Ocular manifestations of ANCA-associated vasculitis.

OBJECTIVES: ANCA-associated vasculitis (AAV) is a group of multisystem diseases that can have several ocular manifestations. There are published data on ocular manifestations of granulomatosis with polyangiitis (GPA), but few for eosinophilic granulomatosis with polyangiitis (EGPA) or microscopic polyangiitis (MPA). There is little information concerning chronicity, complications, and association with other cranial manifestations of AAV. METHODS: This study retrospectively analysed longitudinal multicentre cohorts of individuals with AAV followed between 2006 and 2022. Data included diagnosis, demographics, cranial manifestations of disease, presence of manifestations at onset of disease and/or follow-up, and ocular complications of disease. Univariate and multivariable logistic regression analysis assessed associations across disease manifestations. RESULTS: Data from 1441 patients were analysed, including 395 with EGPA, 876 with GPA, and 170 with MPA. Ocular manifestations were seen within 23.1% of patients: 39 (9.9%) with EGPA, 287 (32.7%) with GPA, and 12 (7.1%) with MPA at any time in the disease course. There were more ocular manifestations at onset (n = 224) than during follow-up (n = 120). The most common disease-related manifestations were conjunctivitis/episcleritis and scleritis. In multivariable analysis, dacryocystitis, lacrimal duct obstruction, and retro-orbital disease were associated with sinonasal manifestations of GPA; ocular manifestations were associated with hearing loss in MPA. The most common ocular complications and/or damage seen were cataracts (n = 168) and visual impairment (n = 195). CONCLUSION: Ocular manifestations occur in all forms of AAV, especially in GPA. Clinicians should be mindful of the wide spectrum of ocular disease in AAV, caused by active vasculitis, disease-associated damage, and toxicities of therapy.

Added diagnostic yield of temporal artery magnetic resonance angiography in the evaluation of giant cell arteritis.

OBJECTIVES: Temporal artery magnetic resonance angiography (TAMRA) is a useful tool to investigate possible diagnoses of GCA. As acquired images also reveal other local structures, they may assist in finding alternative diagnoses when assessing for possible GCA. We sought to assess the utility of TAMRA in identifying other significant abnormalities either associated with a diagnosis of GCA or potentially mimicking a clinical presentation of GCA. METHODS: A retrospective cohort study was undertaken at St Joseph's Healthcare in Hamilton, Ontario, Canada between February 2007 and April 2020 and included patients who underwent TAMRA for a possible diagnosis of GCA. Patient demographics, diagnosis and imaging findings were extracted, and descriptive analysis of findings was performed. RESULTS: We included 340 individuals who underwent TAMRA for assessment of a potential diagnosis of GCA and had clinical information available; there were 126 (37.1%) diagnoses of GCA. Fourteen (4.1%) patients had findings on TAMRA that demonstrated an alternative diagnosis, findings were predominantly in the temporomandibular joint, orbit and meninges. Eighteen (14.3%) patients with GCA had intracranial vascular changes that were demonstrative of intracranial vasculitis; one stroke was attributed to intracranial GCA. CONCLUSIONS: TAMRA has proven utility in diagnosing GCA, and these data suggest that it also has utility in identifying alternative diagnoses to rule out the disease. Intracranial vasculitis was also seen in 14.3% of patients; the clinical impact of these findings is currently poorly understood and requires further study.

Value of second opinion subspecialty radiology consultation in suspected abdominal medium vessel vasculitis.

PURPOSE: To perform a descriptive analysis of individuals with suspected Polyarteritis nodosa (PAN) referred for second opinion imaging consultation. METHODS: A retrospective observational cohort study was performed at a single institution. A consecutive sample was performed of individuals who underwent a second opinion CT or MR angiography subspecialty radiologist consultation between January 2008 and September 2019 for suspected abdominal medium vessel vasculitis. Demographic, clinical, and imaging data were collected. Clinical and imaging findings were reported for PAN, small vessel vasculitis, and "non-vasculitis" groups. Agreement and diagnostic accuracy between final clinical and second opinion imaging diagnoses for PAN were determined. Two-tailed t-tests with a significant p-value < 0.05 were utilized. RESULTS: Of the 58 participants, 9 were clinically diagnosed with PAN, 11 with small vessel vasculitis (including lupus, IgA, and ANCA-associated vasculitis), and 38 with non-vasculitis diagnoses. The non-vasculitis group included 15 SAM, 3 FMD, and 1 SAM-FMD spectrum diagnoses. Higher C-reactive protein level (51 vs 17, p = 0.04) and superior mesenteric artery involvement (56% vs 21%, p = 0.04) were more common in PAN than non-vasculitis diagnoses, while arterial dissection (40% vs 0%, p = 0.02) and celiac vasculature involvement (53% vs 0%, p = 0.003) were more common in the non-vasculitis group. There was 88% agreement (51/58; Cohen's kappa 0.56); sensitivity was 67% [95%-confidence interval (CI) 30-93%] and specificity was 92% (95%-CI 80-98%). CONCLUSION: Isolated celiac artery involvement and arterial dissection were more common in non-inflammatory vasculopathies than PAN. Our findings highlight the need for multidisciplinary collaboration and awareness of the diverse findings of abdominal vasculopathies.

Contextualizing temporal arterial magnetic resonance angiography in the diagnosis of giant cell arteritis: a retrospective cohort study.

OBJECTIVES: Imaging modalities have become common in evaluating patients for a possible diagnosis of GCA. This study seeks to contextualize how temporal arterial magnetic resonance angiography (TA-MRA) can be used in facilitating the diagnosis of GCA. METHODS: A retrospective cohort study was performed on patients who had been previously referred to a rheumatologist for evaluation of possible GCA in Hamilton, Ontario, Canada. Data including clinical features, inflammatory markers, imaging, and biopsy results were extracted. Multivariable logistic regression model to predict the diagnosis of GCA. Using these models, the utility of TA-MRA in series with or in parallel to clinical evaluation was demonstrated across the cohort as well as in subgroups defined by biopsy and imaging status. RESULTS: In total 268 patients had complete data. Those diagnosed with biopsy- and/or imaging-positive GCA were more likely to demonstrate classic features including jaw claudication and vision loss. Clinical multivariable modelling allowed for fair discriminability [receiver operating characteristic (ROC) 0.759, 95% CI: 0.703, 0.815] for diagnosing GCA; there was excellent discriminability in facilitating the diagnosis of biopsy-positive GCA (ROC 0.949, 0.898-1.000). When used in those with a pre-test probability of 50% or higher, TA-MRA had a positive predictive value of 93.0%; in those with a pre-test probability of 25% or less TA-MRA had a negative predictive value of 89.5%. CONCLUSION: In those with high disease probability, TA-MRA can effectively rule in disease (and replace temporal artery biopsy). In those with low to medium probability, TA-MRA can help rule out the disease, but this continues to be a challenging diagnostic population.

CanVasc Consensus Recommendations for the Management of Antineutrophil Cytoplasm Antibody-associated Vasculitis: 2020 Update.

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 aims to revise existing recommendations and create additional recommendations, as needed, based on a review of new available evidence. METHODS: A needs assessment survey of CanVasc members informed questions for an updated systematic literature review (publications spanning May 2014 to September 2019) using Medline, Embase, and Cochrane. New and revised recommendations were developed and categorized according to the level of evidence and strength of each recommendation. The CanVasc working group used a 2-step modified Delphi procedure to reach > 80% consensus on the inclusion, wording, and grading of each new and revised recommendation. RESULTS: Eleven new and 16 revised recommendations were created and 12 original (2015) recommendations were retained. New and revised recommendations are discussed in detail within this document. Five original recommendations were removed, of which 4 were incorporated into the explanatory text. The supplementary material for practical use was revised to reflect the updated recommendations. CONCLUSION: The 2020 updated recommendations provide rheumatologists, nephrologists, and other specialists caring for patients with AAV in Canada with new management guidance, based on current evidence and consensus from Canadian experts.

Updates in the treatment of granulomatosis with polyangiitis and microscopic polyangiitis: At a crossroad.

There have been great advances in the management of ANCA associated vasculitis over the past decades. We have gone from an era where the disease was almost universally fatal to trying to prevent long-term side effects of treatment regimens. With the ability to use pulse cyclophosphamide or rituximab as alternates to oral cyclophosphamide for induction of remission, side effects of therapy have been greatly reduced. New approaches have drastically changed our approach to maintenance and we now favor much longer durations of maintenance therapy, as they are more successful in preventing relapse. Steroids have long been the bane of treatment as they are associated with a significant risk of infection and metabolic consequences. We are now in a steroid-sparing and looking ahead to a steroid-free era with new data being published showing lower doses of steroids being equally effective and several ongoing seminal trials looking at agents that could completely replace steroids very early on.

Smoldering rheumatoid arthritis: is the Canadian healthcare system neglecting a significant disease population?

OBJECTIVE: To investigate rheumatology practice in Canada with regard to evaluating disease activity status and treatment regimens in patients with rheumatoid arthritis (RA). It was hypothesized that patients with "smoldering" disease activity were not being adequately treated. METHODS: Rheumatologists were invited to participate by the Canadian Rheumatology Association in an audit entitled the Assessment in Rheumatology (AIR) program. From across Canada, 65 rheumatologists participated. One thousand five hundred ninety-six consecutive patients with RA seen in regular clinics were classified according to 4 states of disease activity: remission, controlled adequately, smoldering, and uncontrolled. Demographics (age, sex, geographic region), therapy (nonsteroidal antiinflammatory drugs, disease modifying antirheumatic drugs, biologicals, steroids), joint counts (tender/swollen), comorbidity, and treatment decisions at the time of the visit were recorded. Data were collected at the time of the visit with personal digital assistants (PDA) and aggregated, without personal identifiers, for analysis in SPSS. RESULTS: The majority of patients had "smoldering" (29%) or "uncontrolled" disease (23%), with the remainder in "remission" (15%) or "controlled adequately" (33%) at the time of their visit. Following the appointment, the uncontrolled group had a 100% increase (from 10.4% to 23.4%) in the addition of biological agents; however, there was no significant increase in the rates for those with smoldering disease (19.4% to 20.5%). CONCLUSION: Despite Canada's universal healthcare system, current treatment regimens may not be optimized on the basis of disease activity. A large proportion of patients with RA (29%) seen in Canadian rheumatology practices may be experiencing unnecessary disease for a variety of reasons.