Large-vessel involvement in ANCA-associated vasculitis: A multicenter case-control study.

OBJECTIVE: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) primarily affects small vessels. Large-vessel involvement (LVI) is rare. We aimed to describe the characteristics of LVI, to identify associated risk factors, and to describe its therapeutic management. METHODS: This multicenter case-control (1:2) study included patients with AAV according to the ACR/EULAR classification and LVI as defined by the Chapel Hill nomenclature, together with controls matched for age, sex, and AAV type. RESULTS: We included 26 patients, 15 (58 %) of whom were men, with a mean age of 56.0 ± 17.1 years. The patients had granulomatosis with polyangiitis (n = 20), or microscopic polyangiitis (n = 6). The affected vessels included the aorta (n = 18; 69 %) supra-aortic trunks (n = 9; 35 %), lower-limb arteries (n = 5; 19 %), mesenteric arteries (n = 5; 19 %), renal arteries (n = 4; 15 %), and upper-limb arteries (n = 2; 8 %). Imaging showed wall thickening (n = 10; 38 %), perivascular inflammation (n = 8; 31 %), aneurysms (n = 5; 19 %), and stenosis (n = 4; 15 %). Comparisons with the control group revealed that LVI was significantly associated with neurological manifestations (OR=3.23 [95 % CI: 1.11-10.01, p = 0.03]), but not with cardiovascular risk factors (OR=0.70 [95 % CI: 0.23-2.21, p = 0.60]), or AAV relapse (OR=2.01 [95 % CI: 0.70-5.88, p = 0.16]). All patients received corticosteroids, in combination with an immunosuppressant in 24 (92 %), mostly cyclophosphamide (n = 10, 38 %) or rituximab (n = 9, 35 %). CONCLUSION: Regardless of distinctions based on vessel size, clinicians should consider LVI as a potential manifestation of AAV, with the aorta commonly affected. The risk of developing LVI appears to be greater for clinical phenotypes of AAV with neurological involvement. Standard AAV treatment can be used to manage LVI.

Detection of asymptomatic aortic involvement in ANCA-associated vasculitis using FDG PET/CT.

Large vessel involvement in ANCA-associated vasculitis is very rare. We report here on the case of two patients with ANCA-associated vasculitis and asymptomatic aortic arch involvement diagnosed using FDG-PET/CT. Because aortic involvement in ANCA-associated vasculitis is a potentially life-threatening condition, its early detection can be crucial. FDG-PET/CT may also provide new insights into large vessel involvement as part of the spectrum of ANCA-associated vasculitis.

Tocilizumab and COVID-19: Timing of administration assessment.

Recent evidence showed greater efficacy of tocilizumab (TCZ) in the subgroups of COVID-19 patients who presented with symptoms for less than 7 days and in those only receiving oxygen. We retrospectively analyzed a compassionate use cohort to determine the best timing for TCZ injection. We showed no association between the timing of injection after symptom onset and the efficacy of TCZ on mortality. We then investigated whether the oxygen level at the time of TCZ injection impacted the mortality rate. Our study finally suggested that TCZ could be less effective when oxygen requirement is >11L/min and we hypothesized that earlier administration could be associated with better outcome. However, randomized clinical trials are required to confirm this hypothesis.

[Management of orbital inflammatory disorders in internal medicine: New findings resulting from a retrospective study of 31 consecutive patients]. / Prise en charge des orbitopathies inflammatoires en médecine interne : nouvelles données issues de l'étude d'une série de 31 patients consécutifs.

PURPOSE: To describe a case series of patients investigated in internal medicine for orbital inflammation (OI) since the individualization of the clinical entity of the IgG4-related orbital disease (IgG4 ROD). PATIENTS AND METHODS: Thirty four patients were consecutively referred by a specialized center where orbital biopsy was performed when the lesion was surgically accessible. Fourteen patients were excluded in case of missing data or lymphoma, periocular xanthogranuloma or Graves' orbitopathy. Patients with systemic or auto-immune disease according to the international criteria, or presenting with idiopathic orbital inflammation syndrome (IOIS), were included. Knowing the histological similarities between IOIS and IgG4 ROD, immunohistochemical assessment of plasma cells for IgG4 positivity was performed for every patient with available biopsy. Clinical and biological characteristics, treatment and response to treatment of included patients are reported. RESULTS: Among 22 included patients, 10 presented with orbital manifestation of a systemic or autoimmune disease including 2 sarcoidosis (9%) and 8 (36%) cases of non specific OI which were reclassified in IgG4 ROD. Finally, IOIS of various clinicopathological presentation was diagnosed for 12 patients including 10 with histological documentation. Whereas relapse and resistance were not found to be related to IgG4 positivity (50% in both IOIS and IgG4 ROD groups), another treatment in addition to corticosteroids was more often necessary in IgG4 ROD patients (50%) than in IOIS patients (25%). CONCLUSION: After ruling out auto-immune orbital diseases, especially IgG4 ROD, IOIS should be discussed. Factors conditioning the corticosteroid response are yet to be determined.

[Association between psychiatric comorbidities and hospital length of stay in post-emergency internal medicine patients]. / Association des comorbidités psychiatriques avec la durée de séjour des patients en médecine interne d'aval des urgences.

INTRODUCTION: Patients with psychiatric disorders suffer from a higher rate of somatic disorders than those without psychiatric disorder, often inappropriately managed. Our study aimed to describe patients with psychiatric comorbidity in post-emergency internal medicine units and to compare their length of hospital stay to patients without psychiatric disease. METHODS: This French cross sectional study used the data warehouse of the greater Paris hospitals. It included, all patients hospitalized through the emergency department in 9 internal medicine departments during the year 2017. Psychiatric disorders and the burden of somatic disorders (Charlson score) were determined through diagnostic coding. Charlson score and hospital length of stay were compared between patients with and without psychiatric comorbidity. RESULTS: In total, 8981 hospital stays (8001 patients) were included, 1867 (21%) with psychiatric comorbidity. After adjusting for age, gender, hospital and main diagnosis, the Charlson score was on average 0.68 higher in the psychiatric comorbidity group (P<0.001) and the length of hospital stay was 30% higher after further adjustment on the Charlson score (P<0.001). These differences were consistent for each main diagnosis. CONCLUSION: Patients with psychiatric comorbidity are frequent in post-emergency internal medicine wards. They experience longer hospital stays, only partly related with a higher burden of somatic disorders. Special attention should be paid to this vulnerable population.

[Guidelines for the management of uveitis in internal medicine]. / Uvéites et médecine interne : stratégies diagnostique et thérapeutique.

In developed countries, uveitis is quite common and chronic course is associated with a poor visual prognosis. However, no guidelines for their management have been consensually proposed. Based on the experience of ophthalmologists and internists from tertiary care centers, this article describes the management of uveitis, including the diagnostic procedure, indications and types of anti-inflammatory treatments. We focus on the interest of an exhaustive ophthalmologic examination for the diagnosis of an underlying systemic disease such as sarcoidosis. In this way, a multidisciplinary staff could be useful for an optimal management of uveitis. We also reviewed the main current treatments for severe uveitis. Whatever the origin of ocular inflammation, corticosteroids are consensually used as first line treatment. However, the identification of an underlying systemic disease helps in the refinement of further therapeutic choices. In cases of refractory and sight threatening uveitis, the short-term use of infliximab, a chimeric anti-TNF-alpha antibody, has been shown to be effective and safe. These recommendations do not constitute treatment guidelines but aim at improving the uniformity of clinical practice for the management of uveitis, until higher levels of evidence are obtained.

[Study of a case of cochlear implant with recurrent cutaneous extrusion]. / Extrusion cutanée à répétition d'un implant cochléaire.

OBJECTIVE: An observation of a difficult-to-diagnose complication of the cochlear implant: recurring cutaneous extrusion of a cochlear implant is reported. PATIENTS AND METHODS: The history of an adult patient with total deafness is reported. She received an implant in her right ear in 1988, which was then explanted because of absence of hearing results. She received a second implant in her left ear in 2002, which was then explanted in 2007 because of cutaneous extrusion. In 2008, a second implant of a different brand was placed in her left ear, with the central part placed away from the first site, but extrusion recurred. A new attempt to encapsulate the central part with a hydroxyapatite box also ended in extrusion and was explanted in 2008. RESULTS: Various diagnoses to explain these cutaneous problems were suggested during this clinical progression: infection, allergy, and a reaction to a foreign body. No hypothesis could be clearly ruled out. CONCLUSION: Cutaneous complications after cochlear implant are exceptional. As soon as cutaneous disorders appear, a rigorous diagnostic process must be followed so that the patient can be recommended a long-lasting solution to restore quality hearing.

[Rhabdomyolysis in patients treated by corticosteroids for acute asthma in intensive care unit]. / La corticothérapie, une cause de rhabdomyolyse chez les asthmatiques en réanimation.

INTRODUCTION: Acute muscle involvement is an infrequent complication of corticosteroids, characterized by muscle weakness and a rhabdomyolysis, rapidly regressive after withdrawal of corticosteroids. CASE REPORT: We report the case of a woman admitted in intensive care unit for acute severe asthma, treated with high doses of methylprednisolone. Serum CPK level raised with a peak at 28,160 UI/L (n<250 UI/L) at day 15, suggesting acute rhabdomyolysis with renal failure. CPK rapidly normalized when corticosteroids were discontinued. Other causes of rhabdomyolysis were ruled out. CONCLUSION: This necrosing myopathy under high doses of corticosteroids has been described in patients with severe acute asthma. The mechanism of the muscle damage results from a combination of corticosteroids toxicity, respiratory acidosis and mechanic ventilation.

[A muscular calcium pyrophosphate deposition pseudo-abscess: An atypical localization of chondrocalcinosis]. / Pseudo-abcès musculaire d'origine microcristalline : une localisation exceptionnelle de chondrocalcinose.

INTRODUCTION: Chondrocalcinosis results from calcium pyrophosphate crystals deposition in the joints. We report an exceptional case of aseptic psoas abscess with a deposition of calcium pyrophosphate crystals. CASE REPORT: A 92-year-old man presented to our department for an acute onset of inflammatory pain in the left hip. Computed tomography detected a coxofemoral arthritis and multiple intramuscular collections located in the iliopsoas muscle and the gluteus minimus. A sample of the fluid was obtained with a guided aspiration, and its analysis revealed an inflammatory liquid with no bacteria but numerous calcium pyrophosphate crystals. The final diagnosis was thus a muscular calcium pyrophosphate deposition pseudo-abscess, associated with a hip arthritis. CONCLUSION: Hip chondrocalcinosis is unusual, and the association with intramuscular deposition of calcium pyrophosphate crystals seems extremely rare as we found only four other published cases. A microcrystalline arthritis could have spread from the coxofemoral joint through the iliopsoas bursa and into the muscle. However, the imaging aspect with an abscess and a predominant muscular injury might suggest a mechanism of crystal formation originating directly within the muscle. The outcome was always favourable even if some patients required surgery.

[Vitamine K antagonists overdose: Physician adherence to the French guidelines]. / Surdosages en antivitamines K : audit de la prise en charge hospitalière.

INTRODUCTION: Vitamin K antagonists (VKA) are drugs with a major risk of side effect. Guidelines have been published in 2008 by the Haute Autorité de santé (HAS) concerning the management of an excessively elevated INR ratio. Our research aimed to assess physicians' adherence to those guidelines. METHODS: We realized a retrospective, multicentric study. One hundred and ten cases of excessively elevated INR ratio were identified and analyzed. RESULTS: Overall physicians adherence was 58%. However, patients with the most elevated INR, i.e., INR>6, were treated according to guidelines in only 33% of the cases. The use of vitamin K was the major source of mistakes. The rate of mortality was 20%. CONCLUSION: Adherence to HAS guidelines seems finally limited. It is necessary to put in place procedures to secure the behavior of physicians.

[Usefulness of different techniques of blood pressure measurements in 2016]. / Place des différentes techniques de mesure de la pression artérielle en 2016.

The management of hypertensive patients is greatly influenced by blood pressure levels and accurate measurement of blood pressure is crucial in this context. Mercury sphygmomanometer has been progressively replaced by more precise oscillometric devices that can be widely used in the clinic and ambulatory setting. The purpose of this review was to detail the different methods for evaluating blood pressure, and to refine their indications and clinical benefit. Office blood pressure measurement has a great variability and should follow a strict protocol to give consistent results. National and international guidelines focus on blood pressure measurement in the ambulatory setting. When used by trained patients, home blood pressure monitoring is reproducible and can provide substantial prognostic information, even if ambulatory blood pressure monitoring remains the gold standard. The role of central blood pressure and pulse wave velocity monitoring in the therapeutic strategy of hypertension needs further assessment.

Rheumatoid Factor and Disease Activity Are Independent Predictors of Lymphoma in Primary Sjögren's Syndrome.

OBJECTIVE: To define parameters predictive of lymphoma development in patients with primary Sjögren's syndrome (SS). METHODS: A multicenter case-control survey was performed to identify predictors of lymphoma. Cases were patients who developed lymphoma after diagnosis of primary SS and were mainly recruited through the Club Rhumatismes et Inflammation network. For each case, 2 controls (matched for disease duration and age) were randomly selected among patients with primary SS and without lymphoma. Cases and controls were compared using univariate analysis and then using multivariate analysis to identify independent predictors of lymphoma. RESULTS: One hundred one patients with primary SS and lymphoma were included. Eighty-seven patients were women (86.1%), and the mean ± SD age at lymphoma diagnosis was 57.4 ± 12.6 years. The most frequent histologic type was B cell non-Hodgkin's lymphoma (NHL) in 99 of 101 patients, with marginal-zone lymphoma in 76 of the 99 patients (76.8%) including 58 (58.6%) with lymphoma of the mucosa-associated lymphoid tissue type. Lymphomas were most frequently located in the salivary glands (43 patients). A specific treatment was initiated at diagnosis in 87 patients with B cell NHL, and 61 patients (61.6%) achieved complete sustained remission after the first line of treatment. In the multivariate analysis, salivary gland enlargement, the presence of rheumatoid factor (RF), low C4, cryoglobulinemia, lymphopenia, and disease activity according to the European League Against Rheumatism Sjögren's Syndrome Disease Activity Index (excluding the lymphoma domain) were found to be predictors of lymphoma. No previous treatment for primary SS was associated with any effect on lymphoma occurrence. CONCLUSION: In addition to previously known factors predictive of lymphoma occurrence, the independent roles of RF and disease activity were demonstrated in this case-control study of primary SS-associated lymphoma. Our findings highlight the roles of chronic antigenic stimulation and disease activity in the development of this severe complication.

[Strokes associated with cervical artery dissection, and systemic mastocytosis: an unfortuitous association? A report of two cases]. / Accident vasculaire cérébral sur dissection artérielle, au cours des mastocytoses systémiques: une association non fortuite? A propos de deux cas.

INTRODUCTION: Neuropsychiatric symptoms in systemic mastocytosis are usually cognitive and affective changes. EXEGESIS: We describe here two systemic mastocytosis patients without eosinophilia presenting strokes associated with cervical artery dissection. CONCLUSION: These observations are the first reported and they suggest that systemic mastocytosis could be add to the predisposing factors of spontaneous cervical artery dissections.

[Uveitis associated with thyroïditis in HTLV-1 carriers]. / Uvéite et thyroïdite associée à HTLV-1.

INTRODUCTION: The oncovirus HTLV-1 is aetiologically associated with uveitis and autoimmune thyroiditis in endemic areas. The association of uveitis with autoimmune thyroiditis in HTLV-1 carriers is less common moreover in non-endemic area. EXEGESE: We report two original cases of simultaneous uveitis and autoimmune thyroiditis in HTLV-1 carriers, without other disease due to HTLV-1. The visual outcome was favorable in both cases. CONCLUSION: A significant correlation exists between hyperthyroidism, uveitis and HTLV-1, but still needs to be confirmed. The autoimmune or immune mediated mecanism of HTLV-1 may be involved in the uveitis and the thyroidits.

Orocecal transit time in humans assessed by sulfapyridine appearance in saliva after sulfasalazine intake.

PURPOSE: We propose a noninvasive method for the measurement of orocecal transit time assessed by the sulfapyridine appearance time in saliva after ingestion of sulfasalazine. METHOD: In 12 healthy volunteers, we studied the correlation between plasma and saliva sulfapyridine appearance times and then the sulfapyridine appearance times in saliva under various experimental conditions to assess the reproducibility, the effects of meals, and the role of the formulation, and the effects of gastrointestinal kinetic drugs. RESULTS: The correlation between saliva and plasma sulfapyridine appearance times was strong (r = 0.84; p = 0.0004). The sulfapyridine saliva appearance time was significantly delayed by the meal. Compared with placebo, the saliva sulfapyridine appearance time was reduced by cisapride (312 +/- 128 versus 551 +/- 97 minutes; p = 0.0001) and increased by loperamide (674 +/- 267 versus 501 +/- 131 minutes; p = 0.044). CONCLUSION: We propose the salivary sample method as a validated simplification of the plasma sulfasalazine-sulfapyridine test for the measurement of orocecal transit time.

Acquired hemophilia due to factor VIII inhibitors in 34 patients.

BACKGROUND: Acquired hemophilia is a rare disease caused by the development of auto-antibodies against factor VIII. SUBJECTS AND METHODS: We studied the characteristics and outcomes of 34 patients (19 women and 15 men) with acquired hemophilia from 1980 to 1997. RESULTS: The mean age of the patients was 61 years (range, 22-93 years). An underlying disease was observed in 18 (53%) patients: 5 patients had cancer, 4 an autoimmune disorder, 2 a dermatologic disorder, 3 asthma, 3 were postpartum, and 1 had an adverse reaction to ampicillin. Factor VIII level was <5% in 30 (90%) patients; factor VIII antibodies were elevated (>10 Bethesda units) in 23 (69%) patients. Bleeding requiring transfusions was reported in 25 (75%) patients. Human factor VIII was given to 14 patients and porcine factor VIII to 5. Six patients received prothrombin complex concentrates and one desmopressin. Several immunosuppressive treatments were used, mainly corticosteroids, cyclophosphamide, and intravenous immunoglobulin. Bleeding stopped in all but one patient within 2 weeks. Most patients achieved complete remission, although two relapses were observed subsequently. CONCLUSION: This large study helps to clarify the presentation and clinical course of acquired hemophilia. Prospective studies are needed to determine the efficacy of treatment.

D-dimer testing as the initial test for suspected pulmonary embolism. Appropriateness of prescription and physician compliance to guidelines.

OBJECTIVES: Recent studies have shown that strategies for pulmonary embolism diagnosis which have included D-dimer testing have been most cost effective. The objective of this study is to evaluate the impact of a new strategy for pulmonary embolism diagnosis based on D-dimer results. METHODS: A prospective survey was conducted in the emergency ward and three medical departments of a university teaching hospital. Guidelines for diagnosis of PE were established and implemented through an educational intervention and a specific order form. D-dimer (ELISA) was required for all patients suspected of having PE. A result above 500 ng/ml was to be followed by an a pulmonary imaging procedure. Appropriateness of prescription of D-dimer and non-compliance with guidelines (absence of diagnostic imaging procedure following D-dimer results above 500 ng/ml) were evaluated. RESULTS: One-hundred sixty patients were studied. D-dimer test was performed in 154 patients (96.3%) suspected of PE during a two-month period. Test results were above 500 ng/ml in 111 cases. PE was confirmed in 20 cases. Twenty percent (31/154) of the D-dimer prescriptions were inappropriate. Among those with D-dimer results above 500 ng/ml, 45% (50/111) of the patients experienced no imaging procedure. CONCLUSIONS: Despite implementation of clinical guidelines for its use, D-dimer was excessively prescribed. A large proportion of results was not taken in consideration by prescribers. Often new technologies have good experimental results, but behave differently when used routinely in ordinary care settings. It is important that field studies be developed to evaluate the effectiveness of new technologies.

Central retinal vein thrombosis associated with resistance to activated protein C.

PURPOSE/METHODS: Activated protein C resistance was recently described as a major cause of venous thrombosis. We observed a central retinal vein thrombosis in a woman with activated protein C resistance. RESULTS/CONCLUSION: DNA analysis showed the patient to be heterozygous for the factor V gene mutation, which is related to activated protein C resistance. Patients with retinal vein thrombosis should be examined for activated protein C resistance.

Adie syndrome as the initial sign of primary Sjögren syndrome.

PURPOSE: To report Adie syndrome as the initial sign of primary Sjögren syndrome. METHODS: Case report. RESULTS: Adie syndrome was associated with necrotizing gingivitis and xerostomia. Antibodies against Ro (SS-A) were present. Prednisone and antimalarial drugs were ineffective in treating Adie syndrome but improved the necrotizing gingivitis. CONCLUSION: Search for Sjögren syndrome is mandated in patients with Adie syndrome. The latter condition is likely related to ganglionitis, a mechanism responsible for peripheral nervous system involvement in primary Sjögren syndrome.