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A case of mixed connective tissue disease with pseudo-pseudo Meigs' syndrome (PPMS)-like features

C K Cheah1, S Ramanujam2, N Mohd Noor2

  • 1Division of Rheumatology, Department of Medicine, Hospital Tuanku Ja'afar Seremban, Malaysia cheeken78@gmail.com.

Lupus
|September 18, 2015
PubMed

Insights

Pseudo-pseudo Meigs' syndrome (PPMS) is a rare condition in systemic lupus erythematosus. This case highlights PPMS-like features in mixed connective tissue disease, responding well to steroid treatment.

Area of Science:

  • Rheumatology
  • Internal Medicine
  • Oncology (for CA-125 marker)

Background:

  • Pseudo-pseudo Meigs' syndrome (PPMS) is infrequently associated with systemic lupus erythematosus (SLE).
  • Its occurrence in other connective tissue diseases is uncommon, making diagnosis challenging.
  • PPMS is characterized by ascites, pleural effusion, and elevated CA-125 levels.

Observation:

  • A patient presented with significant ascites, pleural effusion, and markedly elevated serum CA-125 levels.
  • These clinical and laboratory findings mimicked PPMS.
  • The patient's underlying condition was diagnosed as mixed connective tissue disease (MCTD).

Findings:

  • The diagnostic workup revealed mixed connective tissue disease (MCTD) as the cause of the PPMS-like presentation.
  • The patient's symptoms, including ascites and pleural effusion, resolved with treatment.
  • Marked elevation of CA-125 normalized following treatment.

Implications:

  • This case expands the known clinical spectrum of mixed connective tissue disease (MCTD).
  • It suggests that PPMS-like features should be considered in the differential diagnosis of unexplained ascites and pleural effusions in MCTD.
  • Systemic steroid therapy can be effective in managing PPMS-like manifestations in MCTD.
Keywords:
CA-125Mixed connective tissue diseasepseudo-pseudo Meigs’ syndrome

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