Clinical features of elderly-onset Adult-onset Still’s disease

Abstract

Objectives

To clarify the characteristics of patients with elderly-onset Adult-onset Still’s disease (AOSD).

Methods

Patients were classified into elderly-onset (>60 years: 47 patients) and younger-onset (≤60 years: 95 patients) groups according to their age at diagnosis of AOSD. Clinical features, treatments, and prognosis were compared between the elderly-onset and younger-onset groups.

Results

In the elderly-onset group, compared with the younger-onset group, typical skin rashes were less frequent (21.3% vs 58.9%, respectively; p < .0001), whereas pleuritis (27.7% vs 7.4%, respectively; p = .0011) and disseminated intravascular coagulation (19.1% vs 2.1%, respectively; p = .0004) were more frequent, and serum ferritin levels were higher (median 12,700 ng/ml vs 2526 ng/ml, respectively; p < .0001). Overall survival and AOSD-related survival were reduced (p = .0006 and p = .0023, respectively) and drug-free remission was less frequent (p = .0035) in the elderly-onset group compared with the younger-onset group.

Conclusions

Our results demonstrated that elderly-onset AOSD patients had several characteristics that differed from younger-onset AOSD patients, including less typical skin lesions, more AOSD-related complications, higher ferritin levels, and poorer prognoses.

Keywords:

• Adult-onset Still’s disease
• complication
• elderly-onset
• remission

Acknowledgements

The authors thank Kimiko Eguchi for secretarial assistance and Mark Abramovitz, PhD and J. Ludovic Croxford, PhD, from Edanz Group (www.edanzediting.com/ac) for editing a draft of this manuscript.

Conflict of interest

M. Iwamoto received royalties from Chugai and speaker’s fees from Sanofi and Lilly. Y. Tada received a speaker’s fee from Chugai.