Macrophage activation syndrome, a rare complication of primary Sjögren's syndrome: a case report.
Africa south of the Sahara
Case report
Macrophage activation syndrome
Sjögren’s syndrome
Journal
Journal of medical case reports
ISSN: 1752-1947
Titre abrégé: J Med Case Rep
Pays: England
ID NLM: 101293382
Informations de publication
Date de publication:
16 Oct 2019
16 Oct 2019
Historique:
received:
23
11
2018
accepted:
03
09
2019
entrez:
17
10
2019
pubmed:
17
10
2019
medline:
6
2
2020
Statut:
epublish
Résumé
The association of macrophage activation syndrome and primary Sjögren's syndrome has been rarely reported in the literature. We report the first observation of this association in Africa, south of the Sahara, and we discuss the diagnosis and therapeutic challenge. A 26-year-old Mauritanian and Berber woman was followed for primary Sjögren's syndrome. After a voluntary cessation of her usual background treatment, she was admitted to our department for an outbreak of her illness. A clinical examination revealed anemic syndrome, peripheral polyarthritis, coughing rales at both pulmonary bases, and fever at 39.5 °C. On biologic examination, there was bicytopenia with anemia at 5.70 g/dl, lymphopenia at 796/mm Macrophage activation syndrome is a rare entity, rarely reported during primary Sjögren's syndrome. Its spontaneous evolution is invariably fatal. There is no consensus on therapeutic treatment. Etoposide is a therapeutic option especially in forms refractory to corticosteroid therapy.
Sections du résumé
BACKGROUND
BACKGROUND
The association of macrophage activation syndrome and primary Sjögren's syndrome has been rarely reported in the literature. We report the first observation of this association in Africa, south of the Sahara, and we discuss the diagnosis and therapeutic challenge.
CASE PRESENTATION
METHODS
A 26-year-old Mauritanian and Berber woman was followed for primary Sjögren's syndrome. After a voluntary cessation of her usual background treatment, she was admitted to our department for an outbreak of her illness. A clinical examination revealed anemic syndrome, peripheral polyarthritis, coughing rales at both pulmonary bases, and fever at 39.5 °C. On biologic examination, there was bicytopenia with anemia at 5.70 g/dl, lymphopenia at 796/mm
CONCLUSION
CONCLUSIONS
Macrophage activation syndrome is a rare entity, rarely reported during primary Sjögren's syndrome. Its spontaneous evolution is invariably fatal. There is no consensus on therapeutic treatment. Etoposide is a therapeutic option especially in forms refractory to corticosteroid therapy.
Identifiants
pubmed: 31615546
doi: 10.1186/s13256-019-2252-z
pii: 10.1186/s13256-019-2252-z
pmc: PMC6794887
doi:
Substances chimiques
Antineoplastic Agents, Phytogenic
0
Etoposide
6PLQ3CP4P3
Ferritins
9007-73-2
Types de publication
Case Reports
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
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