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2016 ; 2016
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Autoimmune polyendocrinopathy and hypophysitis after Puumala hantavirus
infection
#MMPMID27857837
Tarvainen M
; Mäkelä S
; Mustonen J
; Jaatinen P
Endocrinol Diabetes Metab Case Rep
2016[]; 2016
(ä): ä PMID27857837
show ga
Puumala hantavirus (PUUV) infection causes nephropathia epidemica (NE), a
relatively mild form of haemorrhagic fever with renal syndrome (HFRS).
Hypophyseal haemorrhage and hypopituitarism have been described in case reports
on patients with acute NE. Chronic hypopituitarism diagnosed months or years
after the acute illness has also been reported, without any signs of a
haemorrhagic aetiology. The mechanisms leading to the late-onset hormonal defects
remain unknown. Here, we present a case of NE-associated autoimmune
polyendocrinopathy and hypopituitarism presumably due to autoimmune hypophysitis.
Thyroid peroxidase antibody seroconversion occurred between 6 and 12 months, and
ovarian as well as glutamate decarboxylase antibodies were found 18 months after
acute NE. Brain MRI revealed an atrophic adenohypophysis with a heterogeneous,
low signal intensity compatible with a sequela of hypophysitis. The patient
developed central (or mixed central and peripheral) hypothyroidism, hypogonadism
and diabetes insipidus, all requiring hormonal replacement therapy. This case
report suggests that late-onset hormonal defects after PUUV infection may develop
by an autoimmune mechanism. This hypothesis needs to be confirmed by prospective
studies with sufficient numbers of patients. LEARNING POINTS: Pituitary
haemorrhage resulting in hypopituitarism has been reported during acute HFRS
caused by PUUV and other hantaviruses.Central and peripheral hormone deficiencies
developing months or years after HFRS have also been found, with an incidence
higher than that in the general population. The pathogenesis of these late-onset
hormonal defects remains unknown.This case report suggests that the late-onset
hypopituitarism and peripheral endocrine defects after HFRS could evolve via
autoimmune mechanisms.The sensitivity of current anti-pituitary antibody (APA)
tests is low. A characteristic clinical course, together with typical brain MRI
and endocrine findings may be sufficient for a non-invasive diagnosis of
autoimmune hypophysitis, despite negative APAs.