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Polychlorinated dibenzo-p-dioxins and -furans (PCDD/Fs) are contaminants of concern in the New York/New Jersey Harbor and in the organisms of the Newtown Creek Superfund site, which lies within the harbor. Because PCDD/Fs are never intentionally produced, identifying their sources can be challenging. In this work, sources of PCDD/Fs to the sediment of Newtown Creek were investigated using Positive Matrix Factorization (PMF) to analyze two data sets containing data on concentrations of (1) PCDD/Fs and (2) PCDD/Fs plus polychlorinated biphenyls (PCBs). The PCDD/F data set generated eight factors, but they were not particularly helpful in identifying PCDD/F sources. The combined PCDD/F plus PCB data set generated eleven factors, many of which represented Aroclors. Based on its spatial distribution, the primary source of PCDD/F-related Toxic Equivalency Quotient (TEQ) in the sediment (accounting for 53 % of total TEQ) may be related to a facility that performed smelting and refining of metals (primarily copper). Aroclors appear to be responsible for about 20 % of the total TEQ. This analysis revealed two additional secondary sources of PCDD/Fs to Newtown Creek sediment: the East River (3 % of TEQ) and Combined Sewer Outfalls (CSOs; 0.5 % of TEQ). The East River was responsible for most of the mass of 2,3,7,8-tetrachloro dibenzo-p-dioxin (TCDD) in the sediment, presumably because it transports TCDD-laden sediment from the Passaic River into Newtown Creek. CSOs were proportionately more important in surface sediments. Adding PCBs to the data matrix appears to increase ability of the PMF analysis to identify both primary (Aroclors) and secondary (CSOs, East River) PCDD/F sources, but it is unclear whether it may overstate the fraction of PCDD/Fs arising from Aroclors., Competing Interests: Declaration of competing interest The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: Mahdi Chitsaz reports financial support was provided by Hudson River Foundation. Mohson Al Hello reports financial support was provided by Republic of Iraq Ministry of Higher Education and Scientific Research. Lisa A. Rodenburg reports a relationship with various cities and states suing Monsanto that includes: paid expert testimony. If there are other authors, they declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 The Authors. Published by Elsevier B.V. All rights reserved.)

Polychlorinated biphenyls (PCBs) are a primary contaminant of potential concern at the Newtown Creek superfund site. Measurements of PCBs in hundreds of samples of sediment (surface and cores) within Newtown Creek and at nearby reference locations were obtained from the Remedial Investigation (RI) databases. This data set was analyzed using Positive Matrix Factorization (PMF). A weight-of-evidence approach was used to attribute the PMF-generated fingerprints to sources. The PMF analysis generated eight factors (fingerprints or sources) that represent primary sources, such as Aroclors, as well as secondary sources, including the East River and Combined Sewer Outfalls (CSOs). In addition to the high-production volume Aroclors (1016/1242, 1248, 1254, and 1260), some less-widely used Aroclors (1232 and 1268) were found in Newtown Creek sediment. Aroclor 1268 is disproportionately abundant in the deepest sediments, while PCBs likely from CSOs are relatively more abundant in surface sediment., Competing Interests: Declaration of competing interest The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: LAR and DRB are paid consultants for Chevron, Inc., a potentially responsible party in the Newtown Creek Superfund Site. LAR provides expert testimony to cities and states that are suing Monsanto over issues related to its manufacture of PCBs., (Copyright © 2024 The Author(s). Published by Elsevier Ltd.. All rights reserved.)

Objectives: VEXAS is a recently described acquired auto-inflammatory and hematologic syndrome caused by somatic mutations in UBA1. To date, VEXAS is not a recognized cause of acquired immunodeficiency., Patients and Methods: Two of our 10 VEXAS patients developed a disseminated Mycobacterium avium infection. To shed light on this observation, we retrospectively studied all patients with disseminated non-tuberculous mycobacterial infections (NTMi) seen at our institution over 13 years. Inclusion criteria were a positive blood/bone marrow culture, or 2 positive cultures from distinct sites, or one positive culture with 2 involved sites., Results: patient 1 presented with fever, rash, orbital cellulitis and lung infiltrates. Patient 2 presented with fever and purpura. In both cases, Mycobacterium avium was identified on bone marrow culture. Twenty cases of disseminated NTMi were reviewed. Among 11 HIV-negative patients, three had chronic immune-mediated disease; three had untreated myeloid neoplasm; two had VEXAS; one had undergone kidney transplantation; one had GATA-2 deficiency; and one had no identified aetiology. None had lymphoid neoplasia or had undergone bone marrow transplantation. HIV-negative cases had higher CD4 counts than HIV-positive patients (median CD4: 515/mm3  vs 38/mm3, p< 0.001). Monocytopenia was present in seven cases. At 2 years, six patients had died, including both VEXAS patients., Discussion: VEXAS patients have an intrinsic susceptibility to disseminated NTMi, which may result from monocytic dysfunction. NTMi can mimic VEXAS flare. Clinicians should maintain a high suspicion for opportunistic infections before escalating immunosuppressive therapy. Further studies are needed to confirm and better decipher the herein reported observations., (© The Author(s) 2024. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Polychlorinated biphenyls (PCBs) are persistent, bioaccumulative, and toxic chemicals that are the dominant contaminant in the Upper Hudson River (UHR) in New York State where two General Electric (GE) plants historically discharged PCBs to the river. Portions of the UHR were dredged from 2009 to 2015 to address PCB contamination. In 2017, the first post-dredging survey of yearling feeder fish and sediment PCB contamination was conducted to establish a baseline for the recovery of the river. Prior analysis of the sediment data from the 2017 survey indicated that ∼2% of the PCBs in the surface sediment were higher in molecular weight than the formulation used by GE and therefore arose from non-GE sources. In this work, the fish PCB data from the 2017 survey were analyzed using Positive Matrix Factorization (PMF). Empirical Bayesian Kriging (EBK) was used to estimate PCB concentrations in the sediment at the locations where fish were collected. The results suggest that PCBs that are the products of microbial dechlorination bioaccumulate in the fish and represent 7% of the PCB mass in the fish data set. Further, the results suggest that about 13% of the PCBs in the fish may have come from non-GE sources. This is higher than the percentage of non-GE PCBs in the sediment, but can be explained by the higher molecular weight of the non-GE mixture which causes it to bioaccumulate more effectively than GE PCBs. Concentrations of the non-GE PCBs averaged about 240 ppb wet weight (whole body) in yearling feeder fish. The remedial goals range from 50 to 400 ppb ww in fillet for fish including piscivorous species that are likely to have higher PCB concentrations than feeder fish., Competing Interests: Declaration of competing interest The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: Lisa A. Rodenburg reports a relationship with Various law firms that includes: paid expert testimony., (Copyright © 2022 Elsevier Ltd. All rights reserved.)

Atopic dermatitis (AD) of the adult is a common skin disease. Its prevalence has greatly increased during the past decades. AD is commonly associated with other atopic disorders. Its impact on quality of life is often underestimated. Various immunopathologic mechanisms are involved in AD: innate epidermal barrier dysfunction due to filaggrin gene mutations, innate and adaptative abnormalities of the immune system (an initial Th2 phase precedes a chronic Th1 phase), intestinal and cutaneous microbiomes dysbiosis, and environmental factors. Diagnosis of AD is clinical and there is no predictive biomarker of future severity. The main differential diagnoses are: scabies, psoriasis, cutaneous adverse reaction, cutaneous T cell lymphoma, primary immunodeficiency, and Netherton's syndrome. Therapeutic management is challenging and should integrate a therapeutic education program. Topical corticosteroids are the first line treatment, including a preliminary assessment of possible topical corticosteroids phobia. Systemic treatments are recommended in severe, chronic and resistant AD, after careful evaluation in a reference centre. Dupilumab, an IL4/IL13 inhibitor, might be the first effective targeted therapy in AD, whereas therapies that specifically target the mechanisms of pruritus represent an exciting perspective., (Copyright © 2015 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.)

Whatever their aetiology, monoclonal gammopathies can be associated to several clinical features. Mechanisms are various and sometimes unknown. Skin is frequently involved and may represent a challenging diagnosis. Indeed, skin manifestations are either the presenting features and isolated, or at the background of a systemic syndrome. Our objective was to review the various skin manifestations that have been associated with monoclonal gammopathies., (Copyright © 2013. Published by Elsevier SAS.)

Idiopathic granulomatous mastitis (IGM) is a rare localized granulomatosis of unknown aetiology that usually affects women of childbearing age. It often mimics breast carcinoma or abscess. Histopathologic evaluation and elimination of the others aetiologies of granuloma play a crucial role in the diagnosis. Its etiopathogeny remains poorly understood, but Corynebacteria might be involved. The disease course is usually protracted, with a significant impact on quality of life. The management of IGM remains controversial, but corticosteroids are usually the first-line treatment., (Copyright © 2012. Published by Elsevier SAS.)

Aim: To investigate the presentation, disease course and long-term outcome of a western cohort of idiopathic granulomatous mastitis (IGM) and to analyse the impact of different therapeutic strategies., Methods: Multicentre retrospective study of 23 women followed over an extended period. Patients were recruited in nine French internal medicine departments., Results: The median follow-up was 6 years. IGM presented commonly as a single inflammatory unilateral extra-areolar lump of varying size. Clinical course was heterogeneous and frequently remitting/relapsing. Most patients had at least one recurrence (18/23, 78%). The mean number of recurrences was 1.3 ± 1.5. Seven women had a bilateral evolution. Twelve women received steroids (corticosteroids). Only two of these did not respond to corticosteroids, whereas six relapsed when dose was tapered off. Nine patients received colchicine and/or hydroxychloroquine. First-line treatment consisted of excisional surgery in eight cases. At the date of last interview, 91% of the patients declared to be healed, 15 being free of treatment. However, 12/21 (57%) reported significant sequelae (unsightly scars: eight and/or lasting pain: six). Unsightly scars were not more prevalent in patients who had received steroids whereas they tended to be more frequent after breast excisional surgery. In addition, we found that excisional surgery did not prevent recurrences more successfully than a conservative approach., Conclusions: Despite its retrospective nature, this Caucasian series provides novel information regarding long-term outcomes in IGM and argues in favour of conservative approaches. The value of immunomodulatory drugs such as colchicine or hydroxychloroquine deserves further investigation.

The reversible cerebral vasoconstriction syndrome (RCVS) is an under-estimated transient acute cerebrovascular disorder. It has long been mistaken as central nervous system vasculitis whereas it is now believed to result from an acute but prolonged vasospasm of cerebral arteries. This disorder can be precipitated by postpartum or vasoactive drug. However, it occurs spontaneously in a significant number of cases. The characteristic clinico-radiological presentation and disease course of the RCVS has been delineated only recently. Mean age at onset is 40-45 years, with a female predominance. A provocative factor can be identified in 12-60% out of the patients. Clinical presentation is predominantly marked by recurrent thunderclap headaches, but can be complicated with focal neurological deficit or seizures. Brain imaging is normal in most cases, but can reveal hemorrhagic or ischemic complications. Cortical subarachnoid hemorrhage is a suggestive finding. A posterior reversible encephalopathy syndrome (PRES) can be seen occasionally. Cerebral angiography reveals multifocal arterial narrowing with string and bead appearance. Cerebrospinal fluid reveals no or mild abnormalities. The disease resumes spontaneously within several days to weeks, whereas vasoconstriction reverses within 1 to 3 months. This clinico-radiological presentation should be promptly recognized in order to avoid unnecessary investigations and aggressive treatment, and lead to search for a triggering factor. Further studies are required in order to clarify the precipitating role of several drugs, and clinical trials are needed to reduce the occurrence of strokes., (Copyright © 2012 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.)