Long-term Remission of Severe and Refractory Chronic Actinic Dermatosis with Dupilumab: A Case Report with Review of the Literature.
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ABSTRACT:
SUBMITTER: Clelia Vanhaecke
PROVIDER: S-EPMC11358845 | biostudies-literature | 2024 Aug
REPOSITORIES: biostudies-literature
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Long-term Remission of Severe and Refractory Chronic Actinic Dermatosis with Dupilumab: A Case Report with Review of the Literature.
Clélia Vanhaecke Manuelle Viguier
Acta dermato-venereologica 20240823
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Project description:Background and objectivesClinical remission, defined as the absence of disease activity and symptoms, is an emerging goal in the management of chronic rhinosinusitis with nasal polyps (CRSwNP). This study aimed to evaluate the long-term effects of dupilumab on patients with CRSwNP, with or without asthma, and explore the potential for achieving clinical remission.MethodsA two-year prospective study was conducted on 109 patients with CRSwNP, with or without asthma, who were eligible for dupilumab as an add-on therapy. Comprehensive assessments, including clinical, laboratory, and radiological evaluations, were performed before and after treatment. Clinical remission of CRSwNP was defined as 12 months of dupilumab treatment, no exacerbations requiring oral corticosteroids (OCS), no need for nasal sinus operation, no anosmia or hyposmia, a Sino-Nasal Outcome Test (SNOT-22) score under 20, and a Lund-Mackay score (LMS) below 10. For those with comorbid asthma, clinical remission was defined as an asthma control test (ACT) score of 19 or higher, no asthma exacerbations, and no need for OCS.ResultsDupilumab significantly improved CRSwNP outcomes in both groups, including SNOT-22 scores, nasal polyp size (LMS), and anosmia/hyposmia. Comorbid asthma was highly prevalent (79.8%), and patients with asthma had significantly larger nasal polyps, both before and after dupilumab therapy, despite similar symptom improvement. Higher fractional exhaled nitric oxide (FeNO) and blood eosinophil count (BEC) levels, along with anosmia/hyposmia, predicted larger polyp size. Dupilumab also significantly improved asthma outcomes, increasing forced expiratory volume in 1 s (FEV1) and decreasing FeNO. Clinical remission was achieved in 11% of patients, with a slightly lower rate in those with asthma (7.3%).ConclusionDupilumab treatment can achieve clinical remission in CRSwNP. However, comorbid asthma appears to reduce the likelihood of remission and is associated with larger nasal polyps, even with similar symptom improvement. Asthma may independently influence polyp development, potentially impacting long-term outcomes in CRSwNP.
| S-EPMC11787525 | biostudies-literature
Project description:Bronchial asthma is a chronic inflammatory condition with increasing prevalence worldwide that may present as heterogeneous phenotypes defined by the T2-mediated pattern of airway inflammation T2-high and T2-low asthma. Severe refractory asthma includes a subset of asthmatic patients who fail to control their disease despite maximal therapy and represent a group of patients needing marked resource utilization and hence may be eligible to add-on biological therapies. Among the new biologics, we focused our attention on two monoclonal antibodies: dupilumab, exerting a dual blockade of cytokine (interleukin (IL)-4 and IL-13) signaling; and tezepelumab, acting at a higher level preventing the binding of thymic stromal lymphopoietin (TSLP) to its receptor, thus blocking TSLP, IL-25, and IL-33 signaling, hence modulating airway T2 immune responses. With their different mechanisms of action, these two biologics represent important options to provide an enhanced personalized treatment regimen. Several clinical trials have been conducted testing the efficacy and safety of dupilumab in severe refractory asthmatic patients showing improvements in lung function, asthma control, and reducing exacerbations. Similar results were reported with tezepelumab that, differently from dupilumab, acts irrespectively on eosinophilic or non-eosinophilic phenotype. In this review, we provide an overview of the most important highlights regarding dupilumab and tezepelumab characteristics and mechanism of action with a critical review of the principal results of clinical (Phase II and III) studies concluded and those still in progress.
| S-EPMC9152192 | biostudies-literature
Project description:The investigators will investigate the efficacy of dupilumab in patients with severe eosinophilic CRSsNP who are resistant to the conventional treatment with intranasal corticosteroids and have significantly extensive disease involving more than 2 sinuses bilaterally in sinus CT scan and Lund-Mackay sinus (LMK) CT score >=10 at baseline.
| 2342440 | ecrin-mdr-crc
Project description:BackgroundEosinophilic chronic rhinosinusitis (ECRS) is a subgroup of chronic rhinosinusitis with nasal polyps (CRSwNP), which is a disease characterized by eosinophilic infiltration of the sinonasal mucosa. Few studies that reported the effect of dupilumab on CRSwNP focused on a single phenotype of CRSwNP, such as ECRS.ObjectivesThis study aimed to determine the effectiveness of dupilumab in ECRS with postoperative recurrence.MethodsWe retrospectively enrolled 107 patients and assessed the effectiveness of dupilumab by various clinical outcomes. We performed multivariable analysis on nasal polyp score (NPS) and computed tomography score and a meta-analysis of the effect of dupilumab on chronic rhinosinusitis regarding improvement in the NPS.ResultsAt 12 months of dupilumab treatment, there were 65 patients (60.7%) in the excellent response group and 42 (39.3%) in the moderate response group. Nasal polyps had disappeared in 91 patients (85.9%) at 12 months, and there was improvement in all end points; 104 patients (97.2%) were able to eliminate systemic corticosteroid therapy. In the multivariate analysis, male sex was significantly associated with patients who did not show an improvement to 0 in the NPS and computed tomography score (odds ratios: 7.58 and 2.45; P = .01 and P = .04, respectively). The meta-analysis showed that dupilumab treatment resulted in a trend toward better improvement in the NPS (mean difference = -5.41) than previously reported results.ConclusionsDupilumab shows effectiveness in treating ECRS and could serve as an alternative therapeutic option to systemic corticosteroids. This effectiveness may be further enhanced by limiting the target population to recurrent ECRS.
| S-EPMC11850117 | biostudies-literature
Project description:BackgroundUlcerative colitis (UC)-related post-colectomy enteritis is a very rare condition that is characterized by diffuse small-bowel mucosal inflammation following colectomy and could be very dangerous. In previously reported cases, corticosteroid therapy seemed to be the optimal choice for inducing remission; however, the patient studied herein presented with severe diarrhoea and hypovolemic shock and failed to achieve full remission with corticosteroid therapy.Case presentationWe describe the case of a patient with severe pan-enteritis presenting with life-threatening diarrhoea complicated with hypovolemic shock and acute kidney injury after colectomy and ileal pouch anal anastomosis (IPAA) for UC; this patient was successfully treated by ileostomy closure after failing to achieve full remission with corticosteroid therapy. Next, we review other cases of post-colectomy enteritis reported in the literature and propose a flow-chart for its diagnosis and initial treatment.ConclusionPost-colectomy enteritis can be dangerous, and the early awareness of this condition plays a vital role. Additionally, in patients who do not respond well to corticosteroid or immunosuppressant therapy, early closure of the ileostomy and re-establishment of the natural faecal stream could be important considerations.
| S-EPMC6482549 | biostudies-literature
Project description:Ocular disease, such as scleritis and peripheral ulcerative keratitis (PUK), may be a serious ocular complication. We present a patient with severe and refractory PUK treated with baricitinib. A review of the literature on Janus kinase inhibitors (JAKINIB) in refractory ocular surface pathology was also performed. For the literature review, the search in PubMed, Embase, and the Cochrane library was carried out from inception until 31 May 2021, including conference proceedings from four major rheumatology congresses. All original research articles studying JAKINIB treatment in patients with inflammatory eye disease were included. We present an 85-year-old woman with rheumatoid arthritis (RA) and secondary Sjögren's syndrome refractory to methotrexate, leflunomide, certolizumab pegol, adalimumab, and tocilizumab (TCZ). However, 10 months after starting TCZ, the patient suffered a perforation secondary to PUK, requiring urgent surgical intervention. In the absence of infection, she was treated with boluses of intravenous methylprednisolone followed by oral prednisone at high doses in a decreasing pattern together with baricitinib at a dose of 2 mg/day with a very rapid and persistent favorable response to eye and joint symptoms. After 18 months of treatment, the patient had not presented serious side effects or signs of reactivation of her disease. In addition to this report, three other studies including one PUK associated with RA and two non-infectious scleritis treated with tofacitinib were included in this literature review. All three patients had experienced an insufficient response to conventional treatment, including biologic agents, before being switched to JAKINIB, leading to a complete or partial recovery in all of them without significant adverse effects so far. JAKINIBs (baricitinib and tofacitinib) may be an effective and safe therapy in patients with severe autoimmune and refractory ocular surface pathology, such as scleritis and PUK.
| S-EPMC9677317 | biostudies-literature
Project description: Not available
| S-EPMC4232891 | biostudies-literature
Project description:To understand the persistent inflammation even after 5 years of treatment of dapsone in Rosai-Dorfmann patient, we performed single cell RNA sequencing for persistent nodular lesions.
2022-01-29 | GSE194428 | GEO
Project description:Kyrle's disease is an uncommon form of acquired transepidermal elimination dermatosis frequently associated with diabetes mellitus and chronic kidney disease. An association with malignancy has been sporadically reported in the literature. Here, we describe the clinical course of a diabetic patient with end-stage renal disease who developed this disorder as a herald to a regionally advanced renal cell carcinoma. We provide a focused literature review and rationale for the definitive categorization of acquired perforating dermatosis as a potential paraneoplastic manifestation of systemic malignancies. Clinicopathological correlation and prompt communication among clinicians for occult malignancies are always warranted. Furthermore, we describe a novel association of one of the subtypes of acquired perforating dermatosis with such malignancies.
| S-EPMC10294214 | biostudies-literature
Project description:IntroductionThe prognosis of anaplastic thyroid cancer (ATC) is poor with a mean survival time of six months following diagnosis. Despite various attempts to modify common treatment modalities including surgery, external beam radiation and chemotherapy, an effective treatment is not available yet. We report, here, a patient who achieved long-term survival based on multimodal treatment, including in vitro evaluation of drug response of his tumor cells.Presentation of caseA 42 years old male patient underwent total thyroidectomy with central and lateral neck dissection for ATC (pT4b, pN0 (0/36), L0, V0, Pn1, R0 cM0 - UICC-Stage: IV b). From the tumor tissue a primary cell culture was established. While the patient received a combined radio-chemotherapy cell viability assays were performed using Sorafenib, Vandetanib und MLN8054 (Aurora kinase inhibitor) as inhibitors. Cell viability was determined by MTT-assay after 72 and 144h of treatment.DiscussionAll the three compounds affected cell viability in a time- and dose dependent manner. These effects were most pronounced by Sorafenib. Based on in vitro findings, the patient was treated daily with 400mg Sorafenib for 75days. 43 months after initial diagnosis, the patient had no evidence of disease as shown by MRI, CT and FDG-PET-CT imaging.ConclusionIn the setting of multimodal treatment, in vitro drug evaluation of individual tumor cells of patients might be a promising tool to ameliorate the fatal prognosis of selected ATC patients.
| S-EPMC4933033 | biostudies-literature