IgG4-related chronic sclerosing sialadenitis in a child with recurrent parotitis: a case report.
Case report
Chronic sclerosing sialadenitis
IgG4-related disease
Juvenile recurrent parotitis
Journal
BMC pediatrics
ISSN: 1471-2431
Titre abrégé: BMC Pediatr
Pays: England
ID NLM: 100967804
Informations de publication
Date de publication:
20 12 2021
20 12 2021
Historique:
received:
04
03
2021
accepted:
13
11
2021
entrez:
21
12
2021
pubmed:
22
12
2021
medline:
27
1
2022
Statut:
epublish
Résumé
IgG4-related disease (IgG4-RD) includes a group of immune-mediated diseases histologically characterized by lymphoplasmacytic infiltrate with a prevalence of IgG4-positive plasma cells, storiform fibrosis and obliterative phlebitis. Autoimmune pancreatitis, sialadenitis, dacryoadenitis and retroperitoneal fibrosis are the most frequent manifestations. IgG4-related sialadenitis usually affects submandibular glands and is very rare in children. Here we report the case of IgG4-related sialadenitis in a six-year-old patient previously diagnosed as juvenile recurrent parotitis. A six-year-old patient was referred to our Centre for left parotid swelling of 4 × 3 cm, that was tender, soft in consistency, with overlying red and warm skin. His general condition was good but he was subfebrile; general examination revealed mild enlargement of left cervical lymph nodes. In the last 2 years he had had five episodes of parotitis, diagnosed by another pediatric Center as juvenile recurrent parotitis. On ultrasound examination the left parotid gland appeared enlarged, inhomogeneous, with a colliquative intraparotid lymph node and no evidence of sialolithiasis. Laboratory tests showed an increase of white blood cells and anti-VCA IgM and IgG positivity, with anti-EBNA e anti-EA I negativity. The patient was initially treated with oral antibiotics, but after 10 days the parotid became fluctuating, requiring surgical biopsy and drainage. Postoperative course was regular, with complete remission under oral antibiotic and steroid therapy. Microbiological tests, including cultures for aerobic and anaerobic bacteria, mycobacteria and Bartonella, were negative. Surprisingly, histology showed marked fibrosis and histiocytic and lymphoplasmacellular infiltrate with polyclonal plasma cells mostly expressing IgG4 immunoglobulins. Thus, the diagnosis of IgG4 related chronic sialadenitis in recurrent parotitis and recent EBV infection was made. IgG4-related sialadenitis is very unusual in children. Histology plays a key role in diagnosis, considering that up to 30% of patients have normal serum IgG4 levels, as shown in our case. The lack of previous histological data makes it impossible to attribute our patient's previous episodes of parotitis to IgG4-RD, though it is a very consistent possibility.
Sections du résumé
BACKGROUND
IgG4-related disease (IgG4-RD) includes a group of immune-mediated diseases histologically characterized by lymphoplasmacytic infiltrate with a prevalence of IgG4-positive plasma cells, storiform fibrosis and obliterative phlebitis. Autoimmune pancreatitis, sialadenitis, dacryoadenitis and retroperitoneal fibrosis are the most frequent manifestations. IgG4-related sialadenitis usually affects submandibular glands and is very rare in children. Here we report the case of IgG4-related sialadenitis in a six-year-old patient previously diagnosed as juvenile recurrent parotitis.
CASE PRESENTATION
A six-year-old patient was referred to our Centre for left parotid swelling of 4 × 3 cm, that was tender, soft in consistency, with overlying red and warm skin. His general condition was good but he was subfebrile; general examination revealed mild enlargement of left cervical lymph nodes. In the last 2 years he had had five episodes of parotitis, diagnosed by another pediatric Center as juvenile recurrent parotitis. On ultrasound examination the left parotid gland appeared enlarged, inhomogeneous, with a colliquative intraparotid lymph node and no evidence of sialolithiasis. Laboratory tests showed an increase of white blood cells and anti-VCA IgM and IgG positivity, with anti-EBNA e anti-EA I negativity. The patient was initially treated with oral antibiotics, but after 10 days the parotid became fluctuating, requiring surgical biopsy and drainage. Postoperative course was regular, with complete remission under oral antibiotic and steroid therapy. Microbiological tests, including cultures for aerobic and anaerobic bacteria, mycobacteria and Bartonella, were negative. Surprisingly, histology showed marked fibrosis and histiocytic and lymphoplasmacellular infiltrate with polyclonal plasma cells mostly expressing IgG4 immunoglobulins. Thus, the diagnosis of IgG4 related chronic sialadenitis in recurrent parotitis and recent EBV infection was made.
CONCLUSIONS
IgG4-related sialadenitis is very unusual in children. Histology plays a key role in diagnosis, considering that up to 30% of patients have normal serum IgG4 levels, as shown in our case. The lack of previous histological data makes it impossible to attribute our patient's previous episodes of parotitis to IgG4-RD, though it is a very consistent possibility.
Identifiants
pubmed: 34930210
doi: 10.1186/s12887-021-03004-4
pii: 10.1186/s12887-021-03004-4
pmc: PMC8691005
doi:
Substances chimiques
Immunoglobulin G
0
Types de publication
Case Reports
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
586Informations de copyright
© 2021. The Author(s).
Références
Ann Rheum Dis. 2015 Jan;74(1):14-8
pubmed: 24651618
Am J Surg Pathol. 2010 Feb;34(2):202-10
pubmed: 20061932
Int J Pediatr Otorhinolaryngol. 2016 Apr;83:22-4
pubmed: 26968047
Mod Pathol. 2012 Sep;25(9):1181-92
pubmed: 22596100
Arch Dis Child. 1997 Oct;77(4):359-63
pubmed: 9389246
Acta Paediatr. 2013 Feb;102(2):177-81
pubmed: 22924783
Pediatr Allergy Immunol. 2004 Jun;15(3):281-3
pubmed: 15209964
Am J Surg Pathol. 2014 Jul;38(7):946-55
pubmed: 24705309
Mod Rheumatol. 2008;18(1):86-90
pubmed: 18094933
Pediatr Dev Pathol. 2012 Mar-Apr;15(2):165-9
pubmed: 21985393
Arthritis Rheum. 2012 Oct;64(10):3061-7
pubmed: 22736240
J Gastroenterol. 2008;43(6):409-18
pubmed: 18600384
Lancet. 2015 Apr 11;385(9976):1460-71
pubmed: 25481618
Hepatology. 2007 Jun;45(6):1538-46
pubmed: 17518371
Gut. 2009 Nov;58(11):1504-7
pubmed: 19398440
Acta Paediatr. 2005 Nov;94(11):1566-70
pubmed: 16303695
Eur J Microbiol Immunol (Bp). 2014 Jun;4(2):144-6
pubmed: 24883201
Pediatr Rheumatol Online J. 2016 Mar 25;14(1):18
pubmed: 27012661
Am J Surg Pathol. 2010 Dec;34(12):1812-9
pubmed: 21107087
Viral Immunol. 2018 Oct;31(8):540-547
pubmed: 30222515
J Gastroenterol. 2014 Jun;49(6):961-70
pubmed: 24639058
Gut. 2017 Mar;66(3):487-494
pubmed: 27543430
Mod Rheumatol. 2012 Feb;22(1):21-30
pubmed: 22218969
Int J Rheum Dis. 2016 Aug;19(8):747-62
pubmed: 26259069
Auris Nasus Larynx. 2012 Feb;39(1):9-17
pubmed: 21571468
J Clin Pathol. 2011 Mar;64(3):237-43
pubmed: 21233087
J Autoimmun. 2012 Aug;39(1-2):93-6
pubmed: 22341851
Int J Pediatr Otorhinolaryngol. 2018 Sep;112:151-157
pubmed: 30055724
Gut. 2013 Nov;62(11):1607-15
pubmed: 22936672
Am J Surg Pathol. 2007 Oct;31(10):1586-97
pubmed: 17895762