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A patient presented diffuse pulmonary parenchymal micronodules. Tropheryma whipplei was detected in the saliva, a bronchial biopsy and bronchoalveolar fluid. PAS staining, immunohistochemistry and PCR for T. whipplei were negative in the duodenal biopsies. T. whipplei was isolated from the bronchoalveolar fluid, reinforcing its role as a respiratory pathogen.

Resume Introduction Une myelite transverse complique l’evolution d’un lupus erythemateux systemique (LES) dans moins de 5 % des cas. La myelite longitudinale, caracterisee par une atteinte inflammatoire d’au moins quatre segments medullaires, en est une forme particuliere. Observation Nous rapportons l’observation d’une patiente lupique de 31 ans, hospitalisee pour une paraparesie avec syndrome confusionnel. La ponction lombaire et l’IRM permettaient de retenir le diagnostic de myelite longitudinale. L’evolution etait rapidement favorable sous-corticotherapie. Conclusion Si de nombreuses observations de myelite transverse lupique ont ete rapportees dans la litterature, l’atteinte longitudinale est plus exceptionnelle. Elle est a evoquer devant une paraplegie, voire une tetraplegie, avec deficit sensitif et troubles vesicosphincteriens. En IRM, des hypersignaux T2 medullaires sont retrouves. Des sequelles neurologiques sont possibles. Les formes corticoresistantes sont habituellement traitees par cyclophosphamide.

Resume Introduction La face est une localisation frequente de la sclerose cutanee au cours de la sclerodermie systemique. Les principales manifestations stomatologiques comprennent la limitation de l’ouverture buccale, la xerostomie, l’atrophie cutanee, la nevralgie du V3. L’objectif de cette etude est de decrire les manifestations buccofaciales des patients sclerodermiques issus de notre cohorte. Methodes Entre mars et octobre 2006, une consultation de stomatologie a ete incluse dans le suivi systematique des patients sclerodermiques vus en consultation ou en hopital de jour de medecine interne et de dermatologie. Chaque patient a fait l’objet d’un recueil de donnees demographiques, cliniques et biologiques. Le recueil comportait la mesure de l’ouverture buccale, les tests au sucre et de Schirmer, l’analyse de l’orthopantomogramme, ainsi qu’une evaluation du retentissement des symptomes sur la qualite de vie par echelle visuelle analogique (EVA entre 0 et 10). Resultats Cette etude a inclus 30 patients (87 % de femmes, âge moyen 58,6 ± 13,6 ans). La sclerodermie systemique (cutanee limitee n = 20, diffuse n = 10) evoluait depuis huit ans en moyenne. Les manifestations buccofaciales identifiees etaient les suivantes : atrophie cutanee ( n = 28), rhagades peribuccales ( n = 25), telangiectasies ( n = 21), limitation de l’ouverture buccale ( n = 20), xerostomie ( n = 20), xerophtalmie ( n = 16), elargissement du ligament alveolo-dentaire ( n = 10), erosions osseuses ( n = 2), nevralgie du V3 ( n = 1). La xerostomie etait davantage percue comme genante (EVA moyenne = 3,8) que la limitation de l’ouverture buccale (EVA moyenne = 2,6). La xerostomie, decrite comme le second symptome le plus genant de la sclerodermie, etait significativement associee a la forme cutanee limitee ( p = 0,045) et a la presence d’anticorps anticentromere ( p = 0,002). La limitation de l’ouverture buccale etait correlee a l’atteinte œsophagienne ( p = 0,025). Conclusion Chez des patients sclerodermiques vus en consultation de stomatologie, les manifestations buccofaciales apparaissent frequentes et occasionnent une gene fonctionnelle majeure dominee par la limitation de l’ouverture buccale, souvent associee a une atteinte œsophagienne. La xerostomie est frequente et volontiers observee dans les formes limitees avec anticorps anticentromeres. L’evolution des anomalies radiologiques a type d’elargissement du ligament alveolo-dentaire (33 % des cas) ou d’osteolyse (7 %) est mal connue.

Summary Objective This study used Doppler ultrasonography and computed tomographic angiography (CTA) to assess the prevalence of abdominal aortic lesions that suggested abdominal aortitis at diagnosis of giant-cell or temporal arteritis (GCA). We also evaluated the contribution of these 2 techniques to diagnosis. Methods This single-center study included patients admitted to the internal medicine department of the Nantes (France) University Hospital, between May 1999 and May 2002 at the time of their diagnosis with biopsy-proven GCA. In the 8 weeks after diagnosis, patients underwent a thorough workup, including the collection of clinical and laboratory data. Imaging tests for each patient included Doppler ultrasonography and a CTA scan of the abdominal aorta, looking for aneurysms, ectasia, and thickening of the aortic wall. Results This study included 20 patients (17 women, mean age 73.9 ± 7.2 years, mean CRP = 116 ± 75.9 mg/L). Doppler ultrasonography suggested aortitis in 8 cases (40%): 7 patients (35%) had a hypoechoic halo, 3 (15%) a small aneurysm (diameter Conclusion At the time of GCA diagnosis, both Doppler ultrasonography and CTA can detect morphological abnormalities of the abdominal aorta. Here they suggested asymptomatic abdominal aortitis in half the patients. Doppler ultrasonography appears more effective for detecting aortic aneurysms, while CTA seems helpful for the diagnosis of parietal thickening. The risk factors associated with abdominal aortitis in GCA remain to be identified.

BACKGROUND: The objective of the present study was to compare the silent form of giant cell arteritis (GCA) to the classic cephalic form of the disease. METHODS: We conducted a retrospective study based on a chart review of 50 consecutive, biopsy-proven GCA, recorded at a department of internal medicine. We sought to distinguish a silent form, defined by a prolonged inflammatory syndrome or fever of unknown origin with the absence of cephalic signs, polymyalgia rheumatica, or large artery involvement, from an overt "classic" cranial temporal arteritis. RESULTS: The prevalence of the silent form of GCA was 46% in our study. Abnormal temporal arteries were more frequent in the cephalic group. The silent GCA group had higher C-reactive protein levels (p0.05), a higher platelet count (p0.05), and lower serum albumin (p0.05). There was no significant difference in temporal artery specimens in the two groups. Clinical relapses tended to be more frequent, and patients free of corticosteroids tended to be less frequent, in the cephalic group, though the difference was not statistically significant. CONCLUSIONS: The silent and cephalic forms of GCA could have distinct clinical and biological patterns and different outcomes. The limitation of our study was its retrospective design. Further studies are required to determine if this distinction is useful in treating GCA patients.

Anti-neutrophil cytoplasmic antibodies (ANCA) are typically found in patients with necrotizing vasculitis, especially Wegener’s granulomatosis for cytoplasmic ANCA (cANCA), which recognizes protein...

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.

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.

The Gleich's syndrome is a rare disease that causes recurrent angioedema associated with major eosinophilia with good response to corticosteroids. We describe a 53-year-old man who presented with a Gleich's syndrome with a 6-year follow-up and propose a literature review. This case emphazises the favourable prognosis of this disease. In case of poor tolerance of corticosteroids, mepolizumab could be tested.

Objective To describe the clinical, biologic, and histologic features of temporal artery biopsy (TAB)–localized systemic necrotizing vasculitides (SNV), and to assess their frequency among elderly patients undergoing TAB for suspected giant cell (temporal) arteritis (GCA). Methods The frequency of a TAB localization of SNV was prospectively assessed in a multicenter study of elderly patients undergoing TAB for suspected GCA. All patients with SNV fulfilling the American College of Rheumatology criteria for a specific vasculitic syndrome and with evidence of vasculitis on TAB were included in a retrospective, descriptive study. Results SNV was diagnosed based on the TAB in 1.4% of the patients with suspected GCA and in 4.5% of the positive (inflamed) TAB specimens. We retrospectively selected 27 patients (18 female, 9 male; mean ± SD age 62 ± 15 years, range 22–79 years) with SNV and TAB-localized vasculitis. Only 2 of these patients were known to have SNV before TAB localization. Twenty-two patients (81%) had cephalic symptoms, including jaw claudication in 33%, clinically abnormal temporal arteries in 33%, and neuro-ophthalmologic symptoms in 11%. All patients had systemic symptoms suggestive of SNV and histologically proven NV in the TAB specimens (70%) or elsewhere in other biopsy sites (74%). Abnormal biologic results suggestive of SNV were present in 17 patients (63%). For 4 patients, the TAB-documented involvement led to initial misdiagnoses of GCA, and systemic manifestations that developed under steroid therapy revealed the correct diagnosis. The final diagnoses of the patients were polyarteritis nodosa (PAN) (n = 11), Churg-Strauss syndrome (n = 6), micropolyangiitis (n = 3), Wegener's granulomatosis (n = 3), hepatitis B virus–related PAN (n = 2), hepatitis C virus–related cryoglobulinemic vasculitis (n = 1), and rheumatoid vasculitis (n = 1). Conclusion TAB-localized SNV presents a major diagnostic dilemma because it can mimic GCA. Careful analysis of clinical, biologic, and histologic data should lead to the correct diagnosis and help guide the clinician's choice of appropriate therapy.

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.

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.

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.

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.

Objective To describe characteristics and outcomes of vasculitides associated with malignancies. Methods The requirement for inclusion in this retrospective, 10-year study was development of vasculitis in patients with a progressing malignancy. Malignancies secondary to immunosuppressants used to treat vasculitis were excluded. The main characteristics of vasculitides were analyzed and compared according to the type of malignancy. Results Sixty patients were included (male/female sex ratio 2.53, mean age 62.4 years). Mean followup duration was 45.2 months. Vasculitides were cutaneous leukocytoclastic (45%), polyarteritis nodosa (36.7%), Wegener's granulomatosis (6.7%), microscopic polyangiitis (5%), and Henoch-Schonlein purpura (5%). Malignancies were distributed as follows: hematologic in 63.1%, myelodysplastic syndrome (MDS) in 32.3%, lymphoid in 29.2%, and solid tumor in 36.9%. Vasculitides were diagnosed concurrently with malignancy in 38% of the cases. Manifestations of vasculitides were fever (41.7%), cutaneous involvement (78.3%), arthralgias (46.7%), peripheral neuropathy (31.7%), renal involvement (23.3%; 11.7% glomerulonephritis, 11.7% microaneurysms, 6.7% renal insufficiency), and antineutrophil cytoplasmic antibody (20.4%). Vasculitis treatments were corticosteroids (78.3%) and immunosuppressant(s) (41.7%). Vasculitis was cured in 65% of patients, but 58.3% died, with 1 death secondary to vasculitis. Independent of subtype, patients with vasculitides associated with MDS more frequently had renal manifestations (P = 0.02) and steroid dependence (P = 0.04) and achieved complete remission less often (P = 0.04) than patients with vasculitides associated with other malignancies. Patients with vasculitides associated with a solid tumor more frequently had peripheral neurologic involvement (P = 0.05). Patients with vasculitides associated with lymphoid malignancy had less frequent arthralgias (P = 0.01) and renal involvement (P = 0.02). Conclusion Vasculitides occurring during malignancies present distinctive features according to the vasculitis subtype and nature of the malignancy.

Background Aortitis is a potential life-threatening complication of GCA. Up to 30-50% of cases of GCA may have clinical or radiological signs of aortitis (1). Images of aortic involvement may be non symptomatic and their real outcome remain elusive. Objectives This study was conducted to describe the long-term outcome of patients with or without suspicion of aortitis at the time of diagnosis of GCA. Methods Twenty-two patients with newly diagnosed biopsy-proven GCA were explored in 1999 using aortic computed tomodensitometry (CT). Ten patients (group 1) had aortic inflammatory thickenings ≥3mm (n=7) and/or aneurism (n=3), whereas 12 patients had no aortitis (group 2). A retrospective study of these 2 groups was conducted in 2011. We contacted and questioned the patients, their family and general practitioner, and analysed each medical file. The following items were investigated: demographic data, cardio-vascular risk factors, total and cardio-vascular mortality, cardio-vascular events, GCA relapses, corticosteroids regimen. Satistics were made using R development Core Team (2009) software. Results This study included 17 women and 5 men (mean age at diagnosis = 73.7±7.2 y). Inflammatory parameters and cardio-vascular risk factors were similar in group 1 and 2. The mean follow-up was 94.8 months. Twelve years after diagnosis of GCA, the total mortality was 50% without differences between group 1 (7/10) and group 2 (5/12). However, the 12y cardio-vascular mortality was statistically higher in patients with initial suspicion of aortitis (50%), than in patients without (0%, p=0.029, Log rank test). In group 1, the causes of deaths of cardiovascular origin were: rupture of abdominal aortic aneurism (n=1), thoracic aortic dissection (n=1), stroke (n=1), heart failure (n=1), peripheral arterial disease (n=1). Twelve cardio-vascular events occured in 7/10 patients of group 1 whereas only 5 occured in 4/12 patients of group 2. Stroke were statistically more frequent in group 1 (40% vs 0% in group 2, p=0.03). Recurrent GCA relapses were noted in 5/10 patients of group 1, 0/12 patients of group 2 and this difference was statistically significant (p=0.01). Moreover, definitive steroid treatment discontinuation was more frequent in group 2 (n=2) than in group 1 (n=8, p Conclusions Despite the limitations due to its retrospective character and its small number of patients, our study suggests that GCA clinical course may differ according to initial CT signs of aortic involvement. CT suspicion of aortitis may lead to aortic fatal events and aortic thickenings deserve to be monitored. Initial aortic CT involvements seem to sign a particular form of GCA, with higher rate of cardio-vascular events and mortality, and with frequent relapses requiring longer steroids treatment. References Agard C, Barrier JH, Dupas B, et al. Arthritis Rheum 2008;59(5):670-6 Disclosure of Interest None Declared

Abstract 3908 Poster Board III-844 Background In more than 95% of cases, Polycythemia vera (PV) is characterized by the presence of the V617F mutation of JAK2 (JAK2-V617F). We report on three cases of V617F-positive PV with an additional mutation of JAK2 changing leucine 611 for a valine (L611V). Design and Methods We used allele-specific quantitative PCRs and pyro-sequencing to detect the L611V and V617F JAK2 mutants in genomic DNA and in cloned PCR products. The consequences of the different mutations on the function of JAK2 were investigated using transient expression in BaF-3/EpoR cells. Results We found equal proportions of L611V and V617F alleles in granulocyte DNA (patients Na249, 19%; Na382, 26%; Di362, 27%) and established the presence of alleles with L611V and V617F in cis (L611V/V617F). The double JAK2-L611V/V617F mutant represented 15% to 26% of JAK2 alleles in granulocyte DNA. JAK2-L611V/V617F was detected in 12% to 30% of erythroid colonies, always in a heterozygous fashion. Low levels of single mutant alleles were also found, in 0.5% of cloned PCR products (JAK2-L611V, patient Na249) and in 1.5% of genomic DNA and cloned PCR products (JAK2-V617F, patient Na249), indicating that mutations in JAK2 occurred more than once. Functionally, JAK2-L611V was found comparable to wild-type JAK2, whereas JAK2-L611V/V617F displayed greater constitutive tyrosine phosphorylation of JAK2 and AKT than wild-type JAK2 and JAK2-V617F. The double JAK2-L611V/V617F mutant was associated with a high hematocrit and normal counts of leukocytes and platelets. Patients Na382 and Di362, but not patient Na249, carried the 46/1 haplotype of chromosome 9p associated with a reported pre-disposition to mutations of JAK2 on the same allele. Conclusion PV patients can harbor multiple clones carrying different mutations of JAK2, which may or may not increase JAK2 activity. Functionally silent mutations, not leading to clonal expansion, may remain undetected. The double JAK2-L611V/V617F mutated clone was predominant whereas single L611V and V617F JAK2 mutants represented Disclosures: No relevant conflicts of interest to declare.

Although associations of Sjögren's syndrome (SS) with malignant affections are well known, an association with chronic myelomonocytic leukemia (CMML) is infrequent. Our report concerns an 80-year-old woman with chronic polyarthrosis, hospitalized for an anemic syndrome, who also complained of buccal dryness. Clinical examination showed simply parotid swelling and discrete splenomegaly. The diagnosis of SS appeared to be primary. The hemogram, myelogram and bone biopsy indicated CMML. During SS, the possibility of occurrence of a lymphoproliferative syndrome is well documented, and other malignant affections are much less likely to be found in the absence of immunosuppressive treatment. The particular case of SS and associated CMML detected at the same time suggests either the favorable role of monocytic proliferation on immunity or a stem clonal anomaly affecting monocytes and B lymphocytes.