Atopic dermatitis (AD) is an inflammatory disease affecting over 20% of the pediatric population, with 85% of cases presenting before the age of five. Recently, therapeutic options in pediatric patients have evolved rapidly, following extensive development in adult treatments.

Anti-N-methyl-D-aspartate receptor encephalitis is a form of autoimmune encephalitis with acute or subacute neuropsychiatric symptoms. Despite this fact, due to a couple of factors, this condition remains insufficiently acknowledged and is an under-recognised clinical scenario. We describe a case of a patient presenting with fever, headache and altered sensorium along with a history of disorientation, episodes of abnormal body movements and loss of consciousness in the later phase. She was initially thought to have Status epilepticus with tuberculous meningoencephalitis but her cognitive functions did not improve despite appropriate treatment. She displayed features away from the usual course of disease leading to suspicions of Autoimmune Encephalitis and Anti-N-methyl- D-aspartate receptor reports later confirmed the diagnosis. Methylprednisolone and Intravenous Immunoglobulin was started empirically and she was discharged in stable health with stabilised emotional and cognitive function with Azathioprine and Levetiracetam continued. Our findings suggested early diagnosis and prompt immunotherapy treatment beneficial for the outcome.

Vogt-Koyanagi-Harada disease is a multisystem autoimmune inflammatory disorder that affects the eyes, ears, skin, and the nervous system. It is a rare disease that mainly affects Asian, Hispanic, and Middle Eastern populations. Systemic steroids and immunosuppressants are frequently used to treat autoimmune diseases like this. Despite the fact that they reduce morbidity, immunosuppressants can have a number of side effects and are difficult to use, particularly when treating uncommon autoimmune illnesses. We present a case of a 56-year-old man who visited our health facility complaining of increased tears in both eyes along with bilateral blurring of vision. He was subsequently identified as having Vogt-Koyanagi-Harada disease. After prednisolone and methotrexate failed to have the desired effect, he was treated with azathioprine, which caused pancytopenia, and manifested as fever with positive blood culture for a coagulase-negative staphylococcus infection.

Ulcerative colitis is a condition of chronic inflammation affecting the large intestine. The disease is typically characterized by periods of active inflammation alternating with periods of remission. For patients with moderate to severe ulcerative colitis, the first line for induction therapy options includes biologic agents (with or without an immunomodulator) or glucocorticoids. This case highlights the first successful case of ulcerative colitis treated with adalimumab with mesalamine and azathioprine. The patient was initially treated with mesalamine and azathioprine which did not result in significant clinical improvement but the addition of subcutaneous adalimumab showed a remarkable response with drastic improvement in just one year of treatment. Hence, adalimumab could be considered as an option in the management of severe cases in Nepal.

With the increasing number of options for the treatment of moderate-to-severe atopic dermatitis, clinicians need guidance on a practical approach to selecting a systemic agent for specific patient populations. We convened an expert panel consisting of 12 members to conduct a literature review and summarize relevant data related to six scenarios of clinical interest: comorbid asthma, ocular surface disease, history of cancer, past and ongoing infections of interest (including herpes simplex virus, herpes zoster, hepatitis B, and tuberculosis), pregnancy and lactation, and the elderly. We performed a literature search and examined each clinical scenario with respect to three major categories of available systemic agents: traditional systemics (azathioprine, cyclosporine A, methotrexate, and mycophenolate mofetil), Janus kinase inhibitors (abrocitinib, baricitinib, and upadacitinib), and biologics (dupilumab, lebrikizumab, and tralokinumab). The expert panel and steering committee met virtually to review the data and discuss the drafted consensus statements. A modified Delphi process was used to arrive at a set of final consensus statements related to the systemic treatment of AD in these specific patient populations. To provide practical guidance on the choice of systemic therapy for atopic dermatitis in these six topics of clinical interest, 25 expert consensus statements and a summary of the supporting data are presented herein.

Acute pancreatitis is an inflammatory disorder of the pancreas and a leading gastrointestinal cause of admission in the United States. The most common causes of acute pancreatitis are gallstones and alcohol. It is rarely caused by medications. Azathioprine-induced pancreatitis is rare and is more common in patients with Crohn's Disease. In this case report, we present a rare case of azathioprine-induced pancreatitis in a 58-year-old African American patient with systemic lupus erythematosus.

The pharmacological treatment of systemic lupus erythematosus (SLE) aims to decrease disease activity, progression, systemic compromise, and mortality. Among the pharmacological alternatives, there are chemically synthesized drugs whose efficacy has been evaluated, but which have the potential to generate adverse events that may compromise adherence and response to treatment. Therapy selection and monitoring will depend on patient characteristics and the safety profile of each drug. The aim of this review is to provide a comprehensive understanding of the most important synthetic drugs used in the treatment of SLE, including the current treatment options (mycophenolate mofetil, azathioprine, and cyclophosphamide), review their mechanism of action, efficacy, safety, and, most importantly, provide monitoring parameters that should be considered while the patient is receiving the pharmacotherapy.

Autoimmune hepatitis (AIH) is an immune-mediated inflammatory liver disease of uncertain cause, and its manifestations appear to vary by race and ethnicity. The literature on AIH in the Middle East, including Jordan, is scarce; therefore, this study aimed to determine the clinical characteristics of AIH in an understudied population. This retrospective chart review study was conducted on AIH patients who presented to Jordan University Hospital over a seven-year period (2014-2020). Retrieved data included sociodemographics, liver function tests, autoimmune serologic markers, viral hepatitis serology, findings on liver biopsies, treatment regimens, post-therapy outcomes and treatment-related complications. The total number of AIH patients included in the study was 30, divided as follows: type 1 AIH (n = 17, 56.7%), type 2 AIH (n = 2, 6.7%), seronegative AIH (n = 9, 30.0%), and two patients who had AIH-primary biliary cirrhosis overlap syndrome (6.7%). The mean age at diagnosis was 44 years (standard deviation: 17 years), with a female predominance (n = 25, 83.3%). Acute presentation was seen among 18 patients (60.0%). Mild to moderate fibrosis (F1 and F2 on METAVIR scoring system) without cirrhosis was observed among patients who underwent liver biopsies (10/19, 52.6%). The majority of patients (73.3%) were initially treated with prednisone, with azathioprine combination in 16.7% of the patients. At 6 months post initial treatment, twenty patients (66.7%) achieved biochemical remission, four patients had incomplete response, two patients failed to improve (one died during the induction of remission period due to AIH-related complications), and four patients were lost to follow-up. This study provided an updated overview of AIH in Jordan. The results showed typical female predominance, and interestingly high rates of acute presentation and seronegative disease. Future longitudinal studies are recommended to address the nature and long-term prognosis of AIH in Jordan.

Patients with chronic inflammatory bowel diseases (IBD) have increased risk of developing intestinal and extraintestinal cancers. However, once a diagnosis of malignancy is made, the therapeutic management of Crohn's disease (CD) and ulcerative colitis (UC) can be challenging as major guidelines suggest discontinuing the ongoing immunosuppressant and biological therapies for at least 2-5 years after the end of cancer treatment. Recently, new molecules such as vedolizumab and ustekinumab have been approved for IBD and limited data exist on the real risk of new or recurrent cancer in IBD patients with prior cancer, exposed to immunosuppressants and biologic agents. Thus, a multidisciplinary approach and case-by-case management is the preferred choice. The primary aim of our review was to summarize the current evidence about the safety of reintroducing an immunosuppressant or biologic agent in patients with a history of malignancy and to compare the different available therapies, including gut-selective agents. The secondary aim was to evaluate the clinical course of the IBD patients under cancer treatment who do not receive any specific immunosuppressant treatment after the diagnosis of cancer.

The polyomaviruses that infect humans, JC virus (JCV) and BK virus (BKV), can establish persistent infections in the cells that make up the renal system, causing nephritis and BKV-associated nephropathy in up to 10% of renal transplant patients, and of these, 90% lose the graft and return for hemodialysis. This study aimed to determine the prevalence of polyomaviruses (PyV) in the population with chronic kidney disease (CKD), classified into three groups (conservative, dialysis, and transplanted) and a control group. Urine samples were collected from 290 individuals, including 202 patients with CKD and 88 from the control group. PyV screening was performed by PCR amplification of a fragment of the VP1 region, and the JCV and BKV species were distinguished through enzymatic digestion with the restriction endonuclease BamHI from the amplification of a TAg region. All amplification products were visualized on a 3% agarose gel. The prevalence of PyV infection was correlated with clinical-epidemiological variables using the chi-squared and Fisher's exact tests. In the group with CKD, the prevalence of PyV was 30.2%, a higher rate being observed in conservative patients (36.66%; 22/60), followed by dialysis patients (30.48%; 25/82), and transplanted patients (20%; 12/60). In the control group, the prevalence was 46.59% (41/88). The differentiation between species revealed that JCV was present in 77.8% and BKV in 22.2% of the group with CKD. The prevalence of infection was higher in male patients (59.32%), whose most common pathology was systemic arterial hypertension (35.59%). In the group of transplanted patients, there was a statistically significant association between infection and the use of the immunosuppressant azathioprine (p = 0.015). The prevalence of PyV infection was higher in the control group than in the group with CKD, being predominant in males and in patients with systemic arterial hypertension.

The prevalence of this rare and fatal entity varies in different regions and ethnicities. The objective of this study was to determine clinicopathological characteristics and predictors of outcome in rapidly progressive glomerulonephritis (RPGN). We aimed to prospectively study the clinicopathological profile and determine the predictors of renal outcome in patients with RPGN. This study included 40 patients of biopsy-proven RPGN. The diagnosis of RPGN was based on renal histology showing crescents in >50% of glomeruli. All patients were given induction with intravenous (IV) methylprednisolone (0.5 g) for three days followed by maintenance with oral prednisolone (1 mg/kg/day) and six IV pulses of cyclophosphamide (0.5 g) given fortnightly followed by maintenance therapy with azathioprine (2 mg/kg/day). The outcomes were recorded. Three-fourth of the patients (77.4%) required renal replacement therapy (RRT) at diagnosis. More than half of patients (57.5%) were antineutrophil cytoplasmic antibodies mediated. Immune complex and anti-glomerular basement membrane (GBM) disease constituted 25% and 17.5%, respectively. Clinical features, biochemical parameters, histological features, and type of RPGN were analyzed for association with primary outcomes. Entry serum creatinine, entry estimated glomerular filtration rate (eGFR), RRT on admission, interstitial fibrosis, tubular atrophy, and interstitial infiltrates were the parameters which showed association with primary outcomes of the study (P <0.05). In secondary outcomes, infections were the most common (55%), followed by neutropenia (40%). One-fourth of the patients (25%) died during the course of the study. Cause of mortality was infections (50%), cardiovascular system (30%), stroke (10%), and unknown (10%). Our prospective study from north India shows that RPGN is not an uncommon cause of renal failure and there is preponderance in the elderly patients (>60 years). Pauci-immune RPGN is the most common cause of RPGN followed by immune-complex and anti-GBM disease. Entry serum creatinine, eGFR, and RRT on admission predicted the outcome.

Autoimmune hepatitis (AIH) is a rare immune-mediated disease predominantly seen in women and triggered by various environmental factors. Rarely, AIH can be triggered by an underlying malignancy. We report a woman in her 60s who presented with markedly abnormal liver biochemical tests. Serology was positive for anti-smooth muscle antibodies and a liver biopsy confirmed AIH. During the hospital course, she developed sepsis and acute renal failure requiring dialysis support. Serum protein electrophoresis (SPEP) showed a monoclonal IgG kappa protein of 1.92 g/dL and a bone marrow biopsy revealed 7% clonal plasma cells. She had lytic lesions on skeletal survey confirming the diagnosis of a coexisting multiple myeloma (MM). Given her markedly abnormal liver chemistries, we decided to treat the AIH first and use the steroids (an important anti-myeloma therapy) as a bridge to the specific treatment of the MM once her clinical condition improved. She was treated with oral prednisone and azathioprine for AIH. One month later, a marked improvement in liver biochemical test results was noted and she was started on oral ixazomib, lenalidomide and dexamethasone. She received palliative radiotherapy to the lumbar spine (L2), left femur, and ischium lesions. This case highlights a rare co-occurrence of AIH and MM, the underlying mechanism of which is unknown.

The International Study of Kidney Disease in Children (ISKDC), begun in 1966, conducted the first international collaborative randomized blinded controlled trial in pediatric nephrology and one of the first in either pediatrics or nephrology. For this trial, the ISKDC developed the criteria, such as those for response and relapse, used today to describe the clinical course of the nephrotic syndrome, and the trial generated the nephropathologic terminology and criteria which largely remain the current standards. Over an approximately 20-year span, the ISKDC followed the natural history and evaluated the therapeutic effectiveness of therapies in over 500 children with the nephrotic syndrome from three continents. It published 14 peer-reviewed studies and several reports and commentaries, many of which helped create current standards of practice for therapy of childhood nephrotic syndrome and consequently remain highly cited today. The ISKDC continues to be an important model for subsequent collaborative studies and was the impetus for the development of regional and national pediatric nephrology societies leading to the recognition and growth of pediatric nephrology as a separate subspecialty. A higher resolution version of the Graphical abstract is available as Supplementary information.

BACKGROUND: Patient decision aid (PDA) is a tool, which helps the improvement of shared decisionmaking and is a part of the paradigm shift from physician-centered decisions to patient-centered shared decision making. In this study, we aimed to describe the process used to develop a PDA for facilitating shared decision-making about treatment in patients with inflammatory bowel disease (IBD) who need medication (corticosteroid, azathioprine, anti-TNF, and infliximab) or surgery. METHODS: The development process of PDA included: 1) The development of a prototype based on literature review and interview 2) 'Alpha' testing with patients and clinicians 3) 'Beta' testing in real conditions and 4) The production of a final version. This process took about 12 months (2019-2020). The participants were adult patients with IBD, gastroenterologists, and nurses. RESULTS: The final PDA contains four important sections: 1) Introduction about IBD disease, the purpose of developing PDA, and emphasis on shared decision-making 2) Benefits and risks of main medicines 3) The success rate as well as the incidence of complications after surgery, and 4) The conclusion about patients' satisfaction with PDA to choose the treatment options. Besides, PDA evaluation in the real world setting showed that 100% of physicians (n=4) and 86% of patients (n=12) were completely satisfied with the content of the PDA and considered it applicable and useful. CONCLUSION: This PDA can help patients participate in the shared decision-making process and select the best medical and surgical treatment methods. The feedback received from clinicians and patients showed their satisfaction with using the PDA.

Pruritus is a major and burdensome symptom in atopic dermatitis (AD). The number of systemic treatments available for AD has increased recently, enabling improved patient relief.

Azathioprine (AZA) drug hypersensitivity reaction (DHR) is an uncommon yet potentially lethal condition that often goes unrecognised in patients with anti-Neutrophil Cytoplasmic Antibody (ANCA) associated vasculitis (AAV). We conducted a retrospective review of AAV patients on AZA maintenance therapy (N = 35). Participants were categorised into those who had experienced AZA-DHR (N = 15) and those who were AZA-tolerant (N = 20). Human leukocyte antigen (HLA) typing was performed in both groups. The primary endpoint was identification of a HLA gene association with AZA-DHR in the context of AAV. HLA-C*06:02, was solely expressed in AZA-DHR patients (33.3 %), whilst no patient who tolerated AZA carried this allele (0.0 %). This yielded a positive predictive value of 100 % for HLA-C*06:02 in predicting AZA-DHR in AAV patients, negative predictive value of 66.7 %, sensitivity of 33.3 % and specificity of 100 %. HLA-C*06:02 may predict the development of AZA-DHR in patients with AAV and inform safer therapeutic choice.

Oral manifestations of IBD can be specific or nonspecific, due to intestinal malabsorption or induced by pharmacological treatments. Oral manifestations may precede the diagnosis of IBD or interfere with timely diagnosis and treatment. The paradigm of treatment for oral lesions in patients with IBD is based on treating and controlling the intestinal manifestations of the underlying disease as well as local methods of treatment can be used. Here, we report a case of a patient with the oral manifestation of IBD, who responded to treatment with infliximab. The patient was admitted with complaints of long-term nonhealing ulcers of the lips and oral cavity, odynophagia, and there were no intestinal manifestations at that time. The appearance of the disease in 2008 with lesions of the oral cavity, however, Crohn's disease was diagnosed in 2016. The patient began therapy with azathioprine and prednisolone, and later developed hormone dependence and osteoporosis. In 2020, against the background of immunosuppressive therapy, the patient has an exacerbation, especially increased symptoms from the lesion of the oral cavity. In 2020 was started therapy with vedolizumab, with slight improvement. Due to the ineffectiveness of the latter's therapy, therapy with monoclonal antibodies (infliximab) was started in February 2021. Currently, patient is in clinical, laboratory, and endoscopic remission.

Biologics are recommended to treat paediatric ulcerative colitis (UC) that is chronically active or steroid-dependent despite aminosalicylic acids (5-ASA) and thiopurine treatments. Anti-tumour necrosis factor inhibitors (Anti-TNF inhibitors) are the agents of choice and vedolizumab could be considered as second-line biologic therapy.In the current case, we aim to describe a successful long-term treatment with vedolizumab in a 9-year-old boy with severe UC and primary non-response to infliximab. Concomitant azathioprine was used, and steroid refractoriness was also detected. Drug and anti-drug antibody levels were negative after infliximab induction so a switch to a 6-week-induction vedolizumab regimen followed by a maintenance regimen as a monotherapy was decided. The clinical response and tolerability to vedolizumab allowed long-term disease remission. Vedolizumab is currently non-authorised to treat paediatric patients and there is limited data on long-term treatments to date. This case contributes to the literature by adding evidence on the long-term efficacy and safety of vedolizumab in paediatric UC.