Case Report: An Adult-Onset Still’s Disease Mimic

Conclusion

Our case highlights a rare presentation of a paraneoplastic syndrome presenting as adult-onset Still’s disease. The patient met Yamaguchi criteria for a diagnosis of adult-onset Still’s disease, but a diagnosis of esophageal cancer was made within weeks. This demonstrates the need for aggressive exclusion of underlying malignancy in cases of suspected adult-onset Still’s disease.

Theodore Korty, DO, is a first-year rheuma­tology fellow at Regional Medical Center Bayonet Point, Hudson, Fla.

Ken Baxa, DO, is a first-year rheumatology fellow at Regional Medical Center Bayonet Point, Hudson, Fla.

Kiren Sahni, DO, is a first-year rheumatology fellow at Regional Medical Center Bayonet Point, Hudson, Fla.

Adam Grunbaum, DO, is the rheumatology program director at Regional Medical Center Bayonet Point, Hudson, Fla.

Maria Soto-Aguilar, MD, is the rheumatology program core faculty at Regional Medical Center Bayonet Point, Hudson, Fla.

References

1. Yamaguchi M, Ohta A, Tsunematsu T, et al. Preliminary criteria for classification of adult Still’s disease. J Rheumatol. 1992 Mar;19(3):424–430.
2. Fautrel B, Zing E, Golmard JL, et al. Proposal for a new set of classification criteria for adult-onset still disease. Medicine (Baltimore). 2002 May;81(3):194–200.
3. Bagnari V, Colina M, Ciancio G, et al. Adult-onset Still’s disease. Rheumatol Int. 2010 May;30(7):855–862.
4. Lièvre A, Bachet JB, Le Corre D, et al. KRAS mutation status is predictive of response to cetuximab therapy in colorectal cancer. Cancer Res. 2006 Apr 15;66(8):3992–3995.
5. Blanchard P, Quero L, Pacault V, et al. Prognostic significance of anti-p53 and anti-KRAS circulating antibodies in esophageal cancer patients treated with chemoradiotherapy. BMC Cancer. 2012 Mar 26;12:119.
6. Hsieh CC, Hsu HS, Li AY, Chen YJ. Clinical relevance of PD-L1 and PD-L2 overexpression in patients with esophageal squamous cell carcinoma. J Thorac Dis. 2018 Jul;10(7):4433–4444.
7. Bellini MF, Cadamuro AC, Succi M, et al. Alterations of the TP53 gene in gastric and esophageal carcinogenesis. J Biomed Biotechnol. 2012;2012:891961.
8. Shibuya Y, Matuo K, Kawada T, et al. [Adult onset Still’s disease associated esophageal cancer: A case report.] [Article in Japanese] Ryumachi. 2003 Jun;43(3):577–582.
9. Sun NZ, Brezinski EA, Berliner J, et al. Updates in adult-onset Still disease: Atypical cutaneous manifestations and associations with delayed malignancy. J Am Acad Dermatol. 2015 Aug;73(2):294–303.
10. Rogues AM, Vidal E, Boudinet F, et al. Breast cancer with systemic manifestations mimicking Still’s disease. J Rheumatol. 1993 Oct:20(10):1786–1787.
11. Ahn JK, Oh JM, Lee J, et al. Adult onset Still’s disease diagnosed concomitantly with occult papillary thyroid cancer: Paraneoplastic manifestation or coincidence? Clin Rheumatol. 2010 Feb;29(2):221–224.
12. Raza A, Houk L, Yousaf W, et al. Unusual para-neoplastic manifestation of cholangiocarcinoma. J Gastrointest Cancer. 2013 Jun;44(2):228–230.
13. Cabane J, Lebas J, Wattiaux MJ, Imbert JC. [Pseudo-Still disease and neoplasm. 2 cases]. [Article in French] Rev Med Interne. 1988 Jan–Feb;9(1):81–84.
14. Chavda R, Bussey MR, Tehrani R, Ostrowski RA. The prevalence of malignancy in adult-onset Still’s disease [abstract]. Arthritis Rheum. 2014 Oct;66(Suppl 10):S545.