Adult-onset Still disease
ICD-10 M06.1 · ICD-11 FA23

Treatment of Adult-onset Still Disease with Lung Disease or Down Syndrome — Managing Pulmonary Risk

Clinical Scenario

This protocol applies to patients with adult-onset Still disease (AOSD) who have developed Still's disease-related lung disease, or who carry recognised risk factors for it. The at-risk population includes patients with Down syndrome, younger age at disease onset, a history of macrophage activation syndrome (particularly recurrent MAS), high serum IL-18 levels, and carriage of HLA-DRB1*1501.

Pulmonary Surveillance

Lung disease should be actively screened. Clinical features to monitor include:

Pulmonary function tests (pulse oximetry, DLCO measurement) and high-resolution CT scan are indicated in any patient with clinical symptoms.

Treatment Approach (Partial Summary)

The approach centres on IL-1 or IL-6 inhibitor therapy, which is not contraindicated by lung disease or its risk factors; whether additional immunosuppressive measures are warranted depends on individual risk assessment.

The complete regimen, decision criteria, and sequencing are available in the structured protocol below.

Instant Access to Structured Evidence-Based Regimens
References
DOI: 10.1136/ard-2024-225851
  1. LD appears to be associated with younger age at onset, Down's syndrome, occurrence of MAS and particularly of recurrent MAS, as well as high serum IL-18 levels.
  2. Lung disease should be actively screened by search for clinical symptoms (eg, clubbing, persistent cough, shortness of breath) and pulmonary function tests (pulse oximetry, DLCO measurement), and investigated by high resolution CT scan in any patients with clinical symptoms.
  3. Based on the available data, the presence of risk factors for Still's LD or the development of Still's LD should not be considered as a contraindication to IL-1 or IL-6 inhibitors.
  4. Considering the overall benefit-risk ratio, the TF recommends that IL-1 and IL-6 inhibitors should not be withdrawn and thus should be continued in patients with Still's LD.
  5. Additionally, given the potential involvement of T cells in LD pathogenesis, some TF experts felt that it is reasonable to initiate T cell-directed immunosuppression in patients at high risk for LD or developing LD, although direct clinical evidence for this strategy is still absent.
  6. Benefits of JAK inhibitors in patients with Still's LD has been reported in two single cases.
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