Article

[Adult-onset Still's disease revealed by a pericardial tamponade: report of two cases].

Service de Médecine Interne, Hôpital La-Rabta, 1007 Tunis, Tunisie.
La Revue de Médecine Interne (Impact Factor: 0.9). 08/2006; 27(7):546-9. DOI: 10.1016/j.revmed.2006.03.027
Source: PubMed

ABSTRACT Adult onset Still's disease is a systemic inflammatory disorder of unknown etiology characterized by the association of a high spiking fever, an evanescent skin rash, arthritis, and hyperleukocytosis. Pericarditis is amongst the most common systemic manifestations of adult onset Still's disease.
We report on two patients with a pericardial tamponade revealing an adult onset Still's disease in a 52-year-old female and a 31-year-old male. Pericardial fluid was bloody in the two cases, and histopathology only disclosed non specific inflammatory changes. Both patients received corticosteroids and outcome was uneventful with a follow-up of 8 years and 12 months, respectively.
Pericardial tamponade is an uncommon clinical feature of adult-onset Still's disease and usually occurs at disease onset. It makes the diagnosis of adult-onset Still's disease difficult as the other disease manifestations are commonly neglected. Adult onset Still's disease should be added to the differential of acute pericarditis and tamponade.

1 Bookmark
 · 
94 Views
  • [Show abstract] [Hide abstract]
    ABSTRACT: Cardiopulmonary involvement in adult-onset Still's disease is not as common as cutaneous and articular involvement. Pleuropericarditis is the most frequent thoracic manifestation. Although difficult, diagnosis of other thoracic manifestations, which may reveal the disease, is crucial, due to the high risk to life and the efficacy of new immunosuppressive agents. The pathophysiology involves essentially immunological factors, Still's disease being increasingly seen as an autoimmune inflammatory disease. Pro-inflammatory cytokines such as interleukine (IL) 1, 6 and 18 play a crucial role in macrophage activation, which is central in the pathophysiology of adult Still's disease. The classification of cardiopulmonary manifestations is based on anatomy. Cardiac lesions may involve all the tissues of the heart and the pulmonary arteries. Respiratory lesions may involve the pleura, the lung parenchyma (organizing pneumonitis, infiltrative lung disease, alveolar damage, amyloidosis), and the respiratory muscles, including the diaphragm. Finally, some manifestations may be provoked by the treatment itself. Steroids, the first-line treatment, are very effective in pleuropericarditis. Methotrexate used to be prescribed when steroids failed, but biotherapies such as IL1 and IL6 inhibitors have transformed the prognosis of forms resistant to these drugs.
    Revue des Maladies Respiratoires 04/2013; 30(4):262-71. · 0.50 Impact Factor
  • Source
    Annales francaises d'anesthesie et de reanimation 01/2013; 32(1):65. · 0.77 Impact Factor
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Adult Still's disease may present with multiple non-specific clinical manifestations leading to diagnostic difficulties. Presentation as initial myopericarditis is rare but must be known. We report a 49-year-old man who presented with the sudden onset of a high fever, arthralgia and myalgia, skin rash, dyspnea and chest pain related to a myopericarditis. Infectious, neoplastic and autoimmune diagnostic work-up was non-contributive. Adult Still's disease was discussed and confirmed by the decrease in the glycosylated fraction of ferritin. Corticosteroids followed by interleukin-1 receptor antagonist (anakinra) therapy allowed a rapid improvement in clinical and biological manifestations. Adult Still's disease should be considered in any systemic inflammatory disease without a diagnosis, even with atypical manifestations, especially since it can be life-threatening in the absence of treatment.
    La Revue de Médecine Interne 12/2013; · 0.90 Impact Factor

Full-text

View
144 Downloads
Available from
May 21, 2014