Journal List > J Rheum Dis > v.21(2) > 1064166

TOOLS
Park, Lee, Byon, Lee, Lee, Nam, Park, Kim, Park, Park, Lee, Lee, and Baek: A Case of Tjalma Syndrome Coincidentally Accompanied by an Ovarian Teratoma Successfully Treated with Intravenous Immunoglobulin-G Adjunctive Therapy
Case Report

J Rheum Dis 2014;21(2):77-81.

Published online: 31 October 2014

DOI: https://doi.org/10.4078/jrd.2014.21.2.77

A Case of Tjalma Syndrome Coincidentally Accompanied by an Ovarian Teratoma Successfully Treated with Intravenous Immunoglobulin-G Adjunctive Therapy

Eun-Kyoung Park1, Seung-Geun Lee1, Ik Soo Byon2, Sun-Hee Lee1, Seong-Jun Lee1, Yoon-Jeong Nam1, Ji-Hye Park1, Geun-Tae Kim3, Young-Eun Park4, Seong-Hu Park5, Joung-Wook Lee6, Jun-Hee Lee7, Seung-Hoon Baek7

1Department of Internal Medicine, Pusan National University School of Medicine, Busan, Korea

2Department of Ophthalmology, Pusan National University Yangsan Hospital, Yangsan, Korea

3Division of Rheumatology, Department of Internal Medicine, Kosin University College of Medicine, Busan, Korea

4Malgeunsem Hospital, Busan, Korea

5Changwon, Young-do Hospital, Busan, Korea

6Busan St. Mary's Medical Center, Busan, Korea

7Department of Internal Medicine, Ilsin Christian Hospital, Busan, Korea

Corresponding to: Seung-Geun Lee, Division of Rheumatology, Department of Internal Medicine, Pusan National University School of Medicine, 179, Gudeok-ro, Seo-gu, Busan 602-739, Korea. E-mail: sglee@pnuh.co.kr

Received 22 April 2013       Revised 16 June 2013       Accepted 17 June 2013

Copyright © 2014 The Korean College of Rheumatology

This is a Free Access article, which permits unrestricted non-commerical use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Tjalma or pseudo-pseudo Meigs' syndrome is a clinical condition that is characterized with ascites, pleural effusion, and increased serum CA-125 levels in patients with systemic lupus erythematosus (SLE) without the presence of ovarian tumor. On the other hand, Meigs' and pseudo-Meigs' syndromes represent the same manifestations with ovarian tumor. In this case report, we present a 43-year-old SLE patient suffering from Tjalma syndrome with the coexistence of incidental ovarian teratoma, who was successfully treated with intravenous immunoglobulin-G adjunctive therapy after inadequate response to surgical excision of the ovarian tumor, steroid, and cyclophosphamide pulse therapy.

Keywords: Systemic lupus erythematosus, Intravenous immunoglobulin, Ascites, Pleural effusion

REFERENCES

1. Meigs JV, Cass JW. Fibroma of the ovary with ascites and hydrothorax: with a report of seven cases. Am J Obstet Gynecol. 1937; 33:249–66.
2. Nagakura S, Shirai Y, Hatakeyama K. Pseudo-Meigs' syndrome caused by secondary ovarian tumors from gastrointestinal cancer. A case report and review of the literature. Dig Surg. 2000; 17:418–9.
3. Widra EA, Armstrong J. Pseudo-Meigs' syndrome and lupus. Int J Gynaecol Obstet. 1995; 49:193–4.
crossref
4. Schmitt R, Weichert W, Schneider W, Luft FC, Kettritz R. Pseudo-pseudo Meigs' syndrome. Lancet. 2005; 366:1672.
crossref
5. Tjalma WA. Ascites, pleural effusion, and CA 125 elevation in an SLE patient, either a Tjalma syndrome or, due to the migrated Filshie clips, a pseudo-Meigs syndrome. Gynecol Oncol. 2005; 97:288–91.
crossref
6. Ural UM, Kiliç A, Gü ngör T, Ozdal B, Mollamahmutoğlu L. Tjalma's or pseudo-pseudo-Meigs' syndrome: a case report. Clin Exp Dermatol. 2008; 33:363–4.
crossref
7. Bes C, DağlıÜ , Memedoğlu P, Soy M. A rare form of SLE: pseudo-pseudo meigs syndrome and hydrocephalus. Rheumatol Int. 2013; 33:2175–6.
crossref
8. Dalvi SR, Yildirim R, Santoriello D, Belmont HM. Pseudo-pseudo Meigs' syndrome in a patient with systemic lupus erythematosus. Lupus. 2012; 21:1463–6.
crossref
9. Prasad S, Abujam B, Lawrence A, Aggarwal A. Massive ascites as a presenting feature of lupus. Int J Rheum Dis. 2012; 15:e15–6.
crossref
10. Abramov Y, Anteby SO, Fasouliotis SJ, Barak V. The role of inflammatory cytokines in Meigs' syndrome. Obstet Gynecol. 2002; 99:917–9.
crossref
11. Yang Z, Liang Y, Li C, Zhong R. Serum CA125 elevation is independently associated with serositis in SLE patients. Clin Exp Rheumatol. 2012; 30:93–8.
12. Bayry J, Negi VS, Kaveri SV. Intravenous immunoglobulin therapy in rheumatic diseases. Nat Rev Rheumatol. 2011; 7:349–59.
crossref
13. Damianovich M, Blank M, Raiter A, Hardy B, Shoenfeld Y. Anti-vascular endothelial growth factor (VEGF) specific activity of intravenous immunoglobulin (IVIg). Int Immunol. 2009; 21:1057–63.
crossref

Figure 1.
Chest radiograph showing right pleural effusion (arrow).
jrd-21-77f1.tif
Figure 2.
Abdominal computed tomography showing ascites (arrowhead in A) and 4.3 cm sized ovarian tumor (arrow in B).
jrd-21-77f2.tif
Table 1.
Changes in serum CA-125 and albumin concentrations according to therapy
Day 0 98 102 109 141 143 148 150 154 156 162 178 197
Serum albumin (mg/dL) 1.4 1.5   1.5 1.8     1.6 1.5   2 3.4 3.8
Serum CA-125 (U/mL) 544.4 2,266   1,910 2,951     2,783     1,710 455.1 33.6
Treatment     Oophorectomy     MPT for 3 days Cyclophosphamide     IVIG for 5 days      

MPT: methylprednisolone pulse therapy, IVIG: intravenous immunoglobulin-G.

TOOLS
Similar articles