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

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 pres...

Full description

Saved in:
Bibliographic Details
Published in:Rheumatology international Vol. 40; no. 2; pp. 337 - 343
Main Authors: Vakrakou, Aigli G., Evangelopoulos, Maria-Eleptheria, Boutzios, Georgios, Tzanetakos, Dimitrios, Tzartos, John, Velonakis, Georgios, Toulas, Panagiotis, Anagnostouli, Maria, Andreadou, Elissavet, Koutsis, Georgios, Stefanis, Leonidas, Fragoulis, George E., Kilidireas, Constantinos
Format: Journal Article
Language:English
Published: Berlin/Heidelberg Springer Berlin Heidelberg 01.02.2020
Springer Nature B.V
Subjects:
Adolescent
Asymptomatic Diseases
Autoimmune Hypophysitis - diagnostic imaging
Autoimmune Hypophysitis - drug therapy
Autoimmune Hypophysitis - immunology
Autoimmune Hypophysitis - physiopathology
Azathioprine - therapeutic use
Case Based Review
Cervical Vertebrae
Female
Glucocorticoids - therapeutic use
Humans
Hypesthesia - physiopathology
Immunoglobulin G - immunology
Immunoglobulin G4-Related Disease - diagnostic imaging
Immunoglobulin G4-Related Disease - drug therapy
Immunoglobulin G4-Related Disease - immunology
Immunoglobulin G4-Related Disease - physiopathology
Immunomodulators
Immunosuppressive Agents - therapeutic use
Inflammatory diseases
Magnetic Resonance Imaging
Medicine
Medicine & Public Health
Myelitis - diagnostic imaging
Myelitis - drug therapy
Myelitis - immunology
Myelitis - physiopathology
Paresthesia - physiopathology
Pulse Therapy, Drug
Recurrence
Rheumatology
Spinal cord
Magnetic resonance imaging (MRI)
IgG4-related disease
Longitudinally extensive transverse myelitis (LETM)
Hypophysitis
ISSN:0172-8172, 1437-160X, 1437-160X
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary: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.
Bibliography:ObjectType-Article-1
SourceType-Scholarly Journals-1
ObjectType-Feature-2
content type line 14
ObjectType-Case Study-2
ObjectType-Review-5
ObjectType-Feature-4
content type line 23
ObjectType-Report-1
ObjectType-Article-3
ISSN:0172-8172
1437-160X
1437-160X
DOI:10.1007/s00296-019-04502-6