Inselspital Bern, University Hospital and University of Bern
Neurology

Author Of 2 Presentations

Disease Modifying Therapies – Risk Management Poster Presentation

P0359 - Natalizumab and eosinophilia: epidemiological characteristics and clinical associations (ID 1442)

Speakers
Presentation Number
P0359
Presentation Topic
Disease Modifying Therapies – Risk Management

Abstract

Background

Natalizumab is used as an immunomodulatory treatment in relapsing-remitting multiple sclerosis (RRMS). Blood eosinophilia as an adverse effect has been described.

Objectives

We aim to describe the frequency of blood eosinophilia and associated clinical symptoms in our monocentric cohort of natalizumab treated patients with relapsing remitting Multiple Sclerosis.

Methods

In our tertiary neurological care centre in Switzerland, we retrospectively longitudinally identified 115 natalizumab-treated and 116 untreated RRMS patients with eosinophil counts since July 2016 with our pharmacovigilance system and compared eosinophil counts, clinical symptoms and patient demographics.

Results

In total, 44/115 natalizumab-treated patients (38%) developed eosinophilia (>0.4 G/l), which occurred significantly more frequently compared to 116 untreated MS patients (n=3; 3%). Of 44 natalizumab-treated patients with eosinophilia, 43 remained asymptomatic; one patient one patient developed an eosinophilic pneumonia after 2 infusions of natalizumab, which resolved without sequelae after cessation. All untreated MS patients with eosinophilia remained asymptomatic.

Conclusions

Our cohort confirmed that eosinophilia is a potential side effect of natalizumab in RRMS patients and most commonly remains asymptomatic. However, in one of our natalizumab-treated patients (0.9% of all patients), an eosinophilic pneumonia occurred as a rare but severe side effect. Physicians should be vigilant for symptoms of an eosinophilic disease in natalizumab-treated patients. Further studies on drug safety in real-life settings using automated big data approaches are warranted to better describe drug-associated adverse effects.

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Observational Studies Poster Presentation

P0890 - Neurologic manifestations of IgG4-related disease: A single-center case series (ID 1379)

Speakers
Presentation Number
P0890
Presentation Topic
Observational Studies

Abstract

Background

IgG4-related disease (IgG4-RD) is a multisystem disorder, which can affect nearly every organ system. Although involvement of the nervous system is commonly described, systematic descriptions of neurological involvement are rare.

Objectives

Our objective was to describe the characteristics of patients diagnosed with IgG4-RD presenting with neurologic involvement.

Methods

Patients with IgG4-RD were retrospectively identified by screening medical records of all patients, who had signed general consent, treated at the outpatient neurology clinic of our university hospital.

Results

Since 2012, we found 12 cases of IgG4-RD of whom eight cases presented with neurologic manifestations. Of those 6/8 (75%) patients were male. Mean age at symptom onset was 62 years (range: 35 - 81 years). In 2/8 (25%) of patients, neurologic presentation was the only disease manifestation and consisted of pachymeningitis, cranial neuropathy affecting cranial nerves II, V and VII, peripheral neuropathy, myalgia, carotid stenosis and cerebral ischemia. Lumbar puncture was performed in 5/8 (62%) patients and showed elevated cell count in 1 patient (range: 2- 9 M/l). Mean serum IgG4 concentration was 4.58 g/l (range 1.35 - 12.3 g/l) and was elevated in 7/8 patients. In the patient with normal IgG4 values biopsy was compatible with IgG4-RD. Total serum IgG was elevated in 4/8 (50%) patients with a mean of 14.9 g/l (range 8.92 – 21.6 g/l). Immunotherapy was started in 7/8 (87%) patients. Three patients received a high dose steroid monotherapy; other treatments were rituximab, methotrexate and leflunomide. Under these therapies, the outcome was favorable in 4/8 (50%) of patients, 3/8 (38%) remained unchanged and one patient succumbed to an unrelated pathology (colorectal cancer).

Conclusions

IgG4-related neurologic disease can manifest with multisystem involvement, but isolated neurologic manifestation is possible. If diagnosis is considered in patients with normal IgG4 levels biopsy should be obtained.

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