Atypical hemolytic uremic syndrome (aHUS) is a rare form of thrombotic microangiopathy (TMA) which generally presents as a triad of thrombocytopenia, hemolytic anemia and renal failure. We present the case of a 69-year-old woman with ongoing fevers, arthralgias, diffuse rash and pharyngitis for 3 months. Investigation revealed an elevated erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and ferritin; however, autoimmune and infectious studies were unremarkable, raising the suspicion for adult onset Still's disease (AOSD). Before out patient therapy could be initiated, she presented to our emergency room (ER) with a grand mal seizure and persistence of her initial triad of fevers, arthralgias and rash. Evaluation revealed non-immune hemolytic anemia, thrombocytopenia, and abnormal renal function consistent with TMA and the patient was subsequently started on plasmapheresis, hemodialysis and corticosteroid therapy. ADAMTS (a disintegrin and metalloproteinase with thrombospondin motifs)-13 activity was 38%, ruling out thrombotic thrombocytopenic purpura (TTP). A kidney biopsy demonstrated glomerular changes of TMA and a diagnosis of secondary aHUS triggered by AOSD was established. The patient was treated with eculizumab and high dose steroids with improvement in her laboratory values, eventually becoming hemodialysis-independent. This case highlights the clinical urgency in the prompt recognition of AOSD, a potent inflammatory disorder, which when co-existing with a complementdysregulatory defect, can significantly augment TMA disease severity.
Schnitzler’s syndrome is a rare clinical entity characterized by intermittent, non-pruritic urticarial rash, fevers, arthralgias, myalgias and monoclonal gammopathy, most commonly of the immunoglobulin M (IgM) subtype. Schnitzler’s syndrome should be considered in the differential diagnosis of fever of unknown origin. We report a case of a 56-year-old healthy Caucasian female, who initially presented to the primary care physician’s office with complaints of severe generalized fatigue and myalgias involving thighs and calves. Patient subsequently underwent extensive rheumatologic workup, and was treated with multiple courses of steroids with temporary resolution of symptoms. During the course of her workup she was found to have IgM kappa monoclonal gammopathy, and was referred to hematology for further evaluation. The constellation findings of fever, arthralgias, chronic intermittent non-pruritic urticaria, myalgias, and a negative rheumatologic workup in the presence of IgM monoclonal gammopathy raised the suspicion of Schnitzler’s syndrome. Following completion of additional workup, she was started on anakinra 100 mg daily with prompt resolution of her symptoms. Due to the rarity of the disease, the diagnosis of Schnitzler’s syndrome is often delayed, with an average time to diagnosis being approximately 5 years. The symptoms in most cases can be debilitating and add to significant morbidity as noted in our patient, who required bilateral hip arthroplasty at a much younger age than expected. Published reports discuss the poor quality of life associated with the delayed diagnosis and unawareness of potential end organ damage. With our case report we like to highlight the disease characteristics for an early identification to prevent further organ damage believed to be from chronic inflammation. Early diagnosis and treatment with agents such as interleukin-1 (IL-1) inhibitors can promptly provide symptomatic relief, reduce inflammation and prevent organ damage.
Background Each year, approximately 8000 cases of cholangiocarcinoma are recorded in the USA. Surgical resection is considered to be the only curative option. Despite surgery as a curative approach, many patients will require adjuvant therapies in the form of chemotherapy (ChT) or chemoradiotherapy (CRT). As such, we sought to analyze outcomes in patients with non-metastatic cholangiocarcinoma receiving adjuvant ChT or CRT following surgical resection. Methods We queried the National Cancer Database (NCDB) for patients with a diagnosis of non-metastatic cholangiocarcinoma between the years 2010 and 2015 who underwent adjuvant ChT or CRT following surgery. Overall survival (OS) was calculated using Kaplan Meier method. Cox proportional hazard ratios were used to identify predictors of overall survival, and logistic regression was used to identify predictors of receiving each treatment. Results A total of 875 patients were identified who met the above eligibility criteria. Of these patients, 818 received adjuvant chemotherapy alone with 57 patients receiving adjuvant chemoradiation therapy. The median OS in patients receiving CRT was 19.8 months versus 11.9 months for ChT (p value < 0.0238). The 1- and 5-year survival rates between ChT and CRT were 50% vs 61% and 6% vs 13%, respectively (hazard ratio 0.7005; 95% CI 0.51–0.97; p value < 0.0294). Conclusion The results of this study suggest a potential benefit of chemoradiation therapy in the adjuvant setting, although the trends appear to show rare utilization. Given the limitations of our study, prospective corroboration is warranted.
e15677 Background: Annually approximately 5000 cases of cholangiocarcinoma are reported in the US. Of these, less than 40% are treated surgically with a curative intent. Post resection, local and distant recurrence rates are as high as 35% within two years. Positive surgical margins, with either Microscopic (R1) or Macroscopic (R2) disease, confer a higher risk of recurrence. We aim to analyze survival outcomes for different adjuvant treatment strategies in patients with R1/R2 resection. Methods: We queried the NCDB from 2004-2015 for patients with non-metastatic cholangiocarcinoma, who had an R1/R2 resection status post surgery. We then stratified these patients into three arms based on the adjuvant treatment they received: combined chemotherapy and radiation (CRT) vs chemotherapy (CTH) vs observation (OBS). Mean overall survival (OS) was compared for these three groups. OS was calculated from the date of diagnosis to the date of last contact or death using Kaplan Meier curves to present the cumulative probability of survival, with log-rank statistics to assess significance. Results: We identified 522 patients who met our inclusion criteria. Of these, 178 received CRT adjuvantly, 171 CTH, and 173 were observed. The OS in these arms was 42 vs 32 vs 30 months respectively (pooled p < 0.04). As compared to CRT, the hazard ratio was 1.4 (95% CI 1.07-1.72) for CTH and 1.6 (95% CI of 1.2-2.1) for OBS arm. At one year, 83% vs 41% vs 20% of the patients were alive in the respective cohorts and at 5 years, the OS rates were 56% vs 25% vs 13% respectively. Conclusions: Our data demonstrates that combined chemotherapy and radiation is superior to either chemotherapy increase in overall survival for CRT when compared to CHT and observation alone.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
hi@scite.ai
10624 S. Eastern Ave., Ste. A-614
Henderson, NV 89052, USA
Copyright © 2024 scite LLC. All rights reserved.
Made with 💙 for researchers
Part of the Research Solutions Family.