Clinical Features and Prognosis of Adult-onset Still’s Disease: 61 Cases from China

Abstract

Patients with AOSD had complex symptoms with no specific laboratory findings. Our results indicate that AOSD is not a relatively benign disease, especially in cases that are refractory to high doses of prednisone.

High Serum Ferritin in Adult-Onset Still's Disease

“…Disease activity correlates relatively well with the serum ferritin level which often normalises during remission [6]. In addition, serum ferritin might serve as a prognostic marker in AOSD [7].…”

Adult-onset Still disease: a rare disorder with a potentially fatal outcome

“…DIC is one of the most dramatic events in AOSD and is a possible cause of death [2,18,20,22,28] although complete recovery in several patients has been reported [2,14,22,35,42,[45][46][47][48][49][50]. It can follow or be associated with reactive haematophagocytic syndrome (RHS), a further possible life-threatening complication in AODS [15,17,21,22,43,51]. This event is characterized by an uncontrolled and sustained activation of macrophages associated with impairment of cytotoxic function with a true cytokine storm [22].…”

“…Moreover, hepatomegaly, splenomegaly and lymphoadenopathy can be seen [12,13]. Although mortality in AOSD is quite low, a fatal outcome has been reported in some patients [2,[14][15][16][17][18][19][20][21][22][23][24][25][26][27][28].…”

Pneumonitis in Adult Onset Still’s Disease: Uncommon or Under Diagnosed?

“…2 The prevalence of lung involvement varies according to the sample and the structure of the published studies, and pleuritis is the most common manifestation. 3,4 Although less often considered, pneumonitis deserves particular emphasis given the need for differential diagnosis and because it can progress to severe respiratory failure. 3 In order to highlight the importance of pulmonary parenchyma involvement in AOSD, we describe a case report that progresses with pneumonitis.…”

“…3 However, pulmonary complications that threaten the patient's life, such as the syndrome of acute adult respiratory distress and diffuse alveolar hemorrhage, are rare. 4,6,7 When present, the most frequent respiratory symptoms are cough, pleuritic chest pain and mild dyspnea. Pleuritis is the most common pulmonary manifestation, and can be present in up to 53% of patients.…”