J Rheum Dis.  2018 Jul;25(3):207-211. 10.4078/jrd.2018.25.3.207.

Case of Idiopathic Hypereosinophilic Syndrome with Articular Involvement

• Kim JH
• Ha YJ
• Kang EH
• Song YW
• Lee YJ

• KMID: 2415610
• DOI: http://doi.org/10.4078/jrd.2018.25.3.207

Abstract

Idiopathic hypereosinophilic syndrome (IHES) is a rare disease that is characterized by otherwise unexplained persistent eosinophilia and organ damage caused by eosinophilic infiltration. Its manifestations are highly variable but clinically apparent arthritis is uncommonly observed. Although Korean cases of severe eosinophilia in patients with rheumatoid arthritis (RA) or IHES concurrent with RA have been published, there are no reports of IHES with joint involvement. This paper reports a case of IHES presenting with persistent peripheral eosinophilia, fever, skin rash, multiple lymphadenopathy, and polyarthritis, including the distal interphalangeal joints of the hands.

Keyword

Hypereosinophilic syndrome; Arthritis; Finger joint; Cyclosporine

MeSH Terms

Arthritis
Arthritis, Rheumatoid
Cyclosporine
Eosinophilia
Eosinophils
Exanthema
Fever
Finger Joint
Hand
Humans
Hypereosinophilic Syndrome*
Joints
Lymphatic Diseases
Rare Diseases
Cyclosporine

Figure

• 

  Figure 1 Photographs of skin lesions. Erythematous and exfoliative eruptions (A) progressed into generalized erythroderma and scaling (B) over about 6 months.

• 

  Figure 2 Articular manifestation. Photography (A) and X-ray (B, oblique) of both hands showed prominent swelling in the right 3rd proximal interphalangeal, left 1st interphalangeal, and both 2nd, left 4th, and both 5th distal interphalangeal (DIP) joints. Radiographic joint-space narrowing was observed in the left radio-lunar and radio-scaphoid joints and both 2nd DIP joints.

• 

  Figure 3 Synovial biopsy showing edema, synovial cell hyperplasia, and infiltration of lymphoplasma cells and eosinophils (H&E, ×400).

Reference

1. Curtis C, Ogbogu P. Hypereosinophilic Syndrome. Clin Rev Allergy Immunol. 2016; 50:240–251.

2. Weller PF, Bubley GJ. The idiopathic hypereosinophilic syndrome. Blood. 1994; 83:2759–2779.

3. Brogadir SP, Goldwein MI, Schumacher HR. A hypereosinophilic syndrome mimicking rheumatoid arthritis. Am J Med. 1980; 69:799–802.

4. Spry CJ, Davies J, Tai PC, Olsen EG, Oakley CM, Goodwin JF. Clinical features of fifteen patients with the hypereosinophilic syndrome. Q J Med. 1983; 52:1–22.

5. Martín-Santos JM, Mulero J, Andréu JL, de Villa LF, Bernaldo-de Quirós L, Noguera E. Arthritis in idiopathic hypereosinophilic syndrome. Arthritis Rheum. 1988; 31:120–125.

6. Anders HJ, Schattenkirchner M. Destructive joint lesions and bursitis in idiopathic hypereosinophilic syndrome. Rheumatology (Oxford). 1999; 38:185–186.

7. Chaudhuri K, Dubey S, Zaphiropoulos G. Idiopathic hypereosinophilic syndrome in a patient with long-standing rheumatoid arthritis: a case report. Rheumatology (Oxford). 2002; 41:349–350.

8. Choi JH, Jung JW, Song HJ, Song KE, Choi JH, Suh YJ, et al. A case of seronegative rheumatoid arthritis with idiopathic hypereosinophilic syndrome. J Korean Rheum Assoc. 2003; 10:200–205.

9. Park JH, Lee WS, Park SJ, Yoo WH. Hypereosinophilic syndrome associated with the onset of rheumatoid arthritis: a case report. J Rheum Dis. 2017; 24:165–168.

10. Sohn CI, Kim MK, Lee KC, Jung SS, Lee IH, Bae SC, et al. A case of rheumatoid arthritis accompanied by severe eosinophilia. J Korean Rheum Assoc. 1994; 1:98–102.

11. Tay C. Eosinophilic arthritis. Rheumatology (Oxford). 1999; 38:1188–1194.

12. Guellec D, Milin M, Cornec D, Tobon GJ, Marhadour T, Jousse-Joulin S, et al. Eosinophilia predicts poor clinical outcomes in recent-onset arthritis: results from the ESPOIR cohort. RMD Open. 2015; 1:e000070.

13. Chen Z, Andreev D, Oeser K, Krljanac B, Hueber A, Kleyer A, et al. Th2 and eosinophil responses suppress inflammatory arthritis. Nat Commun. 2016; 7:11596.

14. Kita H. Eosinophils: multifaceted biological properties and roles in health and disease. Immunol Rev. 2011; 242:161–177.

15. Ogbogu PU, Bochner BS, Butterfield JH, Gleich GJ, Huss-Marp J, Kahn JE, et al. Hypereosinophilic syndrome: a multicenter, retrospective analysis of clinical characteristics and response to therapy. J Allergy Clin Immunol. 2009; 124:1319–1325.e3.