[A late onset diagnosis of giant cell arteritis in remission]. / Diagnostic tardif d'une artérite à cellules géantes en rémission.

Giant-cell arteritis (GCA) is a classical cause of chronical inflammation (CI) in the elderly, causing headaches, scalp hypersensitivity and jaw claudication. We describe a patient with a GCA revealed with a year-long biological inflammation and weight loss. Diagnosis was performed on a systematic temporal artery biopsy showing typical histological features. No treatment was intended as the patient had a spontaneous remission, maintained at one year of follow-up. This case highlights the benefit of a systematic temporal artery biopsy to explore CI and reminds us that GCA may undergo spontaneous remission.

Giant cell arteritis-related aortitis with positive or negative temporal artery biopsy: a French multicentre study.

Objective: To compare the clinical presentation and outcome of giant cell arteritis (GCA)-related aortitis according to the results of temporal artery biopsy (TAB).Method: Patients with GCA-related aortitis diagnosed between 2000 and 2017, who underwent TAB, were retrospectively included from a French multicentre database. They all met at least three American College of Rheumatology criteria for the diagnosis of GCA. Aortitis was defined by aortic wall thickening > 2 mm on computed tomography scan and/or an aortic aneurysm, associated with an inflammatory syndrome. Patients were divided into two groups [positive and negative TAB (TAB+, TAB-)], which were compared regarding aortic imaging characteristics and aortic events, at aortitis diagnosis and during follow-up.Results: We included 56 patients with TAB+ (70%) and 24 with TAB- (30%). At aortitis diagnosis, patients with TAB- were significantly younger than those with TAB+ (67.7 ± 9 vs 72.3 ± 7 years, p = 0.022). Initial clinical signs of GCA, inflammatory parameters, and glucocorticoid therapy were similar in both groups. Coronary artery disease and/or lower limb peripheral arterial disease was more frequent in TAB- patients (25% vs 5.3%, p = 0.018). Aortic wall thickness and type of aortic involvement were not significantly different between groups. Diffuse arterial involvement from the aortic arch was more frequent in TAB- patients (29.1 vs 8.9%, p = 0.03). There were no differences between the groups regarding overall, aneurism-free, relapse-free, and aortic event-free survival.Conclusion: Among patients with GCA-related aortitis, those with TAB- are characterized by younger age and increased frequency of diffuse arterial involvement from the aortic arch compared to those with TAB+, without significant differences in terms of prognosis.

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.

[Retroperitoneal fibrosis in adults: Main characteristics and relevance of the diagnostic procedures based on a retrospective multicenter study on 77 cases]. / Fibrose rétropéritonéale de l'adulte : analyse descriptive et évaluation de la pertinence des examens complémentaires réalisés à visée diagnostique à partir d'une série rétrospective multicentrique de 77 cas.

INTRODUCTION: Retroperitoneal fibrosis (RPF) is a rare disorder characterized by the sheathing of retroperitoneal structures by fibro-inflammatory process. It can be either isolated or associated with an underlying disease or condition. In the absence of consistent and consensual approach, the objective of this study was to assess the relevance of diagnostic tests performed during the diagnostic work-up of RPF. METHODS: Seventy-seven patients were included in this retrospective multicenter study. The diagnosis of RPF was defined by the presence of a thickened circumferential homogeneous tissue unsheathing the infrarenal aorta, excluding peri-aneurysmal fibrosis and a clear evidence of a cancer. RESULTS: In 62 cases (80.5%), the RPF was considered as being primary or "idiopathic". Surgical (n=31) or CT-guided (n=9) biopsies of the RPF were performed in half of the patients showing some fibrotic or non-specific inflammatory lesions in 98% of cases. A bone marrow biopsy was performed in 23 patients leading to diagnosis of low grade B cell non-Hodgkin lymphoma in a single patient who also had a monoclonal gammopathy IgM. The systematic search for autoantibodies or serum tumor markers was of no diagnostic value. CONCLUSIONS: Although the diagnostic procedure was heterogeneous, no cause or associated disease was found in the majority of cases of FRP in this series. In the absence of any clinical or paraclinical evidence suggesting an underlying disease or any atypical features at presentation, a number of non-invasive tests (autoantibodies, tumor markers, bone scintigraphy) and also more invasive diagnostic tests (bone marrow and RPF biopsies) seem of little relevance.

[Hashimoto's encephalopathy]. / Encéphalopathie d'Hashimoto.

Hashimoto's encephalopathy was first described by Lord Brain in 1966. Since, other designations have been proposed and the existence of the disease itself has been debated. However, the number of reported cases in the literature is still increasing and physicians are sometimes confronted with patients with neuropsychiatric manifestations and positive thyroid antibodies. This article is an update based upon a search through Medline database that identified 316 references published between 1961 and 2011. Hashimoto's encephalopathy is a rare condition for which there is a need for both diagnostic criteria and therapeutic consensus.

Association of Fcγ receptor IIIA variant with a subset of anti-topoisomerase I-positive patients in systemic sclerosis: a descriptive pilot study.

Hypothesizing a pathophysiological role of anti-topoisomerase I antibodies (anti-topo I) through autoantibody-dependent cell-mediated cytotoxicity (ADCC) and cytotoxic effectors expressing receptors for the Fc portion of IgG in systemic sclerosis (SSc), 267 SSc patients (56 with anti-topo I and 102 with anti-centromere antibodies (ACA)) were genotyped for the functional FCGR3A-V158F polymorphism. A descriptive analysis of patients according to their clinical and immunological status and FCGR3A-158 V/F genotypes was performed using multiple correspondence analysis. This descriptive analysis revealed an association between the FCGR3A-158 VV genotype and the presence of anti-topo I. By contrast, no relationship was found between FCGR3A polymorphism and the presence of ACA. SSc patients with anti-topo I appear to be more frequently homozygous for the high-affinity FcγRIIIA-coding allele, suggesting that some autoantibodies may be pathogenic through ADCC.

[Diabetic amyotrophy: favorable outcome following corticosteroid therapy?]. / Amyotrophie diabétique : intérêt de la corticothérapie ?

Diabetic amyotrophy or lombosacral radiculoplexus neuropathy is a rare complication associated with early-stage diabetes. Thigh pain, quadricipital amyotrophy, proximal weakness of lower limbs and weight loss are the main symptoms of the disease. As neurological damage is related to inflammatory microvasculitis, corticosteroid therapy may be considered as the first line therapy. We report a 54-year-old patient with type 2 diabetes affected with severe diabetic amyotrophy. Following intravenous corticosteroid therapy, the patient reported a rapid pain relief and gained muscle strength.

[Cutaneous polyarteritis nodosa and Crohn's disease: An association not to be ignored]. / Périartérite noueuse cutanée et maladie de Crohn : une association à ne pas méconnaître.

INTRODUCTION: Cutaneous polyarteritis nodosa (CPAN) is an entity which needs to be acknowledged, since it can have a spontaneously adverse outcome. We report two cases of CPAN associated with Crohn's disease. CASE REPORTS: The first patient was suffering from Crohn's disease for 9 years when she was referred for a necrotic toe. A diagnosis of necrotizing angeitis was confirmed by histological examination of a skin biopsy. Despite systemic corticosteroids, the lesions became more severe, requiring immunosuppressive treatment. The second patient was a female patient referred with forefoot ischemia. Cutaneous histology confirmed the diagnosis of necrotizing angeitis that responded favourably to corticosteroid treatment. The patient had been diagnosed with Crohn's disease 2 months previously. CONCLUSION: CPAN differed from systemic PAN by the absence of visceral involvement. Its association with Crohn's disease, although uncommon, must be recognized as it affects treatment and monitoring.

Comparative analysis of different scintigraphic approaches to assess pulmonary ventilation.

A study was carried out to investigate the predictive value of 81-metastable-krypton (81mKr) distribution, high-size 99-metastable-technetium (99mTc) aerosol deposition and low-size 99mTc aerosol (Technegas) deposition on the pulmonary ventilation evaluated by 133-xenon (133Xe) lung scintigraphy, and to assess the correlation between the 81mKr distribution, the 99mTc aerosols deposition, and the respiratory parameters of patients with chronic obstructive pulmonary disease (COPD). Twenty COPD patients were included. The 81mKr, 133Xe, and 99mTc aerosol lung scintigraphies were successively carried out. The 81mKr distribution and 99mTc deposition were compared to the 133Xe distribution at equilibrium and to the 133Xe clearance. Sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) of 81mKr and Technegas lung scintigraphies to detect alterations in ventilation revealed by 133Xe were defined. The 81mKr distribution and 99mTc deposition according to respiratory parameters were described using a principal component analysis. Compared to 133Xe distribution, a significantly higher distribution of 81mKr in the upper parts of the lungs in the more severe patients (p = 0.05), a significantly higher deposition of Technegas in the lower parts of the lungs (p = 0.0008), and a significantly higher deposition in the central parts of the high-size 99mTc aerosol were observed (p = 0.0001). The PPV and the NPV were, respectively, 0.54 and 0.58 for 81mKr and 0.54 and 0.55 for Technegas. There was a significant negative correlation between 81mKr distribution and 133Xe clearance (p = 0.0001) between Technegas deposition and 133Xe clearance (p = 0.0007), and between 99mTc diethylene-triamino-penta-acetate (DTPA) deposition and 133Xe clearance (p = 0.001). Both the 81mKr peripheral distribution and Technegas peripheral deposition correlated negatively with increased obstruction, as measured by forced expiratory volume in 1 sec (FEV1). Peripheral deposition of the high-size 99mTc aerosol deposition correlated with the inspiration/expiration time ratio. In conclusion, 81mKr and 99mTc aerosols' lung scintigraphies do not reflect exactly the pulmonary ventilation as measured by 133Xe scintigraphy.

Surgical treatment of infrainguinal arterial occlusive disease in women.

PURPOSE: This study reviewed the outcome of 131 women who underwent infrainguinal bypass in 150 limbs from 1984 to 1991 for limb-threatening ischemia (95%) or disabling claudication (5%). METHODS: These women were compared with 209 men who underwent infrainguinal arterial reconstruction of 231 lower extremities for limb threat (89%) or claudication (11%) during the same interval. On average, women were 3 years older than men (mean age 72 vs 69 years, p < 0.005) but were less frequently cigarette smokers (56% women, 68% men, p < 0.05). Fifty-two percent of women had diabetes and 67% had hypertension, similar to the male patients. Infrainguinal disease distribution necessitated bypass to the above-knee popliteal artery in 10%, to the below-knee popliteal artery in 25%, and to the tibial or pedal arteries in 65% of women, comparable to the disease distribution in men. Autogenous vein grafts were performed in 90% of both groups. RESULTS: Early postoperative (30-day) mortality was 4% for women and 2% for men (not significant). Life-table survival after 3 years, however, was only 54% in women, compared with 72% in men (p < 0.05). Multivariate analysis indicated that diabetes increased the mortality rate 2.5-fold in women, which was not true in men. Three-year life-table survival of women with diabetes was only 39%, compared with 78% in women without diabetes (p < 0.001). Primary graft patency in women was 59% at 1 year and 54% at 3 years, significantly less than the 73% and 70% graft patency rates observed in men (p < 0.005). Secondary graft patency improved in women to 75% and 69% after 1 and 3 years, but this was still significantly less than the secondary patency rates of 89% and 86% observed in men (p < 0.001). Multivariate analysis indicated that female sex decreased secondary graft patency 2.4-fold and was the only variable associated with graft failure. Cumulative 3-year limb salvage in women was 82%, not statistically different than the 89% limb salvage rate observed in men. CONCLUSIONS: Women and men requiring arterial reconstruction for infrainguinal occlusive disease had comparable operative mortality and limb salvage rates, but long-term survival and graft patency were significantly reduced in women. Our results indicate that sex substantially influences the outcome of patients after infrainguinal bypass.

Current use of inferior vena cava filters.

To study possible changes in the clinical use of inferior vena cava (IVC) filters caused by the introduction of percutaneous delivery systems, we reviewed all patients who underwent placement of IVC filters at our institution from 1988 to 1991. Eighty-four patients (52 men and 32 women) ranging in age from 18 to 90 years (mean 67 years) were identified. Filters were required because of contraindications to anticogulation in 64% anticoagulation failure in 25%, and preoperative prophylaxis in 11% of patients. The underlying disease was lower extremity deep vein thrombosis in 50% and pulmonary embolism in 45% of patients. Five percent of patients received prophylactic filters without documented thromboembolism. All filters were placed percutaneously by interventional radiologists, 77 through the common femoral vein and 7 through the internal jugular vein. Three types of filters were used. One procedure-related death occurred because of acute IVC occlusion. Fatal pulmonary embolism within 48 hours after filter placement was documented in one patient and suspected in one late death. No other clinically apparent pulmonary embolism or leg swelling occurred after filter placement. Minor complications related to filter placement occurred in 13 patients, but none required operative intervention. Analysis of complication rates of the three filter types was precluded by the small sample size. After a mean follow-up of 11 months, 42 patients (50%) had died of malignancy (n = 25), multisystem organ failure (MSOF; n = 7), cardiovascular events (n = 4), recurrent pulmonary embolism (n = 2), cerebrovascular events (n = 4), or an unknown cause (n = 1). Twenty-three patients (27%) died before hospital discharge.(ABSTRACT TRUNCATED AT 250 WORDS)

A blinded comparison of angiography, angioscopy, and duplex scanning in the intraoperative evaluation of in situ saphenous vein bypass grafts.

Angiography, angioscopy, and duplex scanning have each been advocated for intraoperative assessment of in situ saphenous vein grafts. We compared these three modalities during operation in a prospective, blinded study during the construction of 20 femoral-infragenicular in situ saphenous vein grafts. Each modality was used and interpreted by a surgeon blinded to the results of the other studies. Abnormalities requiring intervention were defined as (1) patent vein side branches, (2) residual valve cusps, and (3) anastomotic stenoses greater than 30%. Criteria, specific to the modality, corresponding to each category were prospectively defined. Fourteen residual valve cusps, 49 patent vein branches, and 6 anastomotic stenoses were suggested by at least one modality. Nine residual valve cusps, 32 patent vein branches, and no anastomotic stenoses were actually found (and corrected) by direct inspection. Sensitivity of detecting patent side branches for angiography, duplex scanning, and angioscopy was 44%, 12%, and 66%, respectively. Both angiography and angioscopy were significantly more sensitive than duplex scanning for detection of unligated side branches (p less than 0.01). Sensitivity of detecting residual valve cusps was 22% (angiography), 11% (duplex scanning), and 100% (angioscopy). Angioscopy was significantly more sensitive than either duplex scanning or angiography in detection of residual valve cusps (p less than 0.01). Since no anastomotic stenoses were confirmed, the false-positive rates for stenosis detection were 20% for angiography, 10% for duplex scanning, and 0% for angioscopy. Time requirement was 17 to 20 minutes and did not differ among the three modalities. No stenosis or arteriovenous fistula has been detected in any graft by postoperative duplex surveillance (mean, 10-month follow-up).(ABSTRACT TRUNCATED AT 250 WORDS)