Rituximab and Intravenous Immunoglobulin (IVIG) for Refractory Eosinophilic Fasciitis - A Case Report

Sep 29, 2026

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Eosinophilic fasciitis (EF) is a rare disorder with an unknown cause. This case study describes a 40-year-old man whose EF did not respond to standard treatments, but improved after receiving rituximab combined with intravenous immunoglobulin (IVIG). The report highlights a potential therapeutic role for rituximab in refractory EF. Further studies are required to verify effectiveness and evaluate long-term safety outcomes.

LanguageEnglish

CategoryHealthcare

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Abstract

Introduction

Case History and Physical Examinations

History/Examination

Investigations, Diagnosis and Treatment

Clinical Case Reports CASE REPORT OPEN ACCESS Rituximab and Intravenous Immunoglobulin (IVIG) for Refractory Eosinophilic Fasciitis: A Case Report Maryam Sahebari1 | Motahhareh Karimoddini2 | Naser Tayyebi Meibodi3 | Sajad Ataei Azimi4 | Shadan Tafreshian 1Rheumatic Diseases Research Center, Mashhad University of Medical Sciences, Mashhad, Iran | 2Internal Medicine Department, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran | 3Department of Pathology, Mashhad University of Medical Sciences, Mashhad, Iran | 4Hematology and Oncology Department, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran Correspondence: Shadan Tafreshian ([tafreshiansh4022@mums.ac.ir](tafreshiansh4022@mums.ac.ir)) Received: 23 January 2025 | Revised: 26 November 2025 | Accepted: 21 December 2025 Keywords: eosinophilic fasciitis | intravenous immunoglobulin | refractory eosinophilic fasciitis | rituximab ABSTRACT Eosinophilic fasciitis (EF) is a rare condition with an unknown cause. This case study showed that a 40-year-old man with EF did not respond to standard treatments but improved after receiving rituximab and intravenous immunoglobulin (IVIG) . Further studies are needed to confirm rituximab's effectiveness and long-term safety for EF. 1 | Introduction Eosinophilic fasciitis (EF), also known as Shulman's syndrome, is a rare connective tissue disorder characterized by inflammation and thickening of the fascia. This condition is often associated with peripheral eosinophilia, and its exact cause remains unknown. Symptoms of EF primarily include skin hardening and stiffness, particularly in the extremities [1, 2] . Standard treatment typically consists of high-dose corticosteroids and immunosuppressive agents, such as methotrexate. However, some patients do not respond effectively to these therapies, which poses a significant challenge for management [3] . Research is ongoing to better understand the role of B cells in the pathogenesis of EF. Rituximab, a monoclonal antibody that targets CD20-positive B cells, has been used successfully to treat various autoimmune diseases. Its positive outcomes in other conditions suggest that it could be a new treatment option for EF patients who do not respond to standard treatments [4, 5] . In this case report, a patient with EF resistant to corticosteroids and methotrexate demonstrated mild clinical improvement after receiving treatment with rituximab. This suggests that rituximab may bean effective treatment strategy for managing refractory EF. 2 | Case History and Physical Examinations 2.1 | History/Examination A 40-year-old professional bodybuilder and fireman with no medical history and no drug and supplement use developed severe myalgia and arthralgia 5 h after long-distance mountain climbing with a 30-kg backpack, and 48 h later, night fever and sweating, non-productive cough and fatigue were added to his symptoms. The patient went to the emergency department and underwent a workup. In the initial examination, the vital signs were stable and the temperature was 37.9°C. Wheezing was heard in lung auscultation. Other examinations were normal. 2.2 | Investigations, Diagnosis and Treatment The findings of the laboratory tests are shown in Table 1. Due to hyper eosinophilia (eosinophil count: 3944/mm3), spirometry and lung Computed Tomography (CT) were performed, which were normal. Additional laboratory tests were requested, which were all negative (Table 1) . Due to hyper eosinophilia, a This is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited. © 2026 The Author(s). Clinical Case Reports published by John Wiley & Sons Ltd. Clinical Case Reports, 2026; 14:e71766 1 of 6 [https://doi.org/10.1002/ccr3.71766](https://doi.org/10.1002/ccr3.71766) TABLE 1 | The results of laboratory t

FAQs

What condition is discussed in the case report?

The case report focuses on eosinophilic fasciitis (EF), a rare connective tissue disorder also known as Shulman's syndrome.

Why was the patient considered refractory?

The patient did not respond effectively to standard treatments such as high-dose corticosteroids and immunosuppressive agents like methotrexate.

What treatment combination led to improvement?

Treatment with rituximab together with intravenous immunoglobulin (IVIG) was associated with mild clinical improvement.

Sep 29, 2026

Uploaded by Cipher