Case Report: Diagnosing a Lupus-Flare Mimic

Conclusion

iMCD-TAFRO may mimic SLE. An accurate differential diagnosis and consideration for an underlying autoimmune mechanism are critical to ensure appropriate disease management. 

Nobuya Abe, MD, is a clinical fellow in the Department of Rheumatology, Endocrinology and Nephrology, Graduate School of Medicine, Hokkaido University, Sapporo, Hokkaido, Japan.

Yuichiro Fujieda, MD, PhD, is an assistant professor in the Department of Rheumatology, Endocrinology and Nephrology, Faculty of Medicine, Hokkaido University.

Tatsuya Atsumi, MD, PhD, is a professor in the Department of Rheumatology, Endocrinology and Nephrology, Faculty of Medicine, Hokkaido University.

Authors’ note: All authors contributed to the patient’s care and the writing of the report. Written consent for publication was obtained from the patient.

References

1. Iwaki N, Fajgenbaum DC, Nabel CS, et al. Clinicopathologic analysis of TAFRO syndrome demonstrates a distinct subtype of HHV-8-negative multicentric Castleman disease. Am J Hematol. 2016 Feb;91(2):220–226.
2. Fajgenbaum DC. Novel insights and therapeutic approaches in idiopathic multicentric Castleman disease. Blood. 2018;132(22):2323–2330.
3. Takai K, Nikkuni K, Shibuya H, Hashidate H. [Thrombocytopenia with mild bone marrow fibrosis accompanied by fever, pleural effusion, ascites and hepatosplenomegaly.] [article in Japanese] Rinsho Ketsueki. 2010 May;
   51(5):320–325.
4. Masaki Y, Kawabata H, Takai K, et al. 2019 updated diagnostic criteria and disease severity classification for TAFRO syndrome. Int J Hematol. 2020 Jan;111(1):155–158.
5. Masaki Y, Kawabata H, Takai K, et al. Proposed diagnostic criteria, disease severity classification and treatment strategy for TAFRO syndrome, 2015 version. Int J Hematol. 2016 Jun;103(6):686–692.
6. Konishi Y, Takahashi S, Nishi K, et al. Successful treatment of TAFRO syndrome, a variant of multicentric Castleman’s disease, with cyclosporine A: Possible pathogenetic contribution of interleukin-2. Tohoku J Exp Med. 2015 Aug;236(4):289–295.
7. van Rhee F, Voorhees P, Dispenzieri A, et al. International, evidence-based consensus treatment guidelines for idiopathic multicentric Castleman disease. Blood. 2018 Nov 15;132(20):2115–2124.
8. Fajgenbaum DC, Langan RA, Japp AS, et al. Identifying and targeting pathogenic PI3K/AKT/mTOR signaling in IL-6-blockade-refractory idiopathic multicentric Castleman disease. J Clin Invest. 2019 Aug 13;129(10):4451–4463.
9. Kubokawa I, Yachie A, Hayakawa A, et al. The first report of adolescent TAFRO syndrome, a unique clinicopathologic variant of multicentric Castleman’s disease. BMC Pediatr. 2014 Jun 2;14:139.
10. Iwaki N, Gion Y, Kondo E, et al. Elevated serum interferonγ-induced protein 10 kDa is associated with TAFRO syndrome. Sci Rep. 2017 Feb 13;7:42316.
11. Mavragani CP, Nezos A, Sagalovskiy I, et al. Defective regulation of L1 endogenous retroelements in primary Sjogren’s syndrome and systemic lupus erythematosus: Role of methylating enzymes. J Autoimmun. 2018 Mar;88:75–82.
12. Pullabhatla V, Roberts AL, Lewis MJ, et al. De novo mutations implicate novel genes in systemic lupus erythematosus. Hum Mol Genet. 2018 Feb 1;27(3):421–429.
13. Hasegawa E, Sato H, Wada Y, et al. Characterization of patients with systemic lupus erythematosus who meet the diagnostic criteria for TAFRO syndrome. Lupus. 2018 Mar;27(3):417–427.
14. Shirai T, Onishi A, Waki D, et al. Successful treatment with tacrolimus in TAFRO syndrome: Two case reports and literature review. Medicine (Baltimore). 2018 Jun;97(23):e11045.