T. Ponge

Aortic involvement in recent‐onset giant cell (temporal) arteritis: A case–control prospective study using helical aortic computed tomodensitometric scan

OBJECTIVE The prevalence of the involvement of large vessels in giant cell arteritis (GCA) is 3-13%. Aortitis is the most serious complication of GCA. Computed tomodensitometric (CT) scan allows analysis of both the aortic wall and endoluminal part of the aorta. Therefore, we conducted a study using CT scan to analyze aortic abnormalities in patients with recent-onset GCA. METHODS This prospective controlled study compared patients with biopsy-proven GCA with a matched control group based on sex, age, and cardiovascular risk factors. During the 4-week period following diagnosis of GCA, patients underwent an aortic CT scan. The aortic imaging results were blindly compared between both groups. RESULTS From January 5, 1998 to January 11, 1999, 22 patients and 22 controls were screened by CT scan for aortic involvement. Thickening of the aortic wall was more frequent among patients than controls (45.4% versus 13.6%; P = 0.02). Aortic thickening (mean 3.3 mm) was located on the ascending part of the thoracic aorta in 22.7% of the patients, with no evidence of thickening in the controls (P = 0.05). Thickening of the abdominal aortic wall was noted in 27.3% of the patients and none of the controls (P = 0.02). CONCLUSION This study suggests that inflammatory aortic thickening, detected by CT scan, occurs frequently at the time of diagnosis of GCA, and that this condition predominantly occurs on the ascending part of the aorta.

Giant Cell Arteritis with or without Aortitis at Diagnosis. A Retrospective Study of 22 Patients with Longterm Followup

Objective. Studies have shown that aortitis may be present in half the patients with recent-onset giant cell arteritis (GCA). We assessed whether aortitis at diagnosis affects longterm outcome in patients with GCA. Methods. We retrospectively analyzed the longterm outcome of a prospective cohort of 22 patients with biopsy-proven GCA who all had aortic computed tomography (CT) evaluation at the time of diagnosis of GCA between May 1998 and November 1999. Longterm outcome, especially vascular events such as aortic aneurysm, mortality, relapses of GCA, and requirement for steroids, was assessed in 2011 by chart review and patient/physician interviews. Results. At disease onset, 10/22 patients had aortitis on CT scan. Patients with and without aortitis had similar baseline characteristics, including cardiovascular risk profile. At the time of the study, 12/22 (57%) patients had died. Vascular causes of death were more frequent in patients with aortitis (5/7 vs 0/5; p = 0.02). A higher number of vascular events was noted in patients with aortitis (mean events per patient 1.33 vs 0.25; p = 0.009). Stroke was more frequent in patients with aortitis. These patients seemed to exhibit a more chronic or relapsing disease course, and they were less likely to completely discontinue steroid therapy (p = 0.009, log-rank test). Conclusion. Our study suggests for the first time that inflammatory aortic involvement present at onset of GCA could predict a more chronic/relapsing course of GCA, with higher steroid requirements and an increased risk for vascular events in the long term.

Role for vascular investigations in giant cell arteritis.

Giant cell arteritis is characterized by diffuse arterial inflammation that selectively involves the superficial temporal arteries but can occur in larger arteries. Various vascular investigations can assist in diagnosing and evaluating the extent of giant cell arteritis. Imaging techniques, mainly Doppler ultrasonography of the superficial temporal arteries, seem less reliable for the diagnosis than temporal artery biopsy, which is safe and remains indispensable. Investigations of larger arteries can detect asymptomatic stenotic lesions, which are common, particularly in the axillary and subclavian arteries. Involvement of the aorta can cause life-threatening dissection or aneurysmal rupture. Imaging techniques useful for diagnosing aortic involvement include ultrasonography, computed tomography, magnetic resonance imaging, and aortography. Although there is no standardized strategy for aortic lesion detection, helical computed tomography may be valuable.

Paraneoplastic Cutaneous Leukocytoclastic Vasculitis Disclosing Multiple Myeloma: A Case Report

Cutaneous lesions are unusual during the course of multiple myeloma. In rare cases, multiple myeloma may be associated to skin involvement secondary to amyloidosis, cryoglobulinemia, and POEMS syndrome. Paraneoplastic skin involvement occurs preferentially during solid neoplasms. We report the case of an uncommon presentation of an IgA lambda multiple myeloma in a 58 year-old woman preceded by vascular purpura with cutaneous leukocytoclastic vasculitis (LV) and intense deposit of IgA and kappa light chains in the dermal vessels. Purpura resolved after specific treatment of multiple myeloma and diagnosis of paraneoplastic purpura was asserted. We propose a brief review of the literature about skin involvement during multiple myeloma. This case highlights the necessity to search for MM when we are facing a LV.

Two cases of atypical Whipple's disease associated with cytoplasmic ANCA of undefined specificity

complement component. On a longitudinal analysis of SSc duration, as measured by the interval from the onset of the first nonRaynaud’s symptom, the proportion of patients with hypocomplementaemia remained unchanged. Similarly, there was no significant difference between hypocomplementaemic and normocomplementaemic patients with the changes in mRSS, pulmonary arterial pressures, diffusing capacity of the lung for carbon monoxide (DLCO), and forced vital capacity (FVC) at follow-up (data not shown). The prevalence of hypocomplementaemia in our multicentre analysis was lower than that registered by others (14–17%) (9, 10). Cuomo et al reported an association between hypocomplementaemia and skin, vascular, heart, and lung involvement (10). These discrepancies cannot be explained by a higher proportion of SSc overlap syndromes, as they had been excluded in the study of Cuomo et al, but their analysis might have included patients with autoantibodies other than anti-Scl70 and anti-centromere. Our study is limited by the lack of a centralized assessment of the labile analyte. Measuring degradation products could have been a more sensitive measure of complement activation. On the contrary, complement activation may occur in vitro during blood processing and increase the prevalence of hypocomplementaemia. This artefact, however, cannot account for the relatively low prevalence of hypocomplementaemia encountered in our study. Finally, overlap syndromes patients are not specifically excluded in EUSTAR, but they are more likely to be hypocomplementaemic than those with pure SSc (9). In summary, our results do not support a role of C3 and C4 measurements in the assessment of pure SSc. Acknowledgements

Use of Muscle Biopsies for Diagnosis of Systemic Vasculitides

Objective. Few studies have investigated the use of muscle biopsies (MB) for the diagnosis of systemic vasculitides (SV). We aimed to evaluate the diagnostic use of MB in this condition. Methods. We reviewed 310 consecutive MB performed in our center between 2000 and 2008 and correlated them with clinical data from the corresponding patients. Thirty-one of the patients, representing a total of 33 MB, were diagnosed with active SV. MB were considered positive when they demonstrated either necrotizing vasculitis or nonnecrotizing vasculitis. Results. Twenty-two of the 33 MB were positive (sensitivity of 66.7%), with necrotizing vasculitis and nonnecrotizing vasculitis being equally frequent. The SV were antineutrophil cytoplasmic antibody (ANCA)-associated in 22 patients (71%), and ANCA-negative in 9 cases (29%). Neither the type nor the clinical spectrum of the SV was predictive of MB positivity. None of the muscle symptoms (myalgias or biological rhabdomyolysis) were correlated with MB positivity. All the biopsies were performed uneventfully. Conclusion. The feasibility and positive predictive value of MB make it a valuable tool for ruling out a diagnosis of SV. Since no clinical signs could predict its positivity, MB should be considered in all suspected cases of SV. Unlike other biopsies, including kidney biopsy, MB had no prognostic value.

Place des explorations vasculaires dans la maladie de Horton

Resume La maladie de Horton est une arterite inflammatoire diffuse, touchant preferentiellement les arteres temporales superficielles, mais pouvant se localiser aux arteres de plus gros calibre. Differentes techniques dˈexploration vasculaire peuvent apporter une aide dans le diagnostic ou le bilan dˈextension de cette affection. A visee diagnostique, les techniques dˈimagerie, principalement lˈechographie couplee au Doppler des arteres temporales superficielles, apparaissent moins performantes que la biopsie dˈartere temporale, qui reste un geste indispensable et bien tolere. Lˈexploration vasculaire des arteres de gros calibre permet de depister de frequentes stenoses parfois asymptomatiques, avec un tropisme preferentiel pour les axes axillo-sous claviers. Lˈaortite de Horton est une forme particulierement grave de la maladie, pouvant se compliquer de dissection ou de rupture dˈanevrisme aortique. Les techniques dˈimagerie pouvant conduire au diagnostic dˈaortite sont lˈechographie, la tomodensitometrie, l’imagerie par resonance magnetique (IRM) ou lˈaortographie. Le depistage des lesions aortiques nˈest pas codifie mais le scanner spirale de lˈaorte pourrait avoir un interet dans cette indication.

C'est en cas de dyspnée aiguë qu'il est urgent de cesser de végéter...

C. Agard a,∗, O. Grossi b, S. Pattier c, A. Espitia-Thibault a, B. Le Goff d, M. Audrain e, T. Ponge a, D. Raoult f, M. Hamidou a, C. Pagnoux g a Service de médecine interne, CHU Hôtel-Dieu, place Alexis-Ricordeau, 44093 Nantes cedex 01, France b Service de maladies infectieuses et tropicales, CHU Hôtel-Dieu, place Alexis-Ricordeau, 44093 Nantes cedex 01, France c Service de cardiologie, CHU Hôtel-Dieu, place Alexis-Ricordeau, 44093 Nantes cedex 01, France d Service de rhumatologie, CHU Hôtel-Dieu, place Alexis-Ricordeau, 44093 Nantes cedex 01, France e Laboratoire d’immunologie, CHU Hôtel-Dieu, place Alexis-Ricordeau, 44093 Nantes cedex 01, France f Unité des Rickettsies, CNRS, UMR 6020, IFR 48, université de la Méditerranée, Marseille, France g Pôle de médecine interne, hôpital Cochin, 27, rue du Faubourg-Saint-Jacques, 75014 Paris, France