Background/Purpose:

Adult-onset Still’s disease (AOSD) is a systemic inflammatory disease of unknown etiology.  It is characterized by high fever, evanescent salmon-colored rash, sore throat, liver dysfunction, lymphadenopathy, hepato-splenomegaly, arthritis, and leukocytosis.  Although most inflammatory disorders have a known risk of malignancy there is a lack of published data regarding the risk of malignancy in AOSD.  There are multiple case reports suggesting the presence of AOSD or an AOSD-like syndrome in the setting of various solid tumors (breast, lung, renal and thyroid cancer) and hematological malignancies (leukemia and lymphoma).  To date, there are no published studies of the relationship between AOSD and cancer.  We conducted a retrospective observational study to identify an association between AOSD and malignancy.

Methods:
A chart review was performed of patients 18 years of age and older who were diagnosed with AOSD based on Yamaguchi criteria between January 1, 2006 and June 30, 2013 at Loyola University Medical Center.  Patients with known infection, pre-existing malignancy, or other rheumatic disorders at the time of diagnosis of AOSD were excluded.  Patient charts were reviewed for a subsequent diagnosis of cancer.

Results:

From January 2006 to June 2013 twenty-one cases of AOSD were identified.  Of these cases, eight fulfilled Yamaguchi criteria for definite AOSD while one was diagnosed with probable AOSD (Table 1).  The median age was 38 years (range 18-63 years) and median follow up was 25 months (1- 72 months).  One patient was subsequently diagnosed with Hodgkin’s lymphoma at 7 months follow up.  Another patient was diagnosed with non-small cell lung cancer (NSCLC) at 3 months follow up and then acute myeloid leukemia (AML) at 19 months follow up.

Conclusion:

In our case series of 9 patients followed over a median period of 25 months, we noted 1 case of Hodgkin’s lymphoma, 1 case of AML and 1 case of NSCLC.  The National Cancer Institute reports the incidence of Hodgkin’s lymphoma as 2.7 per 100,000 and the incidence of leukemia as 13.0 per 100,000 per year.  The incidence reported for all lung cancers is 49.2-66.8 per 100,000 per year.  The observed incidence of Hodgkin’s lymphoma, AML and NSCLCA in our case series is many times higher.  Although the association between AOSD and malignancy has been rarely reported, careful screening for malignancy in patients diagnosed with AOSD may be warranted.  Additional studies of association between AOSD and malignancy are needed to further explore the relationship between these diagnoses.