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A subset of research in the history of economics is organizational history - i.e. the study of the organizations producing, circulating and applying economic ideas. This article maintains that some research questions in organizational history call for quantitative methods because they ask about magnitudes. More precisely, we claim that quantitative methods should complement rather than replace other research methods when the research question is at least partly about magnitudes. We walk the walk with a study of one type of organization, central banks, and of its changing relationship with economic science. Our results point unambiguously toward a growing dominance of central banks in the specialized field of monetary economics. Central banks have swelling research armies, they publish a growing share of the articles in specialized scholarly journals, and these articles tend to have more impact today than the articles produced outside central banks. [ABSTRACT FROM AUTHOR]

Objective Relapsing polychondritis (RP) is a rare condition characterized by recurrent inflammation of cartilaginous tissue and systemic manifestations. Data on this disease remain scarce. This study was undertaken to describe patient characteristics and disease evolution, identify prognostic factors, and define different clinical phenotypes of RP. Methods We performed a retrospective study of 142 patients with RP who were seen between 2000 and 2012 in a single center. Results Of the 142 patients, 86 (61%) were women. The mean ± SD age at first symptoms was 43.5 ± 15 years. Patients had the following chondritis types: auricular (89%; n = 127), nasal (63%; n = 89), laryngeal (43%; n = 61), tracheobronchial (22%; n = 32), and/orcostochondritis (40%; n = 57). The main other manifestations were articular (69%; n = 98), ophthalmologic (56%; n = 80), audiovestibular (34%; n = 48), cardiac (27%; n = 38), and cutaneous (28%; n = 40). At a mean ± SD followup of 13 ± 9 years, the 5- and 10-year survival rates were 95 ± 2% and 91 ± 3%, respectively. Factors associated with death on multivariable analysis were male sex ( P = 0.01), cardiac abnormalities ( P = 0.03), and concomitant myelodysplastic syndrome (MDS) ( P = 0.004) or another hematologic malignancy ( P = 0.01). Cluster analysis revealed that separating patients into 3 groups was clinically relevant, thereby separating patients with associated MDS, those with tracheobronchial involvement, and those without the 2 features in terms of clinical characteristics, therapeutic management, and prognosis. Conclusion This large series of patients with definite RP revealed an improvement in survival as compared with previous studies. Factors associated with death were male sex, cardiac involvement, and concomitant hematologic malignancy. We identified 3 distinct phenotypes. [ABSTRACT FROM AUTHOR]

ABSTRACT Background Methods Results Conclusions Trial Registration Hypereosinophilic syndromes (HES) are a heterogenous group of eosinophilic disorders. To date, only retrospective studies of limited sample‐size and/or follow‐up duration are available.The COHESion study is a national prospective multicenter multidisciplinary cohort recruiting both adults or children with the spectrum of eosinophilic disorders (including reactive HE/HES [HE/HES‐R], idiopathic HES [HES‐I], lymphocytic HES [HES‐L], neoplastic HE/HES [HE/HES‐N], HE of unknown significance [HE‐US], as well as IgG4‐related disease [IgG4RD] or ANCA‐negative eosinophilic granulomatosis with polyangiitis [EGPA] overlaps). Patients are followed‐up yearly. All data about final diagnosis, organ involvement assessments, and outcome profiles in HES‐I were captured and analyzed centrally by HES expert centers.From May 2019 to November 2023, 779 patients were included. For this preliminary analysis, 550 cases were available for centralized review (mean ± SD age: 56 ± 18 years, 42% of female patients). The final diagnoses were HES‐I (47%), HE/HES‐R (16%), HE‐US (15%), HE/HES‐N (7%), HE/HES‐L (6%), IgG4RD (2%), and ANCA‐negative EGPA (7%). In the 258 HES‐I patients, outcome profiles were classified as follows: 16.3% had a “single‐flare” without further relapse, 28.3% had a “relapsing–remitting” disease when there was at least a 6‐month period free of symptoms between two flares, 46.1% had a “persistent disease” requiring continuous treatment to avoid relapses (9.3% remained unclassified because of insufficient follow‐up).The COHESion cohort is the first nationwide prospective multicenter study collecting data on the full spectrum of HE/HES disorders. This preliminary analysis confirms that idiopathic HES patients have various outcome profiles, suggesting different underlying pathophysiological mechanisms and the need of patient‐specific management.ClinicalTrials.gov identifier: NCT 04018118 [ABSTRACT FROM AUTHOR]

The article presents a case study of a 30-year-old woman presented with a 3-month history of cough, haemoptysis, sinusitis, and fever during pregnancy. It notes the splinter haemorrhages of the nails, bilateral excavated lung nodules, and splenic infarcts in examination and diagnosis of Granulomatosis with polyangiitis; and also mentions treatment with a combination of intravenous methylprednisolone followed by prednisone and rituximab.

A response from the author of the article related to myelodysplastic syndrome (MDS) associated with relapsing polychondritis (RP) that was published in the previous issue of the periodical is presented.

Background: Cerebral vasculitis caused by neurosyphilis is a re-emerging problem with diagnostic and treatment issues, especially for human immunodeficiency virus patients.Methods: We present a case of relapsing syphilis-associated cerebral vasculitis, despite the recommended first-line antibiotic treatment, that was successfully treated with a second intravenous penicillin G course and corticosteroids.Results: A 50-year old man went to the emergency department for bilateral episodes of red and painful eyes with progressive but severe visual acuity loss. He was diagnosed with bilateral panuveitis and neurosyphilis favored by an unknown human immunodeficiency virus infection with a CD4 count of 236 mm3. Despite appropriate and well-conducted treatment including intravenous penicillin G, short-term corticosteroid, and highly active antiretroviral therapy, a symptomatic relapse of the syphilis-associated cerebral vasculitis occurred. After a second course of penicillin and corticosteroids, he made a complete recovery.Conclusions: Neurosyphilis and human immunodeficiency virus co-infection is a reappearing challenging situation that should be considered with care by physicians because recommended antibiotic treatment sometimes fails. Corticosteroid therapy should be discussed in case of cerebral vasculitis. [ABSTRACT FROM AUTHOR]

Objective The aim of this study was to describe the efficacy and safety of rituximab and MTX (RTX/MTX) combination therapy in ANCA-associated vasculitides (AAV). Methods A retrospective French nationwide study was conducted in patients with AAV who received RTX/MTX combination therapy for persistently active disease. Results Seventeen patients were included. All patients had granulomatosis with polyangiitis (GPA), with positive ANCA in 76% of them, mainly with PR3-ANCA specificity. Sixteen patients (94%) had priorly failed to achieve remission with RTX and 11 (65%) with CYC. Patients had experienced a median of 3 (2–4) flares. Manifestations requiring RTX/MTX combination therapy were subglottic or bronchial stenosis in 6 patients (35%), orbital mass in 6 (35%), disabling ENT involvement in 2 (12%), and epiduritis and pachymeningitis in 1 case (6%) each. The median follow-up duration for the RTX/MTX combination therapy was 11 months (11–26 months). At 6 months, global response had been achieved in 15 patients (88%), including partial response in 11 (65%) and complete response in 4 (24%). At last evaluation, global response had been achieved in 16 patients (94%). Seven patients (41%) experienced severe adverse events (grade 3 or 4), including infections in 4 (24%) and hepatitis in 2 (12%). Combination therapy was withdrawn in 4 patients (24%), but never for safety concerns. In contrast, the MTX dose was decreased in 2 patients (12%) because of adverse events. One patient died of an unknown cause. Conclusion RTX/MTX combination therapy could be an effective salvage therapy to treat persistently active GPA with granulomatous manifestations, with an acceptable safety profile. [ABSTRACT FROM AUTHOR]

Objective: to describe the prevalences, characteristics, and survivals of patients with anti-topoisomerase 1 antibodies (ATA) and limited cutaneous systemic sclerosis (lSSc) and anti-centromere antibodies (ACA) and diffuse cutaneous systemic sclerosis (dSSc). Methods: patients with ATA lSSc or with ACA dSSc were included in a case-control retrospective study. Results: In our cohort of scleroderma, the prevalence of ACA dSSc and ATA lSSc was 1.1% (12/1040) and 8.9% (93/1040), respectively. ACA dSSc patients had less interstitial lung disease (ILD) (5 (41.7) vs. 74 (79.6); p < 0.01), more cardiac involvement, and more muscle involvement (3 (25) vs. 4 (4.3); p = 0.03 and 4 (33.3) vs. 4 (7.5); p = 0.02,) than ATA dSSc patients. ATA lSSc patients had a higher modified Rodnan skin score than ACA lSSc patients (4 [2–7.5] vs. 2 [0–5]; p < 0.01) and less cardiac or muscle involvement than ATA dSSc patients (6 (6.5) vs. 19 (20.4%); p < 0.01 and 15 (16.1) vs. 54 (58.1); p < 0.0001, respectively). The cumulative 5-year survival rate was 71% in ACA dSSc patients, 95% in ATA lSSc patients, 84% in ACA lSSc patients, and 66% in ATA dSSc patients (p < 0.0001). Conclusion: ATA lSSc and ACA dSSc have specific characteristics when compared to ATA dSSc or ACA lSSc. ATA lSSc patients have more ILD than ACA lSSc patients, and ATA dSSc patients have the worst prognosis. Overall, inverted phenotypes show the value of a patient assessment combining antibody and skin subset and should be considered as a separate group. [ABSTRACT FROM AUTHOR]

Background: Subglottic stenosis (SGS) and tracheal stenosis (TS) are characterized by a narrowing of the airways. The goal of this study was to describe the characteristics and prognosis of nontraumatic and nontumoral SGS or TS.Research Question: What are the inflammatory etiologies of SGS and TS, and what are their characteristics and prognosis?Study Design and Methods: This multicenter, observational retrospective study was performed in patients with SGS or TS that was neither traumatic nor tumoral.Results: Eighty-one patients were included, 33 (41%) with granulomatosis with polyangiitis (GPA) and 21 (26%) with relapsing polychondritis (RP). GPA-related stenoses exhibited circumferential subglottic narrowing in 85% of cases, without calcifications. In contrast, RP-related stenoses displayed anterior involvement in 76%, in a longer distance from vocal cords (4 cm), with calcifications in 62%, and extension to bronchi in 86%. Other diagnoses included bullous dermatoses (n = 3), amyloidosis (n = 3), sarcoidosis (n = 2), and Crohn's disease (n = 2); the remaining stenoses (n = 15) were idiopathic. SGS/TS was the initial manifestation of the disease in 66% of cases, with a median interval from stenosis to disease diagnosis of 12 months (interquartile range, 0-48 months). Despite the use of glucocorticoids in 80%, combined with methotrexate in 49%, endoscopic procedures were required in 68% of patients. Relapses of stenoses occurred in 76% without any difference between causes (82% in GPA, 67% in RP, and 75% in idiopathic SGS/TS). Three patients died due to the stenosis, two of RP and one of GPA.Interpretation: These data show that GPA and RP are the two main inflammatory diseases presenting with SGS/TS. GPA-related stenoses are mostly subglottic and circumferential, whereas RP-related stenoses are mostly tracheal, anterior, and calcified with a frequent extension to bronchi. Relapses of stenoses are common, and relapse rates do not differ between causes. Diagnosis and management of SGS/TS require a multidisciplinary approach. [ABSTRACT FROM AUTHOR]

Objectives To characterize lymphocytes dysregulation in patients with granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). Methods Using flow cytometry, we analysed B- and T-cell subsets in peripheral blood from 37 untreated patients with active disease (29 GPA and 8 MPA) and 22 healthy controls (HCs). Results GPA patients had increased Th2 (1.8 vs 1.0%, P  = 0.02), Th9 (1.1 vs 0.2%, P  = 0.0007) and Th17 (1.4 vs 0.9%, P  = 0.03) cells compared with HC. Patients with MPO-ANCAs had significantly more CD21– B cells than HC or PR3-ANCA patients (6.9 vs 3.3% and 4.4%, P  = 0.01). CD69 expressing B cells were significantly higher in GPA and MPA (3.0 and 5.9 vs 1.4%, P  = 0.02 and P  = 0.03, respectively) compared with HC, whereas B-cell activating factor-receptor expression was decreased in GPA and MPA (median fluorescence intensity ratio 11.8 and 13.7 vs 45.1 in HC, P  < 0.0001 and P  = 0.003, respectively). Finally, IL-6-producing B cells were increased in GPA vs HC (25.8 vs 14.9%, P  < 0.0001) and decreased in MPA vs HC (4.6 vs 14.9%, P  = 0.005), whereas TNF-α-producing B cells were lower in both GPA and MPA patients compared with controls (15 and 8.4 vs 30%, P  = 0.01 and P  = 0.006, respectively). Conclusion Skewed T-cell polarization towards Th2, Th9 and Th17 responses characterizes GPA, whereas B-cell populations are dysregulated in both GPA and MPA with an activated phenotype and a decreased B-cell activating factor-receptor expression. Finally, inflammatory B cells producing IL-6 are dramatically increased in GPA, providing an additional mechanism by which rituximab could be effective. [ABSTRACT FROM AUTHOR]

Recent studies show that relapsing polychondritis patients with tracheobronchial involvement are distinct from others in terms of clinical characteristics, therapeutic management, and disease evolution. Tracheobronchial involvement affects 20 to 50% of patients and may reveal the disease. It should be sought at the time of diagnosis and at each follow-up visit. Respiratory impairment is confirmed by computed tomography (CT) of the chest, including the cervical portion of the trachea, with end-inspiratory and dynamic expiratory scans, and pulmonary function tests. These investigations should be performed, even in asymptomatic patients, at the time of diagnosis, and repeated as necessary during follow-up. Bronchoscopy and a fortiori endoscopic intervention should be considered with caution and performed only by expert endoscopists after careful evaluation of the risks and benefits of such procedures, which can lead to damage or perforation of the airways and bronchospasm. Early detection and management of tracheobronchial involvement in relapsing polychondritis has significantly improved the prognosis of patients, especially with the development of interventional fiberoptic bronchoscopy. However, relapsing polychondritis-related morbidity and mortality are still elevated, particularly in tracheobronchial disease. • RP with tracheobronchial involvement is distinct from others in terms of clinical characteristics, therapeutic management, and disease evolution. • Pulmonary function tests should be routinely performed at diagnosis and during follow up on a yearly basis. • A chest computed tomography scan should include the cervical portion of the trachea and dynamic expiratory scans. • It should be performed, even in asymptomatic patients, at the time of diagnosis and repeated as necessary during the evolution of the disease. • Overall survival has improved considerably, partially due to the availability of interventional treatments, but related morbidity and mortality are still elevated. • Therapeutic management is not based on trial data, but on expert recommendations. • It involves a close collaboration between pulmonologists and rheumatologists or internists. [ABSTRACT FROM AUTHOR]