---
title: "Mycophenolate mofetil for refractory acute-onset CIDP in rheumatoid arthritis: case report overview"
id: "frontiers-in-immunology-16-mycophenolate-mofetil-as-salvage-therapy-for-refractory-acute-onset-cidp"
canonical_url: "https://medichelpline.com/clinical-feed/frontiers-in-immunology-16-mycophenolate-mofetil-as-salvage-therapy-for-refractory-acute-onset-cidp"
content_type: "clinical_feed_article"
specialty: "Neurology"
source_name: "Frontiers in Immunology"
source_url: "https://www.frontiersin.org/articles/10.3389/fimmu.2026.1869194"
published_at: "2026-09-30T00:00:00.000Z"
evidence_level: "Journal Feed"
license: "CC-BY-NC-4.0 / Informational Use"
---
# Mycophenolate mofetil for refractory acute-onset CIDP in rheumatoid arthritis: case report overview
## Provenance & Clinical Metadata
- **Canonical URL:** https://medichelpline.com/clinical-feed/frontiers-in-immunology-16-mycophenolate-mofetil-as-salvage-therapy-for-refractory-acute-onset-cidp
- **Specialty:** [Neurology](https://medichelpline.com/clinical-feed/neurology.md)
- **Primary Source:** Frontiers in Immunology
- **Source URL:** [Original Journal Publication](https://www.frontiersin.org/articles/10.3389/fimmu.2026.1869194)
- **Published At:** 2026-09-30T00:00:00.000Z
- **Evidence Rating:** Journal Feed
## Executive GIST (TL;DR)
- The source article title reports use of **mycophenolate mofetil** as salvage therapy for refractory acute-onset **CIDP** occurring with **rheumatoid arthritis**. - The paper is identified as a CASE REPORT in Frontiers in Immunology. - The supplied source content in the prompt contains only site navigation and journal metadata; the clinical case details, methods, diagnostic findings, treatments, dosing, timelines, outcomes, and discussion points were not provided. - Because the body text with patient history, investigations, management, and follow-up is missing from the supplied source, no additional factual clinical details can be extracted or restated. - Any specific clinical data (age, sex, onset timing, electrophysiology, CSF, imaging, prior therapies, mycophenolate dosing, adverse events, or response) were not reported in the provided source content and therefore cannot be inferred or invented. - The title and journal indicate the clinical focus: salvage immunosuppression with **mycophenolate mofetil** in an acute, treatment-refractory form of **chronic inflammatory demyelinating polyneuropathy (CIDP)** associated with systemic autoimmune disease (**rheumatoid arthritis**). - For full clinical details and evidence, readers must consult the complete published article at Frontiers in Immunology; those specifics were not included in the supplied source excerpt.
## Clinical Analysis & Structured Key Points
Frontiers | Mycophenolate mofetil as salvage therapy for refractory acute-onset CIDP associated with rheumatoid arthritis: a case report CASE REPORT article Front. Immunol. , 30 September 2026 Sec. Autoimmune Disorders Volume 17 - 2026 | https://doi.org/10.3389/fimmu.2026.1869194 Published in Frontiers in Immunology Autoimmune Disorders 7 impact factor 11.3 citescore Editor & Reviewers Edited by M G Maria Giovanna Danieli Reviewed by I A Issa Alawneh G Y Gaodi Yuan Outline Figures and Tables Figure 1 View in article Table 1 Serial nerve conduction study findings and fulfillment of the 2021 EAN/PNS electrodiagnostic criteria for CIDP. View in article CASE REPORT article Front. Immunol. , 30 September 2026 Sec. Autoimmune Disorders Volume 17 - 2026 | https://doi.org/10.3389/fimmu.2026.1869194 Mycophenolate mofetil as salvage therapy for refractory acute-onset CIDP associated with rheumatoid arthritis: a case report J T Jinhui Tan 1 * H H Hai Huang 2 * L T Linghua Tan 3 B L Bo Li 1 1. Department of Rheumatology and Immunology, People’s Hospital of Longhua, Shenzhen, Shenzhen, Guangdong, China 2. Department of Health Management, People’s Hospital of Longhua, Shenzhen, Shenzhen, Guangdong, China 3. Department of Health Management, Jiangmen Wuyi Hospital of Chinese Medicine, Jiangmen, Guangdong, China See more Article metrics View details Abstract Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an immune-mediated neuropathy that rarely co-occurs with rheumatoid arthritis (RA). When CIDP proves refractory to first-line therapies, management becomes particularly challenging, especially in patients with multiple autoimmune conditions. A 43-year-old Chinese male with a 3-year history of seropositive RA in sustained remission presented with acute progressive quadriparesis, bilateral ptosis, and facial diplegia. Cerebrospinal fluid analysis revealed albuminocytologic dissociation, and nerve conduction studies demonstrated evolving demyelinating features, consistent with a diagnosis of acute-onset CIDP (A-CIDP). The patient responded inadequately to sequential treatment with intravenous immunoglobulin, high-dose corticosteroids, and the neonatal Fc receptor (FcRn) antagonist efgartigimod, experiencing repeated relapses during corticosteroid tapering. Mycophenolate mofetil (MMF) was subsequently introduced as a steroid-sparing agent at 0.75 g twice daily, enabling further prednisone tapering from 20 mg to 10 mg daily without relapse; MMF was later up-titrated to 1 g twice daily. Under a combined, multi-target immunosuppressive regimen of MMF, prednisone, methotrexate, iguratimod, and hydroxychloroquine, the patient achieved sustained neurological improvement, with prednisone successfully tapered to 10 mg daily by October 2025 and maintained thereafter. By April 2026, only residual mild ptosis persisted, and no infectious complications occurred. This case suggests that MMF-based multi-target immunosuppression may be a therapeutic option for RA-associated, treatment-refractory A-CIDP, potentially enabling successful glucocorticoid tapering even after an inadequate response to a short course of FcRn-targeted therapy. 1 Introduction Rheumatoid arthritis (RA) is a chronic, systemic autoimmune disease characterized by symmetrical polyarthritis, progressive joint destruction, and a broad spectrum of extra-articular manifestations ( 1 , 2 ). Although articular involvement dominates the clinical picture, peripheral nervous system complications are well recognized and include entrapment neuropathies, distal symmetric axonal polyneuropathy, and vasculitic multiple mononeuropathy ( 1 , 2 ). However, the co-occurrence of RA with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP)—a treatable but potentially disabling immune-mediated neuropathy of the peripheral nerves and nerve roots—has rarely been reported ( 3 , 4 ). CIDP is the most common chronic autoimmune neuropathy, with an estimated prevalence of approximately 0.7 to 10.3 per 100,000 population worldwide ( 5 , 6 ). It typically presents with progressive or relapsing symmetric proximal and distal weakness, sensory loss, and diminished or absent deep tendon reflexes, evolving over at least 8 weeks ( 7 ). The 2021 European Academy of Neurology/Peripheral Nerve Society (EAN/PNS) guidelines distinguish typical CIDP from several well-characterized variants, including distal, multifocal, focal, motor, and sensory forms ( 7 ). Both humoral and cell-mediated immune mechanisms are implicated in its pathogenesis ( 6 , 8 ), and first-line therapies—intravenous immunoglobulin (IVIG), corticosteroids, and plasma exchange—achieve symptomatic improvement in approximately 60–80% of patients ( 9 – 11 ). Approximately 15% of patients, however, remain refractory to all conventional treatment modalities, and even patients with initial responses frequently experience relapses or become dependent on high-dose corticosteroids ( 10 , 11 ). The coexistence of CIDP with systemic rheumatic diseases, including RA, is exceedingly rare and has been attributed to shared genetic predispositions and overlapping dysregulation of both T-cell and B-cell compartments ( 3 , 4 , 12 , 13 ). A 2017 case report described Lewis–Sumner syndrome, a multifocal variant of CIDP, developing almost concurrently with RA, raising the question of shared immunopathological mechanisms ( 3 ). The co-occurrence of immune-mediated neuropathies with systemic connective tissue diseases (CTDs), such as systemic lupus erythematosus and Sjögren’s syndrome (SS), is increasingly recognized. For instance, Guillain-Barré syndrome (GBS), an acute monophasic neuropathy, has been reported in association with various CTDs, including systemic lupus erythematosus (SLE) and primary SS ( 14 , 15 ), where it often presents with atypical features and a potentially poorer prognosis ( 16 , 17 ). A systematic review of SLE-related GBS found that over half of the patients presented with GBS before their SLE diagnosis, and the condition often required immunosuppressive therapy beyond standard GBS treatment ( 15 ). Although GBS and CIDP are distinct entities with different diagnostic criteria and typical time courses ( 17 ), they share overlapping immunopathogenetic mechanisms and therapeutic approaches, including the use of intravenous immunoglobulin and, in refractory cases, immunosuppressive agents ( 16 , 17 ). When CIDP presents with an acute onset—as occurs in up to 13% of cases (acute-onset CIDP, A-CIDP)—it may initially be misdiagnosed as GBS, with deterioration beyond 8 weeks or repeated relapses ultimately prompting diagnostic revision ( 7 , 18 ). Here, we report a challenging case of a patient with seropositive, long-standing RA in sustained clinical remission on conventional disease-modifying antirheumatic drugs, who developed A-CIDP. The disease was refractory to sequential treatment with IVIG, high-dose corticosteroids, and the neonatal Fc receptor antagonist efgartigimod, but was ultimately controlled with MMF as part of a multi-target immunosuppressive regimen. This case highlights the diagnostic and therapeutic challenges of managing CIDP in patients with coexisting autoimmune disease, underscores the potential of conventional immunosuppressive intensification as a salvage strategy, and contributes to the limited literature on this rare clinical association. 2 Case presentation A 43-year-old Chinese male with a 3-year history of seropositive rheumatoid arthritis (RA) presented to the neurology outpatient clinic in August 2024 with acute progressive quadriparesis and bilateral ptosis and was directly admitted to the neurology inpatient department. His RA had been diagnosed in February 2021 at the Longhua District People’s Hospital of Shenzhen, based on symmetric polyarthritis involving the proximal interphalangeal, metacarpophalangeal, and wrist joints, accompanied by prolonged morning stiffness exceeding one hour. Laboratory investigations at diagnosis revealed elevated inflammatory markers (erythrocyte sedimentation rate [ESR] 26 mm/h, C-reactive protein [CRP] 27.1 mg/L), strongly positive anti-cyclic citrullinated peptide antibody (>200 U/mL), rheumatoid factor 179.3 IU/mL, and positive anti-keratin antibody. Joint ultrasonography confirmed active synovitis. He was initially treated with prednisone 10 mg daily, methotrexate 10 mg weekly, hydroxychloroquine 200 mg daily, and sulfasalazine 1 g twice daily. Due to persistent articular symptoms, the treatment was modified in March 2021: hydroxychloroquine and sulfasalazine were discontinued, and iguratimod 25 mg twice daily plus celecoxib 200 mg twice daily were added. By July 2021, complete clinical remission of RA had been achieved, and prednisone was successfully discontinued by August 2021. For the subsequent three years, he remained in sustained RA remission on a maintenance regimen of methotrexate 10 mg weekly and iguratimod 25 mg twice daily. On July 24, 2024, the patient underwent elective facial cosmetic injections of hyaluronic acid and botulinum toxin (specific product details unavailable). Five days post-procedure, on July 29, he developed insidious-onset fatigue and limb weakness, initially manifesting as difficulty with shoulder abduction and ambulation. The symptoms progressed steadily: by August 2, 2024, he exhibited bilateral ptosis, which was markedly asymmetric with the left side more severely affected, bilateral lagophthalmos, left-sided peripheral facial droop, masticatory muscle weakness, blurred vision, and intermittent dysphagia. There was no diurnal fluctuation, no sensory complaints, and no headache or gait ataxia. On admission, neurological examination revealed a conscious and oriented patient with mild dysarthria. Cranial nerve examination demonstrated bilateral ptosis (left > right), bilateral lagophthalmos, loss of forehead wrinkling, shallowing of the left nasolabial fold, uvular deviation to the right, and a diminished left gag reflex. Tongue protrusion was midline, and extraocular movements were full without nystagmus. Motor examination revealed bilateral upper limb strength of 5-/5 and bilateral lower limb strength of 4/5. Muscle tone was normal in all four limbs. Deep tendon reflexes were globally diminished. Abdominal reflexes were preserved. Plantar responses were flexor bilaterally. Sensory examination suggested questionable hypoesthesia below both knees. Coordination testing was normal. There was no neck stiffness, and meningeal signs were absent. Brain magnetic resonance imaging (MRI) with magnetic resonance angiography and venography was unremarkable. A lumbar puncture was performed on August 12, 2024, yielding clear and colorless cerebrospinal fluid (CSF) under an opening pressure of 125 mmH 2 O. CSF analysis revealed marked albuminocytologic dissociation: protein 620 mg/L (elevated), glucose 4.00 mmol/L (normal), and white cell count within normal limits. Routine CSF studies, bacterial and fungal cultures, acid-fast bacillus smear, and cryptococcal antigen were all negative. Serum testing for anti-acetylcholine receptor and anti-muscle-specific kinase antibodies was negative, as was serum testing for botulinum toxin neutralizing antibodies (using the mouse neutralization bioassay). Furthermore, the patient’s clinical presentation, featuring ascending and asymmetric weakness, was inconsistent with the typical descending and symmetric flaccid paralysis seen in botulism, effectively ruling out systemic botulinum toxin effect as the primary etiology. The neostigmine test was equivocal. A comprehensive serum anti-ganglioside antibody panel (24 items) was positive for GM4-IgG. The initial nerve conduction study, performed on August 9, 2024, was unremarkable except for equivocal low-frequency decrement on right facial nerve repetitive stimulation; all limb nerve conduction velocities, distal latencies, and compound muscle action potential (CMAP) amplitudes were within normal limits. An initial diagnosis of acute GBS was made; however, given the diagnostic uncertainty between GBS and A-CIDP, the patient received a 5-day course of intravenous immunoglobulin (25 g daily) combined with pulsed intravenous methylprednisolone (500 mg/day for 4 days, tapered sequentially to 250 mg/day for 2 days, then 125 mg/day for 1 day) to cover both potential disease entities. Despite this treatment, significant bilateral ptosis, facial diplegia, and quadriparesis persisted, with only marginal subjective improvement. He was discharged on August 23, 2024, on oral prednisone 60 mg daily with a planned slow taper, alongside RA maintenance therapy. At this time, hydroxychloroquine 200 mg daily was reintroduced and added to his existing regimen of methotrexate and iguratimod, given his stable RA remission. Notably, throughout the entire CIDP disease course (from July 2024 to April 2026), the patient’s RA remained in sustained clinical remission, with consecutive monitoring showing ESR (ranging from 8 to 15 mm/h) and CRP (consistently 53 Yes (slowing) Distal CMAP amplitude (mV) 10.4 6.4 > 3.0 Yes (reduction) F-wave latency (ms) Normal (value NR) 28.01 50 No Distal CMAP amplitude (mV) 8.4 5.8 > 5.0 Yes (reduction) F-wave latency (ms) Normal (value NR) 25.96 53 No Distal CMAP amplitude (mV) 9.1 8.0 > 3.0 No F-wave latency (ms) Normal (value NR) 26.90 35 No Distal CMAP amplitude (mV) 8.9 8.8 > 3.0 No F-wave latency (ms) Normal (value NR) 44.91 < 61 No Serial nerve conduction study findings and fulfillment of the 2021 EAN/PNS electrodiagnostic criteria for CIDP. CMAP, compound muscle action potential; NR, not reported; EAN/PNS, European Academy of Neurology/Peripheral Nerve Society. Abnormal values supportive of demyelination are shown in bold. According to the 2021 EAN/PNS guidelines, electrodiagnostic support for CIDP requires at least two nerves showing definitive demyelinating abnormalities (motor conduction velocity slowing, distal CMAP amplitude reduction, distal latency prolongation, or F-wave prolongation) in the absence of significant conduction block. In this patient, the left ulnar nerve demonstrated both conduction velocity slowing and CMAP amplitude reduction, while the left median nerve showed CMAP amplitude reduction. These findings in two nerves meet the criteria for electrophysiologically supported CIDP (possible CIDP category). In light of this revised diagnosis, the patient received two doses of efgartigimod (1200 mg intravenously on September 9 and 13, 2024), resulting in only partial and transient improvement in facial muscle strength and limb power. Given the suboptimal clinical response and subsequent early relapse, the treatment strategy was reconsidered, and further doses were not administered. He was discharged on September 13, 2024, on prednisone 50 mg daily with a planned taper. However, in October 2024, he experienced a further relapse with worsening ptosis, prominent facial diplegia, masticatory weakness, and limb weakness, necessitating a third hospitalization. Intravenous methylprednisolone pulse therapy was again administered (500 mg/day for 2 days, tapered over 5 days) with modest benefit. Given the relapsing-remitting course and inadequate response to both IVIG and efgartigimod, MMF was introduced as a steroid-sparing immunosuppressant in April 2025 at a dose of 0.75 g twice daily, at which point prednisone had been tapered from 50 mg to 20 mg daily, but further reduction had repeatedly triggered worsening of weakness. Under this initial MMF dose (0.75 g twice daily), prednisone was gradually tapered from 20 mg to 10 mg daily over the following months, without symptom recurrence. In October 2025, MMF was up-titrated to 1 g twice daily to consolidate the therapeutic response and to allow for potential further tapering in the future, and prednisone was successfully maintained at 10 mg daily, thereafter, combined with methotrexate, iguratimod, and hydroxychloroquine. Under this intensified combined immunosuppressive regimen, the patient demonstrated sustained neurological improvement. Although not formally quantified using validated instruments, retrospective estimation based on physical examination records during hospitalizations and follow-up visits suggested that the patient’s Medical Research Council (MRC) sum score improved from approximately 48/70 at nadir (September 2024) to approximately 68/70 by April 2026, with the most pronounced improvement in proximal lower limb strength (from 4/5 to 5-/5) and facial muscle function. Correspondingly, the modified Rankin Scale (mRS) score improved from 4 (requiring assistance with walking) to 1 (no significant disability). By April 2026, only residual mild ptosis persisted, and no functional limitations were reported. Figure 1 provides a chronological overview of the clinical course, key therapeutic interventions, and relevant laboratory trends from the time of CIDP onset through the final follow-up. As shown in the figure, the patient experienced three distinct relapses between August and October 2024, each occurring during corticosteroid tapering; it was only after the introduction of MMF in April 2025 that sustained, stable corticosteroid reduction without relapse was achieved. Figure 1 Clinical timeline, therapeutic interventions, and laboratory trends. This image illustrates the patient’s clinical course from symptom onset (July 29, 2024) to the final follow-up (April 6, 2026). The timeline integrates key clinical events (hospitalizations, relapses), therapeutic interventions (IVIG, corticosteroids, efgartigimod, MMF), and laboratory findings (NCS, CSF protein) in a chronological, color-coded manner. Red bars (crit) indicate critical events, including the three distinct relapses occurring during corticosteroid tapering between August and October 2024. Gray bars (done) denote completed short-term treatments. Blue bars (active) represent ongoing maintenance therapies. Diamond markers (milestone) signify important clinical milestones. Sustained clinical improvement and successful glucocorticoid reduction without relapse were achieved only after the introduction of MMF in April 2025. By the final follow-up, the patient remained on MMF 1 g twice daily and prednisone 1
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