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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 -
Frontiers in Immunology 2024Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) is characterized by a widespread maculopapular rash, lymphadenopathy, fever, and multisystem involvement....
Sulfasalazine-induced drug reaction with eosinophilia and systemic symptoms (DRESS) coinfected with COVID-19 complicated by hemophagocytic lymphohistiocytosis: a case report.
Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) is characterized by a widespread maculopapular rash, lymphadenopathy, fever, and multisystem involvement. Conversely, hemophagocytic lymphohistiocytosis (HLH) is an infrequent yet critical condition presenting with fever, hepatosplenomegaly, cytopenias, coagulation abnormalities, and elevated inflammatory markers. The overlapping clinical and laboratory features between DRESS and HLH poses a significant diagnostic challenge. Secondary HLH (sHLH) typically occurs in adults triggered by viral infections, malignancies, rheumatologic diseases, or immune deficiencies. Recently, COVID-19 has also been identified as one of the triggers for sHLH. Herein, we present a case of Sulfasalazine-induced DRESS coinfected with COVID-19 that subsequently progressed into HLH. Our patient exhibited common hepatorenal and splenic involvement along with rare cholecystitis and appendicitis. However, a significant improvement was observed upon the addition of etoposide and azathioprine. We hypothesize that excessive activation of the immune system and cytokine storm due to DRESS combined with COVID-19 infection led to more extensive systemic damage resulting in HLH development. This highlights the potential for severe consequences when DRESS coincides with HLH during a COVID-19 infection.
Topics: Humans; Lymphohistiocytosis, Hemophagocytic; COVID-19; Drug Hypersensitivity Syndrome; Sulfasalazine; SARS-CoV-2; Coinfection; Male; Middle Aged; Female
PubMed: 38686382
DOI: 10.3389/fimmu.2024.1371490 -
BMC Rheumatology Apr 2024In 2013, rituximab was approved in France for the treatment of ANCA-associated vasculitis (AAV). The aim of the study was to compare the treatment and health events of...
INTRODUCTION
In 2013, rituximab was approved in France for the treatment of ANCA-associated vasculitis (AAV). The aim of the study was to compare the treatment and health events of adult incident patients with granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA), included before rituximab approval (over 2010-2012, Group 1) and those included after rituximab approval (over 2014-2017, Group 2).
METHOD
Data were extracted from the French National Health Insurance database (SNDS) including outpatient health care consumption and hospital discharge forms. Comparisons between inclusion periods were performed using Wilcoxon and χ² tests. Kaplan-Meier method was used to model the duration of treatment induction, maintenance, and off-drug periods. Fine and Gray tests were used to compare treatment phase durations.
RESULTS
A total of 694 GPA and 283 MPA patients were included in Group 1, while 668 GPA and 463 MPA patients were included in Group 2. Between the two inclusion periods, the proportions of patients treated with rituximab increased in the induction and maintenance phases whereas treatment with azathioprine declined. These proportions remained stable in the case of methotrexate, cyclophosphamide, and glucocorticoid-treated patients. Frequency of first-time hospitalized infections, diabetes and renal failure during the first year after inclusion increased for both groups.
LIMITATIONS OF THE STUDY
This is a retrospective study based on claims data including only 76% of people covered by health insurance in France. The period studied includes the learning phase of using rituximab. This study lacks biological data and precise quantitative analysis for the use of steroids, therefore the criteria for establishing diagnosis and therapeutic choice were unknown.
CONCLUSIONS
Introduction of rituximab reduced the use of azathioprine without affecting the use of glucocorticoids or cyclophosphamide.
PubMed: 38679737
DOI: 10.1186/s41927-024-00385-8 -
Medicine Apr 2024Immunoglobulin G4-related disease (IgG4-RD) can involve various organs throughout the body, primarily manifesting as endocrine dysfunction, visual impairment, jaundice,... (Review)
Review
RATIONALE
Immunoglobulin G4-related disease (IgG4-RD) can involve various organs throughout the body, primarily manifesting as endocrine dysfunction, visual impairment, jaundice, and limited sexual function. IgG4-related autoimmune pancreatitis is triggered by autoimmune reactions and characterized by structural changes in the pancreas and pancreatic ducts. The disease mainly affects middle-aged and elderly males, typically presenting as progressive painless jaundice and misdiagnosed as cholangiocarcinoma or pancreatic cancer.
PATIENT CONCERNS
This study reports a 54-year-old male who consulted with different institutions multiple times due to diabetes, pancreatitis, elevated liver enzymes, and jaundice.
DIAGNOSES
Magnetic resonance imaging revealed swollen head of the pancreas and atrophic tail. Liver and pancreatic tissue pathology showed IgG4 plasma cell infiltration, while liver biopsy indicated interface hepatitis, liver fibrosis, and pseudolobule formation, with no evidence of bile duct damage.
INTERVENTIONS
Following hormone therapy, the patient's serum IgG4 levels and liver enzyme levels returned to normal.
OUTCOMES
The disease relapsed 2 years after maintaining hormone therapy, and the patient underwent additional hormone-induced remission therapy combined with azathioprine.
LESSONS
The purpose of this research report is to enhance the awareness and understanding of IgG4-RD, emphasizing the necessity for personalized treatment strategies that take into account its recurrence, associations, and imaging features. This report provides valuable insights and guidance for clinicians in managing and diagnosing patients with IgG4-RD.
Topics: Humans; Male; Middle Aged; Cholangitis, Sclerosing; Autoimmune Pancreatitis; Immunoglobulin G4-Related Disease; Immunoglobulin G; Pancreas
PubMed: 38669380
DOI: 10.1097/MD.0000000000037922 -
Frontline Gastroenterology May 2024Surgery is a vital pillar in the management of Crohn's disease and medical options for prevention of recurrence after surgery are a key consideration. The main classes... (Review)
Review
Surgery is a vital pillar in the management of Crohn's disease and medical options for prevention of recurrence after surgery are a key consideration. The main classes of effective induction therapies have very different efficacy data for maintenance and this is more pronounced in the postsurgical setting. In this review article, the up-to-date Cochrane reviews on the topic are presented, including a network meta-analysis. The Cochrane evidence shows a high relapse rate in the first 5 years after surgery with placebo or no treatment. The reviews demonstrate that 5-aminosalicylic acid (5-ASA) agents are probably more effective than placebo on pairwise and network meta-analysis, with moderate certainty evidence of a number needed to treat (NNT) of 13. The Cochrane evidence demonstrates that adalimumab may be more effective than placebo on pairwise and network meta-analysis, with low certainty evidence of an NNT of 2. Thiopurine analogues may be effective on pairwise analysis, but may not be effective on network meta-analysis. There was no evidence to support the use of any other agent but these findings are of low and very low certainty. It is proposed that clinicians should consider adalimumab, 5-ASA and thiopurine analogue agents based on the findings of the Cochrane synthesis. The use of the evidence, including the Grading of Recomendations, Assessment, Development, and Evaluations (GRADE) certainty and magnitude of effect data, can support discussions with patients. Future research is needed to consider other therapies that are effective in medically induced maintenance given the low certainty of evidence limiting conclusions, either supporting or refuting their use.
PubMed: 38665790
DOI: 10.1136/flgastro-2023-102559 -
BMC Pediatrics Apr 2024Inflammatory bowel disease (IBD) is a chronic gastrointestinal disease that causes anorexia, malabsorption, and increased energy requirements. Childhood IBD can...
BACKGROUND
Inflammatory bowel disease (IBD) is a chronic gastrointestinal disease that causes anorexia, malabsorption, and increased energy requirements. Childhood IBD can significantly impact nutritional status and future health.
OBJECTIVE
This study aimed to analyze the nutritional status of patients with pediatric IBD at presentation and during follow-up and to identify predictors of nutritional outcome.
METHODS
This retrospective cohort study reviewed the medical records of children diagnosed with IBD in the Pediatric Department, Salmaniya Medical Complex, Bahrain, 1984 - 2023. Demographic data, clinical characteristics, and anthropometric data were collected. World Health Organization growth standards were used to interpret nutritional status.
RESULTS
Of the 165 patients, 99 (60%) had anthropometric data at presentation, and 130 (78.8%) had follow-up data. Most patients were males (64.6%) and had Crohn's disease (CD) (56.2%), while 43.8% had ulcerative colitis (UC). The median age at presentation was 10.9 years and the mean follow-up duration was 12.6 years. At presentation, 53.5% of the patients were malnourished, that decreased to 46.9% on follow-up. Thinness was reduced from 27.3% at presentation to 12.1% at follow-up (p = 0.003). There was an increased tendency to normal weight on follow-up (59.6%) compared to time of presentation (46.5%), p = 0.035. Overweightness showed a non-significant increase from 26.3% at presentation to 28.3% at follow-up (p = 0.791). Children with IBD were more likely to become obese when they grow up to adulthood (2.3% versus 20.5%, respectively, p < 0.001). Weight-for-age, and height-for-age at presentation were higher among CD compared to UC, but body mass index (BMI) at follow-up was higher among UC patients (p < 0.05). Thinness at follow up was associated with very early-onset disease (p = 0.02), lower weight and BMI at presentation (p < 0.001 each), younger age at follow-up (p = 0.002), pediatric age group (p = 0.023), lower hematocrit (p = 0.017), and higher C-reactive protein (p = 0.007). Overweight at follow up was associated with increased weight and BMI at presentation (p < 0.001 each), longer disease duration (p = 0.005), older age (p = 0.002), and azathioprine intake (p = 0.026). Considering follow-up duration, univariate analysis exhibited that Bahraini nationality, post-diagnosis disease duration, age at follow-up, occurrence of diarrhea, height, and BMI at presentation were factors that decreased liability to abnormal nutritional status, while CD, history of weight loss, perianal disease, and skin rash, and intake of prednisolone expressed increased liability of abnormal nutritional status (p < 0.05).
CONCLUSION
Pediatric IBD is associated with a high incidence of malnutrition. Thinness is more prominent at presentation, while overweight is higher on follow-up. Multiple risk factors aggravating abnormal nutritional status were highlighted. Accordingly, nutritional counseling should be prioritized in a multidisciplinary approach.
Topics: Humans; Male; Bahrain; Retrospective Studies; Female; Child; Nutritional Status; Adolescent; Crohn Disease; Child, Preschool; Colitis, Ulcerative; Inflammatory Bowel Diseases; Follow-Up Studies; Thinness
PubMed: 38658864
DOI: 10.1186/s12887-024-04720-3 -
Arquivos Brasileiros de Oftalmologia 2024Endophthalmitis is a severe form of purulent inflammation caused by the infection of the intraocular tissues or fluids. This infection infrequently occurs through...
Endophthalmitis is a severe form of purulent inflammation caused by the infection of the intraocular tissues or fluids. This infection infrequently occurs through endogenous routes, which are often correlated with major risk factors. Escherichia coli, a gram-negative rod, can cause endophthalmitis through hematogenous spread. We here report a 59-year-old man who presented to our service with acute visual impairment in his left eye, preceded by floaters. He was taking sirolimus and azathioprine for a transplanted kidney, had undergone catheterization for bladder atresia, and had a history of recurrent E. coli urinary tract infections. On evaluation, the left eye exhibited visual acuity of hand motion, anterior chamber reaction (3+/4+), and intense vitritis (4+/4+) with white flake clusters, which prevented appropriate retinal evaluation. Pars plana vitrectomy was performed, and the culture yielded E. coli. The present case highlights the importance of identifying the signs and symptoms of infection early so that diagnosis and treatment of endophthalmitis can be promptly initiated.
Topics: Humans; Endophthalmitis; Male; Middle Aged; Escherichia coli Infections; Escherichia coli; Vitrectomy; Eye Infections, Bacterial; Visual Acuity; Anti-Bacterial Agents
PubMed: 38656025
DOI: 10.5935/0004-2749.2023-0066 -
Nature Communications Apr 2024Polygenic variation unrelated to disease contributes to interindividual variation in baseline white blood cell (WBC) counts, but its clinical significance is...
Polygenic variation unrelated to disease contributes to interindividual variation in baseline white blood cell (WBC) counts, but its clinical significance is uncharacterized. We investigated the clinical consequences of a genetic predisposition toward lower WBC counts among 89,559 biobank participants from tertiary care centers using a polygenic score for WBC count (PGS) comprising single nucleotide polymorphisms not associated with disease. A predisposition to lower WBC counts was associated with a decreased risk of identifying pathology on a bone marrow biopsy performed for a low WBC count (odds-ratio = 0.55 per standard deviation increase in PGS [95%CI, 0.30-0.94], p = 0.04), an increased risk of leukopenia (a low WBC count) when treated with a chemotherapeutic (n = 1724, hazard ratio [HR] = 0.78 [0.69-0.88], p = 4.0 × 10) or immunosuppressant (n = 354, HR = 0.61 [0.38-0.99], p = 0.04). A predisposition to benign lower WBC counts was associated with an increased risk of discontinuing azathioprine treatment (n = 1,466, HR = 0.62 [0.44-0.87], p = 0.006). Collectively, these findings suggest that there are genetically predisposed individuals who are susceptible to escalations or alterations in clinical care that may be harmful or of little benefit.
Topics: Humans; Leukocyte Count; Male; Genetic Predisposition to Disease; Polymorphism, Single Nucleotide; Female; Multifactorial Inheritance; Leukopenia; Middle Aged; Aged; Adult; Immunosuppressive Agents
PubMed: 38649760
DOI: 10.1038/s41467-024-47804-5 -
Radiology Case Reports Jun 2024A 26-year-old male with Behçet's disease (BD) presented with recurrent oral and genital ulcers, bilateral pneumonia, and a left lower pulmonary artery aneurysm....
A 26-year-old male with Behçet's disease (BD) presented with recurrent oral and genital ulcers, bilateral pneumonia, and a left lower pulmonary artery aneurysm. Endovascular coil embolization was initially performed, followed by treatment with prednisone, colchicine, and azathioprine. Despite treatment, disease progression occurred, requiring additional embolization, intravenous pulse methylprednisolone, and cyclophosphamide. Ultimately, a combination of medical and endovascular interventions resolved the pulmonary arterial aneurysms. This case highlights BD's systemic inflammatory nature and vascular complications like pulmonary artery aneurysms. It emphasizes the importance of early detection and individualized, multidisciplinary care for such complications.
PubMed: 38645547
DOI: 10.1016/j.radcr.2024.02.054