Evaluation of revised classification criteria for giant cell arteritis and its clinical phenotypes.

BACKGROUND: GCA is a systemic vasculitis of the elderly, viewed by many as a disease with multiple and overlapping clinical phenotypes. Retrospective studies have shown differences in clinical presentation between these phenotypes. To reflect the heterogeneity of GCA and novel diagnostic methods, new classification criteria have been proposed. METHODS: This is a retrospective study of newly diagnosed patients with GCA at the outpatient rheumatology clinics at Skåne University Hospital (Malmö and Lund) between 2012 and 2018. All patients were evaluated using two sets of classification criteria, the ACR classification criteria from 1990 and a proposed revision of these criteria requiring objective findings (positive biopsy or imaging) for classification. Patients were further classified as one of four widely used clinical phenotypes. RESULTS: A total of 183 patients with a new diagnosis of GCA were identified. The diagnosis was confirmed by one or two experienced rheumatologists in 116 of these patients during a review of medical records. The ACR criteria were more sensitive than the revised criteria (93.1% vs 72.4%), but the revised criteria had higher specificity (94.0% vs 28.4%). The revised criteria tended to have higher sensitivity in the phenotype with constitutional symptoms compared with cranial GCA (P = 0.08). CONCLUSION: The specificity of the ACR classification criteria for GCA can be improved by using revised criteria requiring objective findings of vasculitis. In addition, the wider symptoms covered by the revised criteria may improve classification of patients with a phenotype characterized by constitutional symptoms.

Respuesta a: Estudio comparativo de la ecografía Doppler frente a la biopsia de arteria temporal en el diagnóstico de la arteritis de células gigantes / Response to: A comparative study of doppler ultrasound against temporal artery biopsy in the diagnosis of giant cell arteritis

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Respuesta: Respuesta a: Estudio comparativo de la ecografía Doppler frente a la biopsia de arteria temporal en el diagnóstico de la arteritis de células gigantes / Reply: Response to: A Comparative study of doppler ultrasound against temporal artery biopsy in the diagnosis of giant cell arteritis

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18F-Fluorodeoxyglucose PET/Computed Tomography in the Diagnosis and Monitoring of Giant Cell Arteritis.

18F-Fluorodeoxyglucose (FDG) PET/computed tomography (CT) is a highly accurate diagnostic tool for large vessel vasculitis (LVV) and is one of the recommended imaging modalities for confirmation of the diagnosis. This article focuses on the role of FDG-PET/CT in LVV diagnosis and disease monitoring, mainly focusing on giant cell arteritis; in particular, the diagnostic accuracy, diagnostic criteria, the potential pitfalls in the interpretation of large vessel FDG uptake, and the clinical indication compared with other imaging modalities are discussed.

Ecografía Doppler de arterias temporales superficiales en la arteritis de células gigantes en una cohorte con alta sospecha clínica / Doppler ultrasonography of superficial temporal artery in a cohort of patients with strong clinical suspicion of giant cell arteritis

Fundamento y objetivo: La arteritis de células gigantes (ACG) es la vasculitis sistémica más frecuente en adultos. En los últimos años, existe un interés creciente en evaluar la utilidad de la ecografía de arterias temporales superficiales (EATS), que permite valorar la inflamación de la pared vascular. Material y métodos: Estudio de cohorte observacional y descriptivo de 120 EATS a 60 pacientes con sospecha clínica de ACG según criterios clásicos de ACR (American College of Rheumatology). Resultados: De todos los pacientes a quienes se les realizó la EATS, el 42,3% presentó diagnóstico de ACG según los criterios de clasificación ACR. La sensibilidad y la especificidad de la ecografía en nuestra cohorte de pacientes con alta sospecha clínica fueron de 81,8 y 93,3%, respectivamente, con un VPP de 90,1% y un VPN de 87,5%. Conclusión: Los resultados avalan la ecografía de arteria temporal bilateral como una técnica útil, indolora, rápida, accesible, de alta especificidad y amplia validez diagnóstica

Background and objective: Giant cell arteritis (GCA) is the most frequent systemic vasculitis in adults. In recent years, the usefulness of temporal artery ultrasound (TAUS) as a diagnostic tool to assess the underlying inflammation of the vascular wall during the inflammatory process has been under clinical investigation. Material and methods: Observational and descriptive cohort study of 120 TAUS in 60 patients with clinical suspicions of GCA, according to the ACR (American College of Rheumatology) classification criteria. Results: Among all patients who underwent ultrasound, 42.3% met clinical criteria for GCA according to ACR. Sensitivity and specificity of TAUS in our cohort with clinical suspicion was 81.8% and 93.3%, respectively. A PPV of 90.1% and a VPN of 87.5% were observed. Conclusion: Our results showed that TAUS as a useful, indolent, fast, and accessible tool with high diagnostic specificity and diagnostic value

Histopathology of vasculitis.

The range of pathologies that are related to primitive vasculitis is broad, complex and not as typical as we would expect. Clinicians should be aware that several forms of primitive and systemic vasculitis, regardless of the size of the affected vessel, may exhibit identical histological alterations. This observation has important clinical implications as it means that cases of vasculitis do not correspond clinically and histologically. Thus, while histology remains the diagnostic gold standard, it can be used only as part of the most complete clinical assessment possible. Another point worth of the clinician's attention is that vasculitis histology changes over time, as do disease evolution and activity, even without considering the masking effects of treatment and the possibility of sampling error due to the patchy occurrence of vasculitis. The purpose of this review is to identify the most common forms of vasculitis in clinical practice, and to provide guidance to the clinician on the pathology of the vessels.

Classification of large vessel vasculitis: Can we separate giant cell arteritis from Takayasu arteritis?

The two main variants of large vessel vasculitis include Takayasu arteritis and giant cell arteritis. While these two conditions have historically been considered different conditions, recent evidence questions whether they are a spectrum of the same disease. Classification criteria are limited in distinguishing between cases with phenotypic overlap. The limitations of the current criteria and directions of future research are reviewed.

Management of giant cell arteritis: Recommendations of the French Study Group for Large Vessel Vasculitis (GEFA).

PURPOSE: Management of giant cell arteritis (GCA, Horton's disease) involves many uncertainties. This work was undertaken to establish French recommendations for GCA management. METHODS: Recommendations were developed by a multidisciplinary panel of 33 physicians, members of the French Study Group for Large Vessel Vasculitis (Groupe d'étude français des artérites des gros vaisseaux [GEFA]). The topics to be addressed, selected from proposals by group members, were assigned to subgroups to summarize the available literature and draft recommendations. Following an iterative consensus-seeking process that yielded consensus recommendations, the degree of agreement among panel members was evaluated with a 5-point Likert scale. A recommendation was approved when ≥ 80% of the voters agreed or strongly agreed. RESULTS: The 15 retained topics resulted in 31 consensus recommendations focusing on GCA nomenclature and classification, the role of temporal artery biopsy and medical imaging in the diagnosis, indications and search modalities for involvement of the aorta and its branches, the glucocorticoid regimen to prescribe, treatment of complicated GCA, indications for use of immunosuppressants or targeted biologic therapies, adjunctive treatment measures, and management of relapse and recurrence. CONCLUSIONS: The recommendations, which will be updated regularly, are intended to guide and harmonize the standards of GCA management.

Inflammatory disease of the aorta: patterns and classification of giant cell aortitis, Takayasu arteritis, and nonsyndromic aortitis.

OBJECTIVE: Inflammatory diseases of the aorta, other than those of known infective etiology, are poorly understood. We analyzed a large series of affected patients who had histologic diagnoses with a view to improving the classification of the extent of aortitis to enable a more targeted approach of treatment. METHODS: Between 1996 and 2012, we operated on 7551 patients with ascending or aortic arch disease, of whom 279 had clinically diagnosed inflammatory disease. Of these, 156 (2%) were found to have aortitis on histologic examination. RESULTS: Patients were divided into 4 histologically based groups: giant cell aortitis, 31% (49/156); Takayasu arteritis, 5.1% (8/156); isolated aortitis, 59% (92/156); and other, 4.5% (7/156). Patterns of anatomic extent were also analyzed, and in particular it was noted that giant cell aortitis and isolated aortitis had more extensive disease. In addition, specimen analysis suggested early indications of unrecognized preexistent infections. Death after surgery occurred in 3.2% (5/156), and stroke in 1.9% (3/156). Kaplan-Meier survival at 8 years was 55%. We present a classification for disease extent and management. CONCLUSIONS: Aortitis continues to be a conundrum; however, good results are achievable with surgery. Intervention should be based on a clearer understanding of the histologic pattern and extent of disease, which helps in subsequent targeted disease management.

Large-vessel giant cell arteritis: a cohort study.

OBJECTIVE: The aim of this study was to compare baseline variables, treatment and outcomes in patients with large-vessel GCA (LV-GCA), primarily of the upper extremities, with those with cranial disease (C-GCA). METHODS: All patients >50 years of age with radiographic evidence of subclavian LV-GCA diagnosed between 1 January 1999 and 31 December 2008 were identified and compared with those with biopsy-positive C-GCA diagnosed in the same period. RESULTS: The study included 120 LV-GCA patients and 212 C-GCA patients. Compared with C-GCA, patients with LV-GCA were younger [68.2 years (s.d. 7.5) vs 75.7 (7.4), P < 0.001] and had longer duration of symptoms at GCA diagnosis (median 3.5 vs 2.2 months, P < 0.001). A history of PMR was more common in LV-GCA patients (26% vs 15%, P = 0.012), but a smaller proportion had cranial symptoms (41% vs 83%, P < 0.001) and vision loss (4% vs 11%, P = 0.035). ACR classification criteria for GCA were satisfied in 39% of LV-GCA patients and 95% of C-GCA patients (P < 0.001). Compared with C-GCA, patients with LV-GCA had more relapses (4.9 vs 3.0/10 person-years, P < 0.001), higher cumulative corticosteroid (CS) doses at 1 year [11.4 g (s.d. 5.9) vs 9.1 (s.d. 3.7), P < 0.001] and required longer treatment (median 4.5 vs 2.2 years, P < 0.001). CONCLUSION: Although patients with LV-GCA had a lower rate of vision loss, they had a higher relapse rate and greater CS requirements. The ACR criteria for GCA are inadequate for the classification of patients with LV-GCA.

Giant cell arteritis restricted to the limb arteries: An overlooked clinical entity.

OBJECTIVE: Giant cell arteritis (GCA) is a systemic vasculitis typically affecting temporal arteries. In at least 15% of cases, GCA also features inflammation of the aorta and its primary branches. Large-vessel inflammation restricted to proximal limb arteries in the absence of temporal and aortic involvement (Limb Restricted, LR) is rare and not well described in literature. Hence, we aim to characterize this neglected clinical entity. METHODS: We describe a series of three cases of LR-GCA. All patients were older than 50 years, had increased erythrocyte sedimentation rate (ESR), normal cholesterol and triglycerides serum levels, negative temporal artery biopsy, suggestive F-18 fluorodeoxyglucose positron emission tomography (FDG-PET) findings, and responded to immunosuppressive therapy. We also reviewed all published cases of LR-GCA (76 cases), for a total of 79 patients. RESULTS: Limb claudication was reported in 87% of the patients, and cranial symptoms and polymyalgia rheumatica in 20%. Constitutional symptoms were never reported. Median ESR levels were 66.5mm/1h. Upper and lower limb arteries were involved in 86% and 9% of the patients respectively, and the remaining 5% had simultaneous upper and lower limb vessel involvement. Conventional angiography was performed in 63% of the cases, color-doppler ultrasound in 20%, FDG-PET in 14%, and computed tomography angiography in 3%. CONCLUSION: If temporal biopsy and aortic imaging are negative for GCA in patients older than 50 years with bilateral limb claudication, elevated ESR, and suggestive vascular radiological findings, LR-GCA should be suspected. Upper limb arteries are more frequently involved. Since constitutional symptoms are typically absent in LR-GCA, differential diagnosis with atherosclerotic plaques may be challenging.

Clinical study of giant cell arteritis in our hospital.

OBJECTIVE: To investigate clinical and laboratory features of giant cell arteritis (GCA). METHOD: We included 24 patients (6 men, 18 women; mean age 69.8 years) in this study. GCA was diagnosed based on the American College of Rheumatology 1990 classification criteria. RESULTS: Mean serum C-reactive protein was 9.03 mg/dl. GCA was classified into three types: classic temporal arteritis type (cranial GCA, nine patients); large-vessel type, affecting the aorta and its major branches without temporal arteries (12 patients); generalized type, affecting both temporal arteries and large vessels (three patients). Swelling and tenderness of temporal arteries were recognized in temporal arteritis and generalized arteritis. Ten of these patients also had histopathologic findings of arteritis, including giant cells in biopsy specimens. Examination of HLA-class 1 expression showed that one patient with cranial GCA, three with generalized GCA, and seven with large-vessel GCA were positive for HLA-A24, and four patients with large-vessel GCA were positive for HLA-B39. One patient with cranial GCA, one with generalized GCA, and six with large-vessel GCA were positive for HLA-B52. Nine patients were positive for anti-phospholipid antibodies (seven for anti-cardiolipin antibody immunoglobulin G, seven for anti-cardiolipin ß2-glycoprotein-1 antibody, one for lupus anticoagulant). CONCLUSION: Our study demonstrated that HLA-class 1 expression in GCA resembles that in Takayasu arteritis, suggesting that these two arteritis types share the same genetic background. In contrast, the difference in the prevalence of anti-phospholipid antibodies in GCA and Takayasu arteritis patients shows a difference in the characteristic aspects of these two arteritis types.

The diagnosis and classification of giant cell arteritis.

Giant-cell arteritis (GCA) involves the major branches of the aorta with predilection for the extracranial branches of the carotid artery. It occurs in individuals older than 50 years and the incidence increases with age. The signs and symptoms of giant cell arteritis can be classified into four subsets: cranial arteritis, extracranial arteritis, systemic symptoms and polymyalgia rheumatica. Patients may develop any combination of these manifestations, associated with laboratory evidence of an acute-phase reaction. The only test that confirms GCA diagnosis is a temporal artery biopsy, showing vasculitis with mononuclear cell inflammatory infiltrates, often with giant cells. Due to the focal and segmental nature of the infiltrates, areas of inflammation may be missed by the biopsy and the histological examination is normal in about 15% of the cases. Some imaging modalities may aid in the diagnosis of GCA. Among those, color duplex ultrasonography of the temporal arteries is more commonly used. There are no independent validating criteria to determine whether giant cell arteritis is present when a temporal artery biopsy is negative. The American College of Rheumatology criteria for the classification of giant cell arteritis may assist in the diagnosis. However, meeting classification criteria is not equivalent to making the diagnosis in individual patients, and the final diagnosis should be based on all clinical, laboratory, imaging and histological findings. Glucocorticoids are the treatment of choice for GCA. The initial dose is 40-60 mg/day for most uncomplicated cases. Addition of low-dose aspirin (100 mg/d) has been shown to significantly decrease the rate of vision loss and stroke during the course of the disease.

[Vasculitis. New nomenclature of the Chapel Hill consensus conference 2012]. / Vaskulitis. Neue Nomenklatur der Chapel-Hill-Konsensuskonferenz 2012.

In recent years, many advances have been made in our understanding of vasculitis etiopathology as well as of different disease courses. The revised Chapel Hill Consensus Conference (CHCC) 2012 nomenclature reflects current knowledge about etiopathology, in addition to the descriptive principles of vessel size and type of inflammation. Anti-neutrophil cyptoplasmic antibody (ANCA)-associated vasculitides have been classified as a separate group, as opposed to immune complex small vessel vasculitis. In cases where consensus was achieved, eponyms have been replaced by systematic names, such as granulomatosis with polyangiitis (Wegener's) or eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome). Moreover, clinically important but less well-known types of vasculitis have now been included in the CHCC nomenclature. This article presents the changes, focussing on those types that are relevant to the histopathologist, and summarizes the results of important new articles on morphology and clinical picture of vasculitis.

Systemic vasculitis: a critical digest of the recent literature.

Herewith we provide a critical digest of the recent literature on systemic vasculitis. In this manuscript, we reviewed all the articles published during the last 12 months on large-, medium- and small-vessel vasculitis and selected the most relevant studies regarding the epidemiology, pathogenesis and management of systemic vasculitis. In particular we focused the attention on giant cell arteritis, ANCA-associated vasculitis and cryoglobulinemia.