Vol.:(0123456789) 1 3 Rheumatology International https://doi.org/10.1007/s00296-019-04502-6 CASE BASED REVIEW Recurrent myelitis and asymptomatic hypophysitis in IgG4‑related disease: case‑based review Aigli G. Vakrakou 1  · Maria‑Eleptheria Evangelopoulos 1  · Georgios Boutzios 2  · Dimitrios Tzanetakos 1  · John Tzartos 1  · Georgios Velonakis 1  · Panagiotis Toulas 1  · Maria Anagnostouli 1  · Elissavet Andreadou 1  · Georgios Koutsis 1  · Leonidas Stefanis 1  · George E. Fragoulis 3  · Constantinos Kilidireas 1 Received: 15 October 2019 / Accepted: 11 December 2019 © Springer-Verlag GmbH Germany, part of Springer Nature 2020 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 frst 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 imag- ing (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 fulflled the organ-specifc diagnostic criteria of IgG4-hypophysitis. Treatment with intravenous glucocorticoids led to rapid clini- cal response, and to the substantial resolution of imaging fndings. 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 frst case of myelitis with radiological features of LETM associated with increased IgG4 serum levels and the simultaneous presence of asymptomatic IgG4-related hypophysitis. Keywords IgG4-related disease · Longitudinally extensive transverse myelitis (LETM) · Hypophysitis · Magnetic resonance imaging (MRI) Rheumatology INTERNATIONAL * Aigli G. Vakrakou avakrakou@med.uoa.gr Maria-Eleptheria Evangelopoulos eandread@med.uoa.gr Georgios Boutzios gboutzios@med.uoa.r Dimitrios Tzanetakos dtzanetakos@med.uoa.gr John Tzartos jtzartos@gmail.com Georgios Velonakis giorvelonakis@gmail.com Panagiotis Toulas ptoulas@yahoo.gr Maria Anagnostouli managnost@med.uoa.gr Elissavet Andreadou eandread@med.uoa.gr Georgios Koutsis gkoutsis@med.uoa.gr Leonidas Stefanis lstefanis@med.uoa.gr George E. Fragoulis geofragoul@yahoo.gr Constantinos Kilidireas kildrcost@med.uoa.gr 1 1st Department of Neurology, Medical School of Athens, National and Kapodistrian University, Aeginition Hospital, Athens, Greece 2 Endocrine Unit, Department of Pathophysiology, Laikon General Hospital, University of Athens Medical School, Athens, Greece 3 First Department of Propaedeutic Internal Medicine, National and Kapodistrian University of Athens, “Laiko” General Hospital, Athens, Greece