ADULT ONSET STILL’S DISEASE – A RARE CASE REPORT
Author : Dr. Charanya sivakumar ,
M.D post graduate ,
Mahatma Gandhi medical college ,
Pondicherry – 607402.
ABSTRACT :
We report a 14 year old girl presented with fever for 45 days with polyarthralgia with a rash ,
who eventually turned out to be adult onset still disease .
CASE HISTORY :
A 14 yr old girl from Patna, Bihar came to our out patient department with complaint of
fever for 45 days , intermittent, high grade, not associated with chiils and rigors , double
quotidian pattern , associated with multiple bilateral joint swelling and tenderness , bilateral
symmetrical shoulder joints then elbow ,ankle and knee joints eventually involving all large and
small joints . patient gave a h/o of pink coloured rash over her chest for the past 15 days .
Patient had persistence of all the symptoms inspite of several antibiotic and analgesic treatment
prescribed in her home town for 40 days ,
No h/o any cough and expectoration , breathless, chest pain , weight loss, loss of appetite.
No h/o any loose stools , burning micturition .
No h/o any sore throat .
No h/o any similar complaints in the past .no h/o early morning stiffness .
Clinically ,
Patient was moderately built and moderately nourished , febrile with 103’F
Pulse rate : 104/min, regular , all the peripheral pulses felt,Blood pressure : 110/60 mm hg
,Respiratory rate : 25/min , thoraco abdominal ,JVP : not seen ,
Cardiovascular , respiratory , per abdominal , central nervous system examination were normal ,
Local examination : multiple large and small joint swelling and joint line tenderness noticed ,
without any deformities
A pinkish erythematous rash seen over the chest of the patient of size 3 x 4 cm ,
On Investigating,
Investigation
Values
Total count
13,900 cells /cu.mm
Differential count
Neutrophils 75%, lymphocytes 20% ,
eosinophils 5%.
Erythrocyte sedimentation rate
42 mm at ½ hour and 84 mm at 1 hour .
RBC
3.40 million/cu.mm
HB
9.1 gm %
PCV
30.4%
MCV
88.7 fl
MCH
26.8 pg
MCHC
30.1 g/dl
Platelet count
1.66 lakhs/cu.mm
Urine microscopy
Normal
HIV
Non reactive
Serum urea
29 mg %
Serum creatinine
0.9 mg %
Serum sodium
132 mEq/L
Serum potassium
4.9mEq/L
Serum chloride
101 mEq/L
Total protein
6.3 gm%
Serum albumin
3.6 gm %
A/G ratio
1.3:1
Bilirubin total
0.6 mg%
indirect
0.2 mg %
AST
37 U/L
ALT
55U/L
ALP
122 U/L
GGT
69 U/L
Peripheral smear
Normochromic normocytic anaemia with
neutrophils leucocytosis
Widal
1: 20 “O” antigen
1:20 “H” antigen (negative )
Test s for malaria
Negative
Thyroid profile
FT3
3.03 pg/ml
(2.0-4.4)
FT4
1.56 ng/ml
(0.9-1.7)
TSH
0.677micro IU/ml
(0.27-4.2)
Anti nuclear antibody
Negative
Anti DsDNA
Negative
ASO , RA factor , Anti CCP
Negative
Mantoux
Negative
Throat swab
No growth
Blood culture
Sterile
Urine culture
Sterile
Serum ferritin
3,051.9 ng /ml ( 10 -291 ng/ml)
Complement C3
1.91 (0.9- 1.8 g/dl)
Complement C4
0.33 (0.1-0.4 g /dl )
HIV
Non reactive
ECG
Normal
ECHO- Trans esophageal
Normal
Chest Xray , other joints x ray
Normal
USG abdomen
Normal
DISCUSSION :
Considering the long course of febrile illness with double quotidian fever with
polyarthralgia , sorethroat , hyperferriteinemia , raised acute phase reactants, with RA and ANA
being negative , therby fulfilling the eric bywaters , yagamuchi and cush criteria , adult onset
still’s disease was diagnosed .
Yamaguchi et al
Major
Diagnosis
Arthralgia > 2 weeks
5 criteria (Atleast 2 major)
Fever > 39 c ,
intermittent ,
> 1 week
Typical rash
Wbc > 10,000 ((>80% granulocytes )
Minor
Sore throat
Lymphadenopathy and / or
spleenomegaly
LFT abnormal
Negative ANA and RF
Adult-Onset Still's Disease (AOSD) is an immune-mediated systemic disease
with quotidian-spiking fever, rash, and inflammatory arthritis.
Eric bywaters in 1971 described the term adult onset still s disease while
working on chronic polyarthralgias between the year 1950 and 1970 and coined a criteria for
diagnosis . Fourteen cases are described of an illness starting in adult life resembling still s
disease or the sero negative chronic polyarthritis of children . characteristic features were fever ,
rash , polyarthritis and raised acute phase reactants with normal antinuclear antibody and
rheumatoid factor (1) all the fourteen cases described by eric bywaters were female .
The typical rash is an evanescent, salmon-pink, maculopapular eruption,
predominantly found on the proximal limbs and trunk,rarely over the face and distal limbs.may
be pruritic when accompanied with rash .(2) . Common haematological abnormalities include
leukocytosis, , anaemia, and thrombocytosis which is often accompanied by increased disease
activity. Leucocytosis is the result of a striking neutrophilia that is probably secondary to bone
marrow granulocyte hyperplasia. Hyperferritinemia is a prominent feature, often used for
screening(5).
IL-1β found to play a major role by upregulating cytokines, acute phase
proteins, and tissue remodeling enzymes. It forms an impartant part of innate immunity and is
a potent pyrogen and facilitates neutrophilic proliferation and diapedesis into inflamed tissues.
In the periphery, IL-1β, along with TNF,results in bone and cartilage erosion also increasing
platelet production, which results in thrombocytosis, and increases the production of IL-6, which
in turns stimulates hepatocytes to synthesize several acute phase proteins.(3)
Treatment was started with aspirin 650 mg TID, along with proton pump inhibitors
, the next day response was dramatic , with patient being afebrile after 45 days with resolution
of the joint symptoms , if aspirin intolerance is suspected patient could be started on second
line drug , methotrexate(4)or to a biological agent like IL-1 Receptor antagonist (Anakinra)(3) or
TNF inhibitors (infliximab >etanercept(3) or anti IL-6 (tocilizumab )
.
We report this case of adult onset still’s disease which improved well with
Aspirin with fall in serum ferritin and other acute phase reactants post treatment and after 6
months during follw up ..
REFERENCES :
(1) Bywaters EG. Still’s disease in the adult. Ann Rheum Dis 1971;30:121–33.
(2) Efthimiou P; Paik PK; Bielory L, Diagnosis and management of adult onset Still's
disease. Annals of the rheumatic diseases. 2006 65(5):564-72.
(3) Giampietro, Cecilia; Fautrel, Bruno. Anti-Interleukin-1 Agents in Adult Onset Still's
Disease. International journal of inflammation. 2012 2012:317820.
(4) Fautrel, Bruno. Adult-onset Still disease. Best practice & research. Clinical
rheumatology. 2008 22(5):773-92.
(5) Mehta, Bella; Efthimiou, Petros. Ferritin in Adult-Onset Still's Disease: Just a Useful
Innocent Bystander?. International journal of inflammation. 2012 2012:298405.