Treatment for adult IgA vasculitis (IgAV), particularly severe cases, is not well established, although recent reports have suggested potential efficacy of rituximab (RTX). Here, we report a case highlighting the effectiveness of RTX based on pathophysiological and pathological considerations of IgAV, as well as a treatment protocol that has not been previously described. A 50-year-old man receiving glucocorticoids for cutaneous IgAV developed nephrotic syndrome and acute nephritic syndrome. Renal biopsy showed marked endocapillary and extracapillary proliferation with IgA deposition, consistent with severe IgAV nephritis. Despite glucocorticoid pulse therapy and intravenous cyclophosphamide, rapidly progressive glomerulonephritis ensued, prompting RTX initiation. With reference to peripheral blood CD19± B-cell monitoring, a single-dose RTX induction regimen was selected. Following RTX administration, the patient showed a favorable renal response. This case supports the potential efficacy of RTX in highly active IgAV with systemic symptoms. It suggests that a single-dose RTX induction approach may represent a feasible strategy to balance effective disease control with reduced infection risk in selected patients with severe adult IgAV, for which peripheral blood CD19+ B-cell monitoring may be helpful.
Rituximab-associated colitis, a rare toxicity that may occur following prolonged latency, has nonspecific features. We report a 42-year-old female with diffuse cutaneous systemic sclerosis and interstitial lung disease whose last rituximab infusion was 195 days before admission. She developed diarrhea and abdominal pain; initial computed tomography revealed no intra-abdominal focus. On day 12, colonoscopy revealed erosions and ulcers from the cecum to the descending colon; cytomegalovirus testing was negative. Symptoms persisted; repeat colonoscopy on day 20 confirmed multifocal ulceration, suggesting cytomegalovirus colitis or severe Crohn’s disease. By day 27, computed tomography showed ascending colon thinning, descending colon thickening, and haustral loss like ulcerative colitis. High-dose prednisolone was initiated owing to the inflammatory bowel disease-like process. Although clinical symptoms and inflammatory markers were improved, follow-up computed tomography revealed free air and perforation, necessitating emergency subtotal colectomy with ileostomy. Histology demonstrated ulcers extending to the subserosa, and noncaseating granulomas in the mucosa and submucosa. Immunohistochemistry demonstrated CD3-positive, CD20-negative, and CD4-predominant lymphocytic infiltration. This case highlights that rituximab-associated colitis may mimic inflammatory bowel disease, show prolonged latency, and progress to perforation despite apparent biochemical improvement, warranting early biopsy and careful radiologic follow-up. Rituximab-associated colitis should be considered even months following anti-CD20 exposure.
Primary hepatic undifferentiated carcinoma is an extremely rare and aggressive malignancy with no established systemic therapy, and the efficacy of immune checkpoint inhibitors (ICIs) remains unknown. We report a 67-year-old man with compensated hepatitis C–related cirrhosis who presented with a 27 mm segment 8 liver lesion and lymph node and bone metastases. Liver biopsy revealed undifferentiated carcinoma with high programmed death-ligand 1 (PD-L1) expression, and combination therapy with durvalumab and tremelimumab (Dur/Tre) was initiated. Shortly after treatment, the patient developed cranial nerve palsies (III, VII, VIII), suspected to represent either an immune-related adverse event or Ramsay Hunt syndrome; his symptoms improved with prednisolone and valaciclovir, allowing Dur/Tre to be resumed. After four courses, partial tumor shrinkage was achieved, and after 17 courses the primary tumor had decreased to 10 mm with complete resolution of nodal metastases, without further significant adverse events. To our knowledge, this is the first report of primary hepatic undifferentiated carcinoma with high PD-L1 expression demonstrating a sustained response to Dur/Tre. This case suggests that PD-L1 expression may serve as a potential biomarker for predicting ICI responsiveness in this rare and aggressive tumor.
This update and revision of the international guideline for urticaria was developed in accordance with the methods recommended by Cochrane and the Grading of Recommendations Assessment, Development and Evaluation (GRADE) working group. It is an initiative of the Global Allergy and Asthma Excellence Network (GA2LEN) and its Urticaria and Angioedema Centers of Reference and Excellence (UCAREs and ACAREs), with the participation of 210 delegates from 107 national and international societies, from 59 countries. The consensus conference was held on December 6th, 2024. This guideline was acknowledged and accepted by the European Union of Medical Specialists (UEMS). Urticaria is a frequent, mast cell-driven disease, defined by a rapid appearance of wheals, angioedema, or both. The lifetime prevalence of acute urticaria is estimated to be approximately 20%. Chronic urticaria, categorized as either chronic spontaneous urticaria or chronic inducible urticaria, is disabling, impairs quality of life, and affects performance at work and school, however, novel therapies are available. This updated version of the international guideline for urticaria covers the definition and classification of urticaria and outlines expert-guided and evidence-based diagnostic and therapeutic approaches for the different subtypes of urticaria.
Mid-pregnancy cervical length (CL) has limited predictive performance for spontaneous preterm birth (sPTB) in the normal range. The uterocervical angle (UCA) is a promising marker, but evidence regarding late-pregnancy UCA and its longitudinal change is limited. We evaluated UCA in mid- and late-pregnancy and the
