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Recurrent myelitis and asymptomatic hypophysitis in IgG4-related disease: case-based review

Abstract

IgG4-related disease (IgG4-RD) is a disorder with various clinical manifestations. Central nervous system (CNS) involvement is well recognized, with hypertrophic pachymeningitis and hypophysitis being the most common manifestations. Spinal cord involvement is an extremely rare manifestation. We present the first case of an IgG4-RD patient with spinal cord parenchymal disease and concurrent hypophysitis. We review also the current literature about CNS parenchymal involvement in the context of IgG4-RD. A young female presented with clinical symptoms of myelitis. Cervical spinal cord magnetic resonance imaging (MRI) displayed features of longitudinally extensive transverse myelitis (LETM). Brain MRI showed a small number of high-intensity lesions in the deep white matter and enlargement of hypophysis with homogeneous gadolinium enhancement (asymptomatic hypophysitis). Diagnostic workup revealed elevated IgG4 serum levels (146 mg/dL). Our patient fulfilled the organ-specific diagnostic criteria of IgG4-hypophysitis. Treatment with intravenous glucocorticoids led to rapid clinical response, and to the substantial resolution of imaging findings. Azathioprine was used as a maintenance treatment. One relapse occurred 2 years after the initial diagnosis and patient was re-treated with glucocorticoids. Three years after relapse, patient is in remission with azathioprine. We present the first case of myelitis with radiological features of LETM associated with increased IgG4 serum levels and the simultaneous presence of asymptomatic IgG4-related hypophysitis.

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Abbreviations

IgG4-RD:

IgG4-related disease

CNS:

Central nervous system

MOG:

Myelin oligodendrocyte glycoprotein

CSF:

Cerebrospinal fluid

NMOSD:

Neuromyelitis optica spectrum disorders

LETM:

Longitudinally extensive transverse myelitis

AZA:

Azathioprine

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Acknowledgements

The authors express their gratitude to the patient for allowing her data to be used for research purposes.

Funding

No funding was obtained for the preparation of this case report.

Author information

All authors listed provided substantial contributions to conception and design, or acquisition, analysis and interpretation of data, and contributed in drafting or revising the manuscript in a critical manner for important intellectual content. All authors read and approved the final version of the manuscript and agreed to be accountable for all aspects of the work ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.

Correspondence to Aigli G. Vakrakou.

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Author GE Fragoulis has received travelling Grants from Abbvie. All the other authors declare that they have no conflict of interests.

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Vakrakou, A.G., Evangelopoulos, M., Boutzios, G. et al. Recurrent myelitis and asymptomatic hypophysitis in IgG4-related disease: case-based review. Rheumatol Int 40, 337–343 (2020). https://doi.org/10.1007/s00296-019-04502-6

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Keywords

  • IgG4-related disease
  • Longitudinally extensive transverse myelitis (LETM)
  • Hypophysitis
  • Magnetic resonance imaging (MRI)