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.

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.

[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.

[Correlation between urinary catecholamines dosage and apnea-hypopnea index in a hypertension population: pilot study]. / Corrélation entre les dosages des dérivés methoxylés urinaires et l'index d'apnée-hypopnée dans une population d'hypertendus : étude pilote.

AIMS: Sleep disorders like obstructive sleep apnea in adults are associated with increased sympathetic activity, which induced high blood pressure and could be associated with resistant hypertension. Some studies have demonstrated that high urinary catecholamine levels in obstructive sleep apnea patients may be decreased with continuous positive airway pressure therapy. However, very few studies have demonstrated a correlation between apnea-hypopnea index and urinary catecholamine levels in hypertension patients. METHODS: In this pilot study, 20 hypertensive patients referred for hypertension work-up including night-time polygraphy and 24h urinary catecholamine dosage were included. RESULTS: Mean age was 51±11 years (30-76), 68% were males. Diagnosis of obstructive sleep apnea was confirmed in 13 patients at the end of the work-up. Mean apnea-hypopnea index was 14±9 (2-32). The only urinary catecholamine parameter significantly increased in patients with obstructive sleep apnea was 24h urinary normetanephrine (1931±1285 vs 869±293nmol/24h; P<0.05). However, this difference was not significant when this parameter was adjusted to 24h urinary creatinine. We observed a significant positive correlation between AHI and 24h urinary normetanephine (r=0.486; P=0.035). CONCLUSION: This pilot study confirms an isolated elevation of 24h urinary normetanephrine in hypertensive patients with obstructive sleep apnea and shows a significant correlation between sleep disorders expressed by apnea-hypopnea index and urinary catecholamines excretion.

[Idiopathic retroperitoneal fibrosis: a multicentric retrospective study of 30 French cases and follow-up of the renal function]. / Fibrose rétropéritonéale idiopathique : évolution à long terme du pronostic rénal dans une série rétrospective multicentrique de 30 cas.

PURPOSE: Idiopathic retroperitoneal fibrosis (IRF) is an inflammatory disorder, affecting the aorta and the surrounding vessels and tissues. The prognosis is mainly driven by the risks of chronic kidney disease and relapse. Our aim was to assess the prevalence of chronic kidney disease at follow-up. METHODS: We retrospectively reviewed the medical records of patients diagnosed for IRF in Seine-Saint-Denis (France) between 1987 and 2011. We collected informations about presentation, radiologic findings and follow-up. Diagnosis of IRF was confirmed when all the following criteria were met: infiltration of the infrarenal aorta or iliac vessels, absence of aneurysmal dilation, lack of clinical suspicion of malignancy. RESULTS: Thirty patients were identified, with a male/female ratio of 4.9. Mean age was 55±13 years old. The mean creatinine clearance was 66 mL/min/1.73 m(2) and the mean CRP was 45±36 mg/L. In 24 (80%) patients, the location of IRF was periaortic and periiliac. Eleven patients (37%) underwent a diagnostic biopsy, and 14 (47%) required an ureteral procedure. A mean follow-up of 63 months was available for 29 patients: 69% relapsed, 7 developed chronic renal disease (24%), and one died of urinary sepsis. Older age (P=0.023), diabetes (P=0.007), and initial renal insufficiency (P=0.05) were associated with a risk of chronic renal insufficiency. CONCLUSION: The high frequency of relapses and chronic renal disease emphasizes the need of close follow-up in patients diagnosed with IRF.

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.

Protease inhibitor treatment effect on aortic stiffness in normotensive patients with human immunodeficiency virus infection.

OBJECTIVES: Human immunodeficiency virus (HIV) infection and protease inhibitor (PI)-based antiretroviral treatment might increase large artery (aortic) stiffness compared with healthy untreated controls. To clarify the role of PI therapy in the progression of subclinical arteriosclerosis in patients with HIV, we investigated the impact of PI treatment on arterial stiffness. METHODS AND RESULTS: In our single-centre, cross-sectional study, normotensive male HIV patients free from overt cardiovascular disease received PI treatment (n=60) or no PI treatment (n=42). The PI group had a significantly higher pulse wave velocity (PWV) than the PI-free group (9.0 ± 1.4 vs. 8.1 ± 1.3m/s; P=0.016). There was a significant positive correlation between age and PWV in the PI-free group (R(2) 0.310; P<0.0001) and, to a lesser extent, in the PI group (R(2) 0.181; P<0.0001). PI treatment was associated with a significant increase in the adjusted slope of the curve relating age to PWV as compared with no PI treatment. CONCLUSIONS: In normotensive HIV patients, PI treatment significantly increases both aortic stiffness and the positive correlation between PWV and age. Aortic stiffness predicts cardiovascular mortality, thus these results provide new insight on the relationship between PI treatment, mechanical arteriosclerotic and cardiovascular risk.

Limbic encephalitis and type 1 diabetes with glutamic acid decarboxylase 65 (GAD65) autoimmunity: improvement with high-dose intravenous immunoglobulin therapy.

Glutamic acid decarboxylase antibodies (GAD-abs) are an immunological factor involved in type 1 diabetes and other diseases involving the central nervous system. This report is of a patient with type 1 diabetes and a rare case of non-paraneoplastic limbic encephalitis mediated by anti-GAD65 antibodies that improved with the use of immunosuppressive drugs.

[Management of orbital inflammation in internal medicine: a retrospective case series of 29 patients]. / Prise en charge de l'inflammation orbitaire en médecine interne : à propos d'une série de 29 patients consécutifs.

PURPOSE: To describe a case series of patients investigated in internal medicine for an inflammation of the orbit and to clarify the clinical and pathological features of patients with idiopathic orbital inflammatory syndrome (IOIS). PATIENTS AND METHODS: Forty patients were consecutively referred by a specialized center where an orbital biopsy was performed in case of accessible lesion. Eleven patients were excluded because of missing data or diagnosis of lymphomas, periorbital xanthogranulomas, or Graves' disease. Patients with systemic disease (SD) or auto-immune disorder (AID) that validated the international criteria, or those having an IOIS in the absence of local or systemic etiology, were included. The clinicopathologic and immunologic characteristics of IOIS patients, their treatment and their evolutionary profiles are reported according to the histological types described by Mombaerts. RESULTS: Of the 29 patients enrolled, eight had a dacryoadenitis revealing a SD/AID, mainly a necrotizing vasculitis, seven patients had a presumed IOIS and 14 an IOIS histologically documented. The presentation of IOIS was dominated by a diffuse involvement of the orbit. Corticosteroids were administered alone or with an immunosuppressant in 57 and 24% of IOIS patients, respectively. The incidence of relapse/resistance was higher than that of remission, particularly in case of presumed IOIS or in its classical form. All four patients with a stage III-IV of Chisholm were relapsing or resistant. CONCLUSION: A dacryoadenitis may reveal some types of SD/AID. Unlike the severe sialadenitis, the form of sclerosing IOIS may not be a factor associated with relapse or resistance.

[Vascular involvement in Cogan's syndrome. A case report]. / Atteinte vasculaire et syndrome de Cogan. Une observation.

Vascular manifestations of Cogan's syndrome are rarely reported. We report the case of a young woman followed for typical Cogan's disease. Serious vascular involvement was found only during work-up for arterial hypertension. This case highlights potentially asymptomatic nature of extensive vasculitis affecting large and medium-sized vessels in Cogan's disease. Careful screening is required to prevent life-threatening complications.

[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.