Letter to Editor
Pseudo-pseudo Meigs’ syndrome in
Vitorino Modesto dos Santos*
*Adjunct-professor of Internal Medicine. Catholic University Medical Course and Internal Medicine
Department of Armed Forces Hospital, Brasília-DF, Brazil.
Vitorino Modesto dos Santos, Armed Forces Hospital, Estrada do Contorno do Bosque s/n, Cruzeiro Novo,CEP 70658-900, Brasília-DF, Brazil. Tel: 55-61 39662103; Fax: 55-61 32331599; E-mail:
Received: February 21, 2017; Accepted: February 27, 2017; Published: April 09, 2017
The Meigs’ Syndrome (MS) described in 1937 by
Meigs and Cass was characterized by benign ovarian fibroma,
ascites and hydrothorax that resolve after the tumor removal.
Peritoneal and pleural fluids of the patients originally with MS
were transudates . In pseudo-MS similar features are due to
other benign or malignant tumors; Pseudo Pseudo-MS (PPMS)
occurs in Systemic Lupus Erythematosus (SLE) alone or SLE plus
scleroderma [2-6]. The term PPMS was first utilized in 2005 by
Schmitt et al., in the case report of a 33-year-old woman with the
classical characteristics of the syndrome and enlarged cystic
ovaries . However, the abnormal cystic ovarian changes in this
patient could indicate coexistent MS or PMS with active course of
SLE in a young female with the tendency to multi-organ serositis.
In fact, PPMS may be due to lupus phenotype characterized
primarily by polyserositis . The differential diagnosis of PPMS
constitutes a challenging task mainly for non-specialists. A major
concern is about the interpretation of elevated levels of CA 125
described in patients with PPMS [2-6], because this tumor marker
is considered indicative of ovarian malignancy. High levels of
CA125 may be also due to pelvic tuberculosis and nephrotic
syndrome [3,4]. This tumor is the fifth cause of death by cancer
and the most lethal malignancy in women. Therefore, accurate
clinical evaluation with adequate laboratory determinations,
imaging and pathology studies is mandatory in order to establish
the correct diagnosis as early as possible. The mechanisms of
elevated levels of CA 125 as well as the origin peritoneal exudate
in active SLE have been associated with uncontrolled action
of pro-inflammatory cytokines, local vasculitis, aggregation of
plasma cells, immune complexes, elevated serum levels of ferritin,
and the expression of the tumor marker by cells of the omentum
and mesovarium . Pleural effusion is due to ascites passive
transfer to pleural cavity or by lymphatic route, mechanisms that
explain the difference with the pleural transudate of the classical
MS. Paracentesis and therapeutic control of the SLE flare up will
improve the pleural effusion .
PPMS is an exceeding uncommon condition diagnosed
in rheumatologic patients, and the scarcely reported cases were
associated with SLE, or SLE and scleroderma (MCTD) [2-6].
Taking in account that MS exclusively affects women and SLE more
often occurs in females, the hypothesis of a casual concomitance
between these conditions might be also considered. As benign
and malignant ovarian tumors are more frequent than PPMS,
one must rule out the typical MS and the PMS caused by ovarian
malignancy that are more prevalent entities [1-6].
Key words: Mixed connective tissue disease, Pseudopseudo
Meigs’ syndrome, Systemic lupus erythematosus
- Meigs JV, Cass JW. Fibroma of the ovary with ascites and hydrothorax: with a report of seven cases. Am J ObstetGynecol. 1937;33(2):249-267.
- Schmitt R, Weichert W, Schneider W, Luft FC, Kettritz R. Pseudo-pseudo Meigs' syndrome. Lancet. 2005;366(9497):1672. doi: http://dx.doi.org/10.1016/S0140-6736(05)67666-0.
- Cheah CK, Ramanujam S, MohdNoor N, Gandhi C, D Souza BA, et al. A case of mixed connective tissue disease with pseudo-pseudoMeigs' syndrome (PPMS)-like features. Lupus. 2016;25(2):214-216. doi: 10.1177/0961203315606441.
- Dalvi SR, Yildirim R, Santoriello D, Belmont HM. Pseudo-pseudo Meigs' syndrome in a patient with systemic lupus erythematosus. Lupus. 2012;21(13):1463-1466. doi: 10.1177/0961203312461291.
- Lee SY, Lee SW, Chung WT. Severe inflammation may be caused by hyperferritinemia of pseudo-pseudo Meigs' syndrome in lupus patients: two cases reports and a literature review. ClinRheumatol. 2013;32(12):1823-1826. doi: 10.1007/s10067-013-2362-8.
- McVorran S, Song J, Pochineni V, Abrudescu-Opran A. Systemic lupus erythematosus presenting with massive ascites: A case of pseudo-pseudo Meigs syndrome. Case Rep Rheumatol. 2016;16:71. doi.org/10.1155/2016/8701763.