Clinical features and long-term outcomes of patients with systemic polyarteritis nodosa diagnosed since 2005: Data from 196 patients.

BACKGROUND: The landscape of polyarteritis nodosa (PAN) has substantially changed during the last decades. Recent data regarding causes, characteristics, and prognosis of systemic PAN in the modern era are lacking. METHODS: This retrospective study included patients with systemic PAN referred to the French Vasculitis Study Group between 2005 and 2019. Characteristics, associated conditions and outcomes were collected, and predictors of relapse and death were analyzed. RESULTS: 196 patients were included. Main clinical symptoms were constitutional (84%), neurological (59%), skin (58%) and musculoskeletal (58%) manifestations. Secondary PAN accounted for 55 (28%) patients, including myelodysplastic syndrome (9%), solid cancer (7%), lymphoma (4%) and autoinflammatory diseases (4%). No patient had active HBV infection. All treated patients (98.5%) received glucocorticoids (GCs), alone (41%) or in combination with immunosuppressants (59%), with remission achieved in 90%. Relapses were independently associated with age >65 years (HR 1.85; 95% CI1.12-3.08), gastrointestinal involvement (1.95; 95% CI1.09-3.52) and skin necrotic lesions (HR 1.95; 95%CI 1.24-3.05). One-, 5- and 10-year overall survival rates were 93%, 87% and 81%, respectively. In multivariate analyses, age >65 years (HR 2.80; 95%CI 1.23-6.37), necrotic purpura (HR 4.16; 95%CI 1.62-10.70), acute kidney injury (HR 4.89; 95% 1.71-13.99) and secondary PAN (HR 2.98; 95%CI 1.29-6.85) were independently associated with mortality. CONCLUSION: Landscape of PAN has changed during the last decades, with the disappearance of HBV-PAN and the emergence of secondary PAN. Relapse rate remains high, especially in aged patients with gastrointestinal and cutaneous necrosis, as well as mortality.

Venous thromboembolism during systemic inflammatory and autoimmune diseases associated with myelodysplastic syndromes, chronic myelomonocytic leukaemia and myelodysplastic/myeloproliferative neoplasms: a French multicentre retrospective case-control study.

OBJECTIVES: Myelodysplastic syndromes (MDS) and chronic myelomonocytic leukaemia (CMML) are associated with systemic inflammatory and autoimmune diseases (SIADs) in 10-30% of cases. The aims of this study were (i) to evaluate the prevalence of venous thromboembolism VTE in patients presenting with both MDS/CMML and SIADs, (ii) to describe risk factors associated with thrombosis, and (iii) to analyse the impact of VTE on overall survival and transformation to acute myeloid leukaemia in comparison to patients with MDS/CMML-associated SIADs without VTE. METHODS: This retrospective multicentre case-control study was conducted among patients with MDS/CMML and dysimmune disorders and featured in the French retrospective database of the French Network of Dysimmune Disorders Associated with Hemopathies (MINHEMON), diagnosed with MDS/CMML and dysimmune disorders. RESULTS: During a median follow-up of 16 months (5-48) VTE occurred in 35 patients (21.6 %) whereas 127 patients did not. Among those with VTE, 8 patients (22.9%) experienced two or more VTE. Common prothrombotic risk factors were not significantly different in patients with or without VTE. CMML was more frequent in patients without VTE (37 % vs. 14.3%, p=0.01), whereas myelodysplasic/myeloproliferative neoplasm (MDS/MPN) was higher in VTE patients (20 % vs. 5.5 %, p=0.01). In a multivariate analysis, only MDS/CMML progression at the time of VTE (odds ratio 28.82, 95 % CI (5.52-530.70) was significantly associated with VTE. When treated with an anticoagulation therapy, bleeding occurred in 19.4% of cases (6/31). Overall survival was not significantly different between patients with and without VTE (p=0.68). Leukaemia-free survival between groups was not significantly different (p=0.83). CONCLUSIONS: VTE is a common complication in MDS/CMML-associated SIADSs with an increased risk of bleeding when treated by anticoagulants. In the MDS/CMML subgroup, SIADS flares and MDS/CMML progression seem to be prothrombotic risk factors.

A descriptive, retrospective case series of patients with factitious disorder imposed on self.

BACKGROUND: Despite cases of factitious disorder imposed on self being documented in the literature for decades, it appears to remain an under-identified and under-diagnosed problem. The present study aimed to explore factitious disorder imposed on self in a series of French patients. METHODS: Patients 18 years old and over with factitious disorder imposed on self were retrospectively included by two independent reviewers according to DSM-5 criteria in Rennes University Hospital for the period 1995 to 2019. Patients were identified from a clinical data warehouse. RESULTS: 49 patients with factitious disorder imposed on self were included. Among them, 36 (73.5%) were female. The average age at diagnosis was 38.4 years. The 16 patients with a health-related profession were all female. Direct evidence of falsification was found in 20.4% of cases. Falsification was mainly diagnosed on the basis of indirect arguments: history of factitious disorder diagnosed in another hospital (12.2%), extensive use of healthcare services (22.4%), investigations that were normal or inconclusive (69.4%), inconsistent or incomplete anamnesis and/or patient refusal to allow access to outside information sources (20.4%), atypical presentation (59.2%), evocative patient behaviour or comments (32.7%), and/or treatment failure (28.6%). Dermatology and neurology were the most frequently involved specialities (24.5%). Nine patients were hospitalized in intensive care. Some of them received invasive treatments, such as intubations, because of problems that were only reported or feigned. The diagnosis of factitious disorder imposed on self was discussed with the patient in 28 cases (57.1%). None of them admitted to making up the disorder intentionally. Two suicide attempts occurred within 3 months after the discussion of the diagnosis. No deaths were recorded. 44.9% of the patients returned to the same hospital at least once in relation to factitious disorder imposed on self. CONCLUSIONS: The present study reinforces data in favour of a predominance of females among patients with factitious disorder imposed on self. This diagnosis is difficult and is based on a range of arguments. While induced cases can be of low severity, cases that are only feigned can lead to extreme medical interventions, such as intubation.

Factitious disorder imposed on self: A retrospective study of 2232 cases from health insurance databases.

OBJECTIVE: Patients with factitious disorder imposed on self (FDIS) seek medical care for deliberately falsified problems. Although a large amount of work has been published, the scientific literature lacks robust data on FDIS. The present study aimed to estimate the annual mean of in-hospital FDIS codings in France, describe the sociodemographic characteristics of subjects with FDIS, assess healthcare utilisation and medical nomadism, and describe the pathologies most frequently associated with FDIS. METHOD: Subjects with at least one coding of FDIS in French health insurance databases between January 1, 2009, and December 31, 2017 were included. Subjects younger than 18 years of age at the time of first coding were excluded from the study. Sociodemographic data of subjects and diagnoses associated with the first coding of FDIS were collected. Healthcare utilisation and medical nomadism were analysed descriptively from one year before to one year after the first FDIS coding. RESULTS: 2232 subjects were included, representing an average of 248 new in-hospital FDIS codings per year. The subjects included were 58.2% female. The mean age at diagnosis was 48.5 years. In the year following the first coding of FDIS, 1268 subjects (56.8%) were re-hospitalised at least once, including 159 (7.1%) with at least one new coding for FDIS. From one year before to one year after the first coding of FDIS, 66% of the subjects included had received at least one prescription for benzodiazepines, 58.3% for antidepressants, and 42.6% for antipsychotics. CONCLUSIONS: Our findings bring new data working towards a better understanding of FDIS. The consumption of psychotropic drugs is particularly frequent in patients with FDIS.

Comparison between idiopathic and VEXAS-relapsing polychondritis: analysis of a French case series of 95 patients.

OBJECTIVE: A new adult-onset autoinflammatory syndrome has been described, named VEXAS (Vacuoles, E1 Enzyme, X-linked, Autoinflammatory, Somatic). We aimed to compare the clinical characteristics, the laboratory features and the outcomes between idiopathic-relapsing polychondritis (I-RP) and VEXAS-relapsing polychondritis (VEXAS-RP). METHODS: Patients from French retrospective multicentre cohort of RP were separated into two groups: a VEXAS-RP and an I-RP. RESULTS: Compared with patients with I-RP (n=40), patients with VEXAS-RP (n=55) were men (96% vs 30%, p<0.001) and were older at diagnosis (66 vs 44 years, p<0.001). They had a greater prevalence of fever (60% vs 10%, p<0.001), of skin lesions (82% vs 20%, p<0.001), of ocular involvement (57% vs 28%, p=0.01), of pulmonary infiltrates (46% vs 0%, p<0.001), of heart involvement (11% vs 0%, p=0.0336) and with higher median C-reactive protein levels (64 mg/L vs 10 mg/L, p<0.001). Seventy-five per cent of the patients with VEXAS-RP had myelodysplastic syndrome (MDS) versus none in I-RP group. The glucocorticoids use, and the number of steroid sparing agents were similar in both groups, but patients with VEXAS-RP had more frequent refractory disease (remission obtained in 27% vs 90%, p<0001). VEXAS-RP was associated with higher risk of death: six patients (11%) died in the VEXAS-RP group after a median follow-up of 37 months and none in the I-RP group after a median follow-up of 92 months (p<0.05). CONCLUSION: We report the largest cohort of VEXAS-RP, characterised by high prevalence of male sex, fever, skin lesion, ocular involvement, pulmonary infiltration, heart involvement, older age and MDS association.

Gender inequality among medical, pharmaceutical and dental practitioners in French hospitals: Where have we been and where are we now?

INTRODUCTION: Women are under-represented in senior academic and hospital positions in many countries. The authors aim to assess the place and the evolution of all appointed female and male health practitioners' working in French public Hospitals. MATERIALS AND METHODS: Data of this observational study were collected from the National Management Centre (Centre National de Gestion) from 2015 up to January 1, 2020. First, the authors described demographic characteristics and specialties of all appointed medicine, pharmacy, and dentistry doctors' working as Hospital Practitioners, Associate Professors, and Full Professors in French General and University-affiliated Hospitals in 2020. Then, they retrospectively reported the annual incidence of new entrance according to gender and professional status from 1999 to 2019 thanks to the appointment date of all practitioners in activity between 2015 and 2020. RESULTS: In 2020, 51 401 appointed practitioners (49.7% of female) were in activity in French public hospitals with a large majority being medical doctors (92.4%) compared to pharmacists (6%) and dentists (1.6%). Women represented 52.5% of the Hospital Practitioners, 48.6% of the Associate Professors, and 22.0% of the Full Professors (p < 0.001). There were disparities between the rates of female Full Professors in medicine (20.6%), pharmacy (36.1%), and dentistry (44.3%, p < 0.001). Women were appointed Hospital Practitioners and Associate Professors earlier than men (respectively 37.1 versus 38.8 years, p < 0.001 and 36.1 versus 36.5 years, p = 0.04), and at a later age among Full Professors (43.7 versus 41.9 years, p < 0.001). Compared to men, the annual proportion of appointed women varied significantly between 1999 and 2019 from 47.6% to 60.4% for Hospital Practitioners, from 50.0% to 44.6% for Associate Professors, and from 11.2% to 33.3% for Full Professors (p < 0.001 for trend). CONCLUSIONS: Although more and more women occupy positions in French hospitals, there is still a gender gap regarding access to Full Professor status in medicine and pharmacy, but not in dentistry. The disparity in numbers makes comparison difficult. Despite a trend towards gender equality during the last twenty years, it has not yet been achieved regarding access to the highest positions.

Vasculitis associated with myelodysplastic syndrome and chronic myelomonocytic leukemia: French multicenter case-control study.

INTRODUCTION: Our objective was to evaluate characteristics, treatment and outcome of vasculitis associated with myelodysplastic syndrome (MDS) and chronic myelomonicytic leukemia (CMML) PATIENTS AND METHODS: Retrospective descriptive analysis of MDS/CMML-related vasculitis and comparison with MDS/CMML patients without dysimmune features. RESULTS: Seventy patients with vasculitis and MDS/CMML were included, with median age of 71.5 [21-90] years and male/female ratio of 2.3. Vasculitis was diagnosed prior to MDS/CMML in 31 patients (44%), and after in 20 patients. In comparison with MDS/CMML without autoimmune/inflammatory features, vasculitis with MDS/MPN showed no difference in MDS/CMML subtypes distribution nor International Prognostic Scoring System and CMML-specific prognostic (IPSS/CPSS) scores. Vasculitis subtypes included Giant cell arteritis in 24 patients (34%), Behçet's-like syndrome in 11 patients (20%) and polyarteritis nodosa in 6 patients (9%). Glucocorticoids (GCs) were used as first-line therapy for MDS/CMML vasculitis in 64/70 patients (91%) and 41 (59%) received combined immunosuppressive therapies during the follow-up. After a median follow-up of 33.2 months [1-162], 31 patients (44%) achieved sustained remission. At least one relapse occurred in 43 patients (61%). Relapse rates were higher in patients treated with conventional Disease Modifying Anti-Rheumatic Drug (DMARDs) (odds ratio 4.86 [95% CI 1.38 - 17.10]), but did not differ for biologics (odds ratio 0.59 [95% CI 0.11-3.20]) and azacytidine (odds ratio 1.44 [95% CI 0.21-9.76]) than under glucocorticoids. Overall survival in MDS/CMML vasculitis was not significantly different from MDS/CMML patients without autoimmune/inflammatory features (p = 0.5), but acute leukemia progression rates were decreased (log rank <0.05). CONCLUSION: This study shows no correlation of vasculitis diagnoses with subtypes and severity of MDS/CMML, and no significant impact of vasculitis on overall survival. Whereas conventional DMARDs seem to be less effective, biologics or azacytidine therapy could be considered for even low-risk MDS/CMML vasculitis.

Intoxication with 3-MeO-PCP alone: A case report and literature review.

RATIONALE: 3-Methoxyphencyclidine (3-MeO-PCP) is a new psychoactive substance derived from phencyclidine. Although it can lead to severe intoxications, the main manifestations and optimal management have not been well characterized. Here, we report 2 cases of 3-MeO-PCP intoxication in the same patient, and summarize the manifestations of this intoxication reported in literature. PATIENT CONCERNS: A 17-year-old male purchased a bag of 3-MeO-PCP on the Internet but took an oral dose (200 mg) that corresponds to the less active isomer 4-MeO-PCP. He developed high blood pressure (158/131 mm Hg), tachycardia (100 bpm), and neurological manifestations (confusion, hypertonia, nystagmus, and then agitation). A maculopapular rash appeared, although this may have been related to the administration of midazolam. Hyperlactatemia (2.6 mmol/L) was the main laboratory finding. Seven days later, he returned to the emergency department after sniffing 50 mg of 3-MeO-PCP. High blood pressure, tachycardia, and neurological manifestations (psychomotor impairment and dysarthria) were present but less severe than after the first intoxication. DIAGNOSIS: In the first intoxication, the blood and urine 3-MeO-PCP concentrations were, respectively, 71.1 ng/mL and 706.9 ng/mL. Conventional toxicity tests were all negative. In the second intoxication, biological samples were not available. INTERVENTIONS: In the first intoxication, treatment consisted of intravenous hydration and midazolam. The patient was transferred to an intensive care unit for monitoring. After the second intoxication, he was monitored for 12 hours. OUTCOMES: The patient's condition improved quickly in both cases. LESSONS: These cases provide additional information on the manifestations of 3-MeO-PCP intoxication. These manifestations are mainly cardiovascular (high blood pressure, tachycardia) and neurological. The fact that second (50 mg) intoxication was less severe than the first (200 mg) is suggestive of a dose-effect relationship for 3-MeO-PCP. The first case also emphasizes the risk of dosing errors caused by the similarity between the names "3-MeO-PCP" and "4-MeO-PCP."