TITLE:
Adult Onset Still’s Disease in Tropical Area: Illustration of Diagnostic and Therapeutic Difficulties from 3 Senegaleses Observations
AUTHORS:
Nafy Ndiaye, Ngoné Diaba Diack, Michel Assane Ndour, Biram Codou Fall, Ghislain De Chacus, Daouda Thioub, Ameth Dieng, Yakham Mohamed Leye, Abdoulaye Leye
KEYWORDS:
Still’s Disease, Diagnosis, Treatment, Tropical Area
JOURNAL NAME:
Open Journal of Internal Medicine,
Vol.7 No.4,
November
16,
2017
ABSTRACT: Introduction: The Adult Onset Still’s Disease
(ASD) is a systemic auto-inflammatory affection of unknown cause seldom described in sub-Saharan Africa. We report 3
observations of ASD illustrating the diagnostic and therapeutic difficulties of
this affection in our areas. Observation 1: Our first patient is a 56
years old schoolteacher presenting an ASD in its chronic articular form. She
had been followed for an inflammatory arthralgia for 10 years and of the
pharyngal pains without exact diagnosis. She presented ASD’s criteria of Yamaguchi
and of Fautrel. The prednisone was begun at the dose of 0.8 mg/kg/day with fast
appearance of a progressive muscular weakness. Use of methotrexate at a rate of
15 mg per week, associated with low dose of prednisone was effective in long-term without any flare of the disease so
far. Observation 2: Our second patient is a 30 years old
dressmaker presenting an ASD in its complicated systemic form of
lymphohistiocytic activation syndrome. She validated the criteria of Yamaguchi
and Fautrel for ASD. She also presented resistance to corticosteroid therapy. The evolution was marked by a
hospital-acquired septicemia and a multi-organ failure leading to death.
The diagnosis was retrospectively confirmed after that, with the low level of
the glycosylated ferritin serum value. Observation 3: The third patient
is a 22 years old Guinean student who presented prolonged fever and
inflammatory polyarthralgia without articular deformation. He had been
misdiagnosed for ASD with diagnostic wandering of several months. He was
treated successfully with prednisone after set up of ASD diagnosis according common criteria. Corticosteroid therapy was stopped after 8 months without any relapse noted so far. Conclusion: Caring for ASD is difficult in our context mainly because of high cost of
several explorations needed to set up its exact diagnosis while making
differential one. Evolution under
corticosteroid therapy is usually favorable but diagnostic delay may lead to severe complications and death.