Diagnosis and classification of adult Still's disease

Adult onset Still disease is an unusual multisystem inflammatory disorder of unknown etiology and pathogenesis. Among its protean manifestations is pericarditis which occurs in approximately 30-40% of patients. Cardiac tamponade is a rare complication of pericarditis occurring in the context of Still disease. We conducted a review of the English literature pertaining to the occurrence of pericardial tamponade in Still disease. We searched relevant journals and the PubMed Medline databases. A total of eighteen reported cases were identified; ten among children and eight in adults. Treatment of pericardial tamponade was medical therapy alone in two cases versus combined modality, medical and surgical drainage in the remainder of the cases. Outcomes were death among four of the ten children and there was no recurrence of tamponade in the remaining child and adult series. This review re-emphasizes the importance of considering Still disease in the differential diagnosis of the life-threatening emergency of pericardial tamponade.

Cardiac tamponade in Still disease: a review of the literature.

BACKGROUND Adult-onset Still's Disease (AOSD) is a rare systemic inflammatory disease accompanied by a triad of spiking fever, maculopapular exanthema, and arthralgia. To date, there is no definite laboratory or imaging test available for diagnosing AOSD, and the diagnosis is one of exclusion, which can be very challenging. CASE REPORT We report on the case of a 53-year-old female who presented with fever, arthralgia, and abdominal pain. Her initial laboratory tests showed elevated AST and ALT, and normal leukocytes with bandemia. During her hospitalization, we evaluated the patient for other potential differential diagnoses. After an extensive workup, the patient was diagnosed with AOSD based on Yamaguchi criteria. Her serum ferritin levels were measured and found to be markedly elevated, which is a non-specific finding in AOSD patients. CONCLUSIONS This case highlights the important role of a detailed history and physical examination for timely diagnosis of AOSD to prevent complications and improve patient's prognosis.

/pdf/adult-onset-still-s-disease-still-a-serious-health-problem-a-1le4i7l5fb.pdf

Adult-Onset Still's Disease: Still a Serious Health Problem (a Case Report and Literature Review).

Resume Introduction La maladie de Still de l'adulte est une maladie systemique inflammatoire de cause inconnue qui associe classiquement une fievre elevee hectique, une eruption cutanee evanescente, une atteinte articulaire, et une hyperleucocytose a polynucleaires neutrophiles. Parmi les nombreuses manifestations systemiques rapportees, la pericardite est l'une des plus frequentes. Exegese Nous rapportons deux observations de tamponnade revelant une maladie de Still de l'adulte, chez une femme de 52 ans et un homme de 31 ans. Le liquide pericardique etait serohematique dans les deux cas, et l'histologie du pericarde ne montrait qu'une inflammation aigue non specifique. Les deux patients ont ete traites par corticoides et ont eu une evolution favorable sans recidive de l'epanchement pericardique avec un suivi respectif de huit ans et 12 mois. Conclusion La tamponnade est une complication exceptionnelle de la maladie de Still de l'adulte, et le plus souvent inaugurale. Elle rend le diagnostic difficile car elle risque de faire meconnaitre les autres manifestations de la maladie. La maladie de Still de l'adulte doit etre rajoutee a la liste des diagnostics a evoquer devant une pericardite aigue ou une tamponnade.

Tamponnade révélant une maladie de Still de l'adulte : à propos de deux observations

Resume La maladie de Still de l'adulte est un rhumatisme inflammatoire rare. Il se caracterise par l'association de signes evocateurs, tels qu'une fievre superieure a 39°C, une eruption cutanee lors des pics de fievre, des douleurs articulaires et/ou musculaires, des douleurs pharyngees, une augmentation de volume des ganglions, une augmentation des globules blancs (principalement des polynucleaires neutrophiles) et une perturbation du foie. D'autres symptomes sont egalement possibles. Aucun des signes decrits ne permet de certifier le diagnostic, et plusieurs autres maladies (notamment infectieuses et neoplasiques) peuvent s'exprimer par des symptomes proches. De ce fait, il s'agit tres souvent de ce que l'on appelle un « diagnostic d'exclusion », que l'on retient lorsque les autres maladies possibles ont ete eliminees. L'evolution de la maladie est difficilement previsible ; elle peut se resumer a une poussee et puis guerir, ou durer plusieurs mois ou annees. Des erosions articulaires peuvent apparaitre dans environ 1/3 des cas. Grâce aux traitements, il est possible : de limiter les symptomes de la maladie, c'est le role de l'aspirine et des anti-inflammatoires (AINS), qui sont prescrits en premiere intention, notamment au debut de la maladie, avant que le diagnostic ne soit definitivement retenu ; de controler l'evolution de la maladie, il peut s'agir de corticoides, du methotrexate, de la ciclosporine A, voire des immunoglobulines intraveineuses ou d'agents inhibiteurs de TNF alpha tels Etanercept ou Infliximab.

https://www.orpha.net/data/patho/Pro/fr/Still-FRfrPro5525.pdf

Maladie de Still de l'adulte

Pericarditis is a common manifestation of adult still's diseases (ASD), observed in 20% to 3% cases. Pericardial tamponade is more seldom, less than ten cases have been reported in the literature. We reported two observations of patients with ASD and pericardial tamponade above a series of 18 patients with ASD, in one case revealed the disease. The first patient was a 32-year-old women and had fever, arthritis, high white blood cell count and developed pericardial tamponade. The evolution was favourable with bolus of methylprednisolone. In the second case, cardiac tamponade occurred several years after the onset of the disease. Surgical drainage was first required (800 ml) because of inefficacity of oral prednisone. A dramatic improvement was observed after bolus of methylprednisolone. In conclusion pericardial tamponade is a seldom but sometimes the first manifestation of ASD and may required in first intention bolus of methylprednisolone.

Complications cardiaques de la maladie de Still de l'adulte : de la péricardite à la tamponade parfois révélatrice

Cardiac tamponade is very seldom complication of adult onset-Still's disease. We reported 2 cases; it was the first manifestation of the disease in one case.

[Cardiac complication of adult-onset Still's disease: from pericarditis to tamponade, sometimes a manifestation of the disease].

The present study emphasizes the possible complication of adult onset-Still's disease by psoriatic-arthropathy of the hands (3 cases in a retrospective study of 18 patients).

Arthropathies digitales: une complication exceptionnelle de la maladie de still

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Complications cardiaques de la maladie de Still de l'adulte : de la péricardite à la tamponade parfois révélatrice

[Cardiac complication of adult-onset Still's disease: from pericarditis to tamponade, sometimes a manifestation of the disease].

Arthropathies digitales: une complication exceptionnelle de la maladie de still