Severe acute liver injury (ALI) is mostly triggered by viral infections and hepatotoxic drugs; however, it can also be seen in systemic diseases. Hemophagocytic lymphohistiocytosis (HLH) is a rare, immune-mediated syndrome that presents as a life-threatening inflammatory disorder affecting multiple organs. Secondary causes occur mainly in the set of malignancy, infection, and autoimmune disease, and are seldom triggered by vaccination. Although liver involvement is common, presentation as severe ALI is rare. We describe a case of a 65-year-old male with history of low-risk chronic lymphocytic leukemia and rheumatoid arthritis treated with prednisolone who presented with persistent fever and jaundice 1 week after COVID-19 vaccination. The diagnosis was challenging given the predominant liver impairment, characterized by hyperbilirubinemia, transaminases over 1,000 U/L, and prolonged INR, which prompted an extensive investigation and exclusion of autoimmune, toxic, and viral causes of hepatitis. Laboratory workup revealed bicytopenia, hyperferritinemia, which together with organ failure and evidence of hemophagocytosis in bone marrow suggested the diagnosis of HLH. After excluding infectious etiologies, flare of rheumatological disease, and the progression of hematological disease, HLH was diagnosed. He was successfully treated with etoposide and corticosteroids, with dramatic improvement of liver tests. After exclusion of other causes of secondary HLH, the recent vaccination for COVID-19 was the likely trigger. We report a case of double rarity of HLH, as it presented with severe liver dysfunction which was probably triggered by vaccination. In this case, the predominant liver involvement urged extensive investigation of liver disease, so a high index of suspicion was required to make an early diagnosis. Clinicians should consider HLH in patients with unexplained signs and symptoms of systemic inflammatory response and multiorgan involvement, including severe liver involvement as the first presentation.
Cholestatic liver diseases may be associated with increased plasmatic cholesterol due to an abnormal lipoprotein – lipoprotein X (LpX). Correcting the underlying cause of cholestasis is the critical treatment of LpX-associated hypercholesterolemia without any proven benefit from conventional lipid-lowering agents. In some situations, plasma exchange may apply to prevent associated complications, such as hyperviscosity syndrome. The authors present the case of a 44-year-old man with orbital inflammatory pseudotumor on prednisolone, admitted due to hepatocellular and cholestatic lesion and severe hypercholesterolemia. Laboratory investigation established that hepatitis E virus was responsible for liver injury and showed that LpX mediated the severe hypercholesterolemia. Reduction of the immunosuppressive load contributed to virus clearance. The consequent resolution of cholestasis and cholesterol removal by plasmapheresis allowed lipid profile normalization. The authors report the first case of LpX-associated hypercholesterolemia in a patient with hepatitis E-induced cholestasis and revisit the role of the liver in lipid metabolism.
<b><i>Background/Aims:</i></b> Inflammatory bowel disease (IBD)-related knowledge empowers patients, providing the development of adaptative coping strategies. Recently, a more comprehensive questionnaire for evaluating IBD-related knowledge was developed, the IBD-KNOW. The main aim of our study was to translate to Portuguese and validate the IBD-KNOW questionnaire. We also explored the predictors of high scores of disease-related knowledge and the effect of knowledge on health-related quality of life (HRQoL) and therapeutic adherence. <b><i>Methods:</i></b> This is an observational, unicentric, and cross-sectional study. We translated and adapted the original English version of the IBD-KNOW questionnaire into Portuguese. Afterwards, IBD patients in the outpatient clinics were invited to fill out a multimodal form including the Portuguese version of IBD-KNOW, a visual analogue scale (VAS) of self-perceived knowledge, the Portuguese version of Short IBD Questionnaire (SIBDQ) and the Portuguese version of Morisky Adherence Scale 8-item (MMAS-8). Demographic and disease characteristics were collected. We assessed validity (through discriminate validity among non-IBD volunteers and correlation between IBD-KNOW and VAS) and reliability (through internal consistency, test-retest, and intraclass correlation). Statistical analysis was performed using SPSS version 25.0. <b><i>Results:</i></b> The mean IBD-KNOW score was significantly different among non-IBD validation group (doctors: 23, nurses: 18, and non-medical volunteers: 12, <i>p</i> < 0.001). IBD-KNOW showed a high internal consistency (Cronbach’s α 0.78) and intraclass correlation (0.90). As expected, the IBD-KNOW score was positively correlated with VAS for self-perceived knowledge (<i>r</i> = 0.45, <i>p</i> < 0.001). One hundred and one patients with IBD (54 with ulcerative colitis and 47 with Crohn’s disease) completed the questionnaire at baseline. Multivariate analyses showed that a high IBD-KNOW score was associated with longer disease duration (OR: 2.59 [CI 1.11–5.74]; <i>p</i> = 0.04), previous hospitalization (OR: 3.63 [CI 1.301–9.96]; <i>p</i> = 0.01), current biologic treatment (OR: 3.37 [CI 1.31–8.65]; <i>p</i> = 0.02), and higher educational level (OR: 4.66 [CI 1.74–10.21]; <i>p</i> = 0.02). Moreover, there was no significant correlation between overall IBD-KNOW and SIBDQ, nor between IBD treatment adherence (MMAS-8 = 8) and a higher mean IBD-KNOW score (<i>p</i> = 0.552). <b><i>Conclusion:</i></b> The Portuguese version of IBD-KNOW is a simple, valid, and reliable tool for assessing IBD-related knowledge. Longer disease duration, hospitalization, use of biologics, and higher educational level are associated with higher levels of knowledge. Higher patient knowledge was not associated with higher HRQoL and adherence to therapy.
We present the case of a 69-year-old male with Crohn's disease (CD) (Montreal A3L1B2) treated with infliximab and undergoing an ileocecal resection due to fibrotic stenosis. Surgery and the post-operative period were unremarkable, and infliximab was restarted after four weeks. Two weeks later, he was admitted due to acute onset of diffuse abdominal pain, hematochezia and arthralgias. He denied fever, recent infection, vaccination, or exposure to new drugs. On examination, he was hemodynamically stable, although left knee edema was detected, as well purpuric skin rash on both lower limbs (Fig. 1A). Laboratory evaluation showed renal dysfunction (creatinine 1.56 mg/dl) and no alterations of platelets count or coagulation tests.Abdominal computed tomography (CT) (Fig. 1B) revealed duodenal and jejunal wall thickening extending 20 cm, without changes in the mesenteric vessels.Esophagogastroduodenoscopy showed duodenal mucosa with hyperemia and diffuse erosions (Fig. 1C) and biopsies ruled out features of CD, ischemia or infection.Additional workup revealed nephrotic proteinuria (2.3 g/24 h), low C3 and high IgA.Antinuclear and antineutrophil cytoplasmic antibodies were negative. Epstein-Barr, cytomegalovirus, human immunodeficiency virus (HIV), and hepatitis B and C virus were negative. Colonoscopy was unremarkable. The patient fulfilled EULAR criteria for Henoch-Schönlein purpura (HSP): cutaneous vascular purpura with typical lower limb predominance, as well abdominal pain, arthralgia and renal involvement. Treatment with prednisolone 40 mg/day was started, with clinical resolution in the first week.Although no trigger was clearly pointed out, we decided to switch to ustekinumab.
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