data from a cohort of 55 patients with statin-induced anti-HMGCR myopathy, sequentially stratified by the presence of proximal weakness, early remission, and corticosteroid and IVIG use at treatment induction, were analyzed for...

High-fidelity simulation is an efficient and holistic teaching method. However, assessing simulation performances remains a challenge. We aimed to develop a CanMEDS competency-based global rating scale for internal medicine trainees during simulated acute care scenarios. Methods Our scale was developed using a formal Delphi process. Validity was te...

Résumé D’importants progrès ont été accomplis durant ces dernières décennies dans la prise en charge thérapeutique de la granulomatose avec polyangéite (GPA) et de la polyangéite microscopique (PAM). La survie médiane à 5 ans des patients avec GPA ou PAM avoisine aujourd’hui 80–90 %. De nombreux progrès restent toutefois encore à faire, afin de réd...

Bhaskar Dasgupta, Christian Dejaco, eds. Oxford: Oxford University Press, 2016, 192 pages, $42.50 US This first edition of Polymyalgia Rheumatica and Giant Cell Arteritis is a welcome addition to the pocket book family. The authors’ goals were to provide quick and practical updated information on the epidemiology, diagnosis, and management of poly...