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Biologics : Targets & Therapy 2024The patient was a 50-year-old Japanese woman who was diagnosed with total-colitis-type ulcerative colitis (UC) at the age of 26 years. She was treated with mesalazine...
The patient was a 50-year-old Japanese woman who was diagnosed with total-colitis-type ulcerative colitis (UC) at the age of 26 years. She was treated with mesalazine and azathioprine, and her disease activity was well controlled. At the age of 50 years, the patient was experiencing fever, abdominal pain, diarrhea, bloody stool, and anal pain, which led to a diagnosis of a relapse of UC. Although steroid therapy was administered and tended to improve her symptoms, fecaloid vaginal discharge occurred, and rectovaginal fistula (RVF) was confirmed. Colostomy was performed, and infliximab was initiated as maintenance therapy for UC. All symptoms improved, and RVF closure was confirmed 6 months after the initiation of infliximab. To date, she has been free from relapse of UC. There have been only a few reports of UC complicated by RVF, and this condition is often difficult to treat. To the best of our knowledge, no other case of UC complicated by RVF in which the fistula was closed after treatment with colostomy and infliximab has been previously reported; thus, our report of the present case is valuable to the literature.
PubMed: 38736705
DOI: 10.2147/BTT.S457300 -
Transplantation Proceedings May 2024Deterioration of kidney function after orthotopic liver transplantation is a common complication that may occur after perioperative acute kidney injury (AKI) and...
Deterioration of kidney function after orthotopic liver transplantation is a common complication that may occur after perioperative acute kidney injury (AKI) and preexisting or developing chronic kidney disease (CKD). AKI is described in the early postoperative period in more than half of recipients, whereas the main cause of CKD is pharmacotherapy. When end-stage renal failure occurs, patients may be qualified for additional transplantations. We present a rare case of a 27-year-old woman who, as a teenager, underwent 2 liver transplantations due to Wilson's disease. Surgeries were complicated by systemic infection and multiple organ failure. The kidneys did not regain their function, and therefore, after 6 months of dialysis, the organ was transplanted. Three organ transplantations were performed. Due to the patient's willingness and good graft functions, the patient started trying to conceive. Three months before successful conception, immunosuppressive therapy was changed to tacrolimus and azathioprine. Pregnancy was complicated by pregnancy-induced hypertension, and its course was closely monitored. Organ functions and immunosuppressive therapy were regularly assessed. Due to the pre-eclampsia developed in the 35th week of gestation, a Cesarean delivery was performed, and she gave birth to a daughter weighing 2350 g (Apgar 7-7-8). The patient decided to breastfeed. There were no obstetric complications or graft function deterioration in the early postpartum period. Mother and daughter left home after 7 days of hospitalization. The presented clinical situation proves that multiorgan transplantation recipients can have a successful pregnancy without impairing graft functions. Therefore, the pregnancy requires adequate preparation and increased care.
Topics: Humans; Female; Adult; Pregnancy; Liver Transplantation; Kidney Transplantation; Immunosuppressive Agents; Pregnancy Complications; Hepatolenticular Degeneration; Acute Kidney Injury; Kidney Failure, Chronic
PubMed: 38729837
DOI: 10.1016/j.transproceed.2024.03.033 -
Italian Journal of Dermatology and... Jun 2024SIDeMaST (Società Italiana di Dermatologia Medica, Chirurgica, Estetica e delle Malattie Sessualmente Trasmesse) contributed to the development of the present guideline...
Italian S3-Guideline on the treatment of Atopic Eczema - Part 1: Systemic therapy, adapted from EuroGuiDerm by the Italian Society of Dermatology and STD (SIDEMAST), the Italian Association of Hospital Dermatologists (ADOI) and the Italian Society of Allergological and Environmental Dermatology...
SIDeMaST (Società Italiana di Dermatologia Medica, Chirurgica, Estetica e delle Malattie Sessualmente Trasmesse) contributed to the development of the present guideline on the systemic treatment of chronic plaque psoriasis. With the permission of EuroGuiDerm, SIDeMaST adapted the guideline to the Italian healthcare context to supply a reliable and affordable tool to Italian physicians who take care of patients affected by atopic dermatitis. The evidence- and consensus-based guideline on atopic eczema was developed in accordance with the EuroGuiDerm Guideline and Consensus Statement Development Manual. Four consensus conferences were held between December 2020 and July 2021. Twenty-nine experts (including clinicians and patient representatives) from 12 European countries participated. This first part of the guideline includes general information on its scope and purpose, the health questions covered, target users and a methods section. It also provides guidance on which patients should be treated with systemic therapies, as well as recommendations and detailed information on each systemic drug. The systemic treatment options discussed in the guideline comprise conventional immunosuppressive drugs (azathioprine, ciclosporin, glucocorticosteroids, methotrexate and mycophenolate mofetil), biologics (dupilumab, lebrikizumab, nemolizumab, omalizumab and tralokinumab) and janus kinase inhibitors (abrocitinib, baricitinib and upadacitinib). Part two of the guideline will address avoidance of provocation factors, dietary interventions, immunotherapy, complementary medicine, educational interventions, occupational and psychodermatological aspects, patient perspective and considerations for pediatric, adolescent, pregnant and breastfeeding patients.
Topics: Humans; Dermatitis, Atopic; Italy; Dermatologic Agents; Immunosuppressive Agents; Dermatology
PubMed: 38727633
DOI: 10.23736/S2784-8671.24.07664-3 -
Crohn's & Colitis 360 Apr 2024Since 2009, inflammatory bowel disease (IBD) specialists have utilized "IBD LIVE," a weekly live video conference with a global audience, to discuss the...
BACKGROUND
Since 2009, inflammatory bowel disease (IBD) specialists have utilized "IBD LIVE," a weekly live video conference with a global audience, to discuss the multidisciplinary management of their most challenging cases. While most cases presented were confirmed IBD, a substantial number were diseases that mimic IBD. We have categorized all IBD LIVE cases and identified "IBD-mimics" with consequent clinical management implications.
METHODS
Cases have been recorded/archived since May 2018; we reviewed all 371 cases from May 2018-February 2023. IBD-mimics were analyzed/categorized according to their diagnostic and therapeutic workup.
RESULTS
Confirmed IBD cases made up 82.5% (306/371; 193 Crohn's disease, 107 ulcerative colitis, and 6 IBD-unclassified). Sixty-five (17.5%) cases were found to be mimics, most commonly medication-induced ( = 8) or vasculitis ( = 7). The evaluations that ultimately resulted in correct diagnosis included additional endoscopic biopsies ( = 13, 21%), surgical exploration/pathology ( = 10, 16.5%), biopsies from outside the GI tract ( = 10, 16.5%), genetic/laboratory testing ( = 8, 13%), extensive review of patient history ( = 8, 13%), imaging ( = 5, 8%), balloon enteroscopy ( = 5, 8%), and capsule endoscopy ( = 2, 3%). Twenty-five patients (25/65, 38%) were treated with biologics for presumed IBD, 5 of whom subsequently experienced adverse events requiring discontinuation of the biologic. Many patients were prescribed steroids, azathioprine, mercaptopurine, or methotrexate, and 3 were trialed on tofacitinib.
CONCLUSIONS
The diverse presentation of IBD and IBD-mimics necessitates periodic consideration of the differential diagnosis, and reassessment of treatment in presumed IBD patients without appropriate clinical response. The substantial differences and often conflicting treatment approaches to IBD versus IBD-mimics directly impact the quality and cost of patient care.
PubMed: 38720935
DOI: 10.1093/crocol/otae022 -
Cureus Apr 2024Behçet's disease (BD) is a vascular disorder affecting a variety of organ systems. It is an auto-immune disease with inflammatory vasculitis that is systemic in nature,...
Behçet's disease (BD) is a vascular disorder affecting a variety of organ systems. It is an auto-immune disease with inflammatory vasculitis that is systemic in nature, the exact etiology of which is unknown. Obliterative vasculitis, recurrent aphthous ulcers, mucocutaneous manifestations, recurrent genital ulcerations, and intraocular inflammation, especially chronic relapsing uveitis, are the characteristic features of BD. The case report presents a unique manifestation of BD in a 20-year-old Pakistani male who presents with a one-year-old history of viral encephalitis, after which he developed a blurring of vision. On examination, he had recurrent aphthous ulcers, recurrent ulcerations of genitalia, and a history of lesions of the skin. After making the patient undergo a cascade of investigations for evaluating and assessing the various signs and symptoms, a diagnosis of BD with bilateral panuveitis and a full-thickness macular hole (FTMH) in the right eye was established. Immuno-suppressants, steroids, and azathioprine were used as treatment options, following which the state of remission was attained.
PubMed: 38707073
DOI: 10.7759/cureus.57599 -
Medicine May 2024Despite an increase in global research on the subject of Pemphigus, which seriously affects patient health and quality of life, there is no bibliometric research on this...
Despite an increase in global research on the subject of Pemphigus, which seriously affects patient health and quality of life, there is no bibliometric research on this subject in literature to date. The aim of this study was to conduct a holistic analysis of scientific articles published on Pemphigus, using bibliometric methods. Articles published on the subject of Pemphigus between 1980 and 2021 were downloaded from the web of science (WoS) database and analyzed using various statistical methods. To determine trend subjects, collaboration between countries, and the most effective studies with citation analyses, visual network maps were obtained with bibliometric analyses. A total of 3034 articles were analyzed. The 3 countries making the greatest contribution to literature were the USA (n:831, 27.3%), Japan (n:402, 13.2%), and Germany (n:221, 7.2%). The 3 most active institutions were Keio University (n:163, 5.3%), Kurume University (n:130, 4.2%) and Tel Aviv University (n:107, 3.5%). The 3 journals publishing the most articles were the British Journal of Dermatology (n: 88), Journal of the American Academy of Dermatology (n:171) and the Journal of Investigative Dermatology (n:143). The 3 leading journals according to the mean number of citations (NC) per article (citation count: CC) were the New England Journal of Medicine (CC:246), the Lancet (CC:143) and the Journal of Cell Biology (CC:133). The author with the most articles published was Hashimoto Takashi (n.168, 5.5%). As a result of cluster analysis, it was seen that 9 different main clusters had been studied on Pemphigus subjects to date (1: desmoglein, 2: paraneoplastic Pemphigus (PNP) - Pemphigus types-desmosome, 3: desmoglein 1 ve 3-autoimmunity, 4: treatment-rituximab, 5: acantholysis-apoptosis, 6: quality of life-remission-relapse, 7: autoantibodies, 8: epidemiology-mortality, 9: corticosteroids). The most commonly studied subjects were determined to be pemphigus vulgaris (PV), pemphigus foliaceus (PF), autoimmunity, rituximab, PNP, desmoglein (desmoglein3-desmoglein1), autoantibodies, acantholysis, autoantibody, treatment, autoimmune disease, desmosome, ELISA, and immunofluorescence. The primary trending topic was rituximab drug, which is used in the treatment of Pemphigus. The other most studied trend topics were azathioprine drug used in treatment, intravenous immunoglobulin treatment, quality of life, mortality rates, Pemphigus herpetiformis, and wound healing.
Topics: Bibliometrics; Pemphigus; Humans; Periodicals as Topic; Biomedical Research; Efficiency
PubMed: 38701303
DOI: 10.1097/MD.0000000000038047 -
Heliyon May 2024Primary Sjogren's syndrome (pSS) is an autoimmune exocrinopathy in which extraglandular signs of pSS are determinant for the prognosis. Involvement of both peripheral...
BACKGROUND
Primary Sjogren's syndrome (pSS) is an autoimmune exocrinopathy in which extraglandular signs of pSS are determinant for the prognosis. Involvement of both peripheral and central nervous system (CNS) are known to be among the sites of high systemic activity in pSS.
CASE PRESENTATION
We, herein, report a case of a 57-year-old female patient with pSS presenting with typical (GBS), shortly followed by acute headaches accompanied by cortical blindness. Cerebral magnetic resonance imaging (MRI) demonstrated T2 signal abnormalities on the occipital region with narrowing and irregularities of the cerebral arteries, suggestive of CNS vasculitis.Subtle sicca symptoms occurring prior to neurological symptoms by 8 months together with immunological disturbances (anti-SSA, anti-SSB antibodies positivity, type II cryoglobulins positivity, and C4 hypocomplementemia) allowed us to retain the diagnosis of pSS. Recovery of motor symptoms was possible under the combined use of immunoglobulins and corticotherapy during the initial phase. A three-years follow-up confirmed progressive motor recovery and stabilization under 6-months cyclophosphamide cycles relayed by azathioprine therapy.
CONCLUSIONS
Neurological complications can be inaugural in lead to urgent investigations and treatment. Peripheral and central neurological manifestations can coexist. The approach should integrate careful clinical assessment, as well as radiological and immunological findings.
PubMed: 38698975
DOI: 10.1016/j.heliyon.2024.e30004 -
Cureus Apr 2024Granulomatous lymphocytic interstitial lung disease (GL-ILD) is a rare, non-infectious pulmonary manifestation of common variable immunodeficiency (CVID). Diagnosing and...
Granulomatous lymphocytic interstitial lung disease (GL-ILD) is a rare, non-infectious pulmonary manifestation of common variable immunodeficiency (CVID). Diagnosing and managing GLILD remains challenging due to its poorly understood pathogenesis and high mortality. We present a complex case of a young female with CVID associated with lung and spinal cord involvement managed with azathioprine and rituximab.
PubMed: 38689676
DOI: 10.7759/cureus.59399