What is the management approach for Tjalma syndrome?

Tjalma Syndrome (Pseudo-Pseudo Meigs' Syndrome)

Definition and Clinical Presentation

Tjalma syndrome is a rare manifestation of systemic lupus erythematosus (SLE) characterized by the triad of ascites, pleural effusion, and elevated CA-125 levels in the absence of any benign or malignant ovarian tumor. 1, 2

• The syndrome presents with massive ascites and/or pleural effusion that can be refractory to standard treatment 1
• CA-125 levels are markedly elevated (often >800 U/ml), mimicking ovarian malignancy 3
• Some patients may present with ascites alone without pleural or pericardial effusions, representing an atypical presentation 2
• Hyperferritinemia may accompany severe cases, functioning as an inflammatory marker of disease activity 4
• The condition can manifest as an acute surgical abdomen, leading to diagnostic confusion with gynecological emergencies 5

Diagnostic Approach

The key diagnostic step is excluding ovarian pathology through imaging while confirming active SLE, preventing unnecessary surgical interventions.

• Perform vaginal ultrasound and abdominal CT to rule out pelvic masses or ovarian tumors 3
• Measure serum CA-125 levels, which will be elevated but should not automatically trigger oncologic workup in known SLE patients 3
• Assess SLE disease activity markers including complement levels, anti-dsDNA antibodies, and inflammatory markers 4
• Check serum ferritin levels, as hyperferritinemia correlates with severe inflammation in this syndrome 4
• Perform diagnostic paracentesis with cytology to exclude malignancy and confirm inflammatory ascites 3
• Consider laparoscopy only if diagnostic uncertainty persists after non-invasive evaluation, as cytology typically shows acute inflammation without malignant cells 3

Critical pitfall: Four-thirds of patients evaluated by gynecologists underwent unnecessary hysterectomy due to lack of awareness of this syndrome 5. Always consider Tjalma syndrome in the differential diagnosis when evaluating SLE patients with ascites and elevated CA-125.

Management Strategy

Aggressive immunosuppressive therapy targeting the underlying SLE activity is the definitive treatment, not surgical intervention.

• Adjust or intensify SLE maintenance therapy immediately upon diagnosis 3
• Initiate high-dose systemic corticosteroids as first-line therapy for the acute inflammatory phase 1
• Add methotrexate or mycophenolate mofetil for steroid-sparing effect and long-term disease control, following standard SLE treatment protocols 1
• Monitor response clinically through resolution of ascites and pleural effusion 3
• Track CA-125 levels serially, which should normalize within 10 weeks of appropriate immunosuppressive treatment 3
• Perform therapeutic paracentesis for symptomatic relief if massive ascites causes respiratory compromise or severe discomfort 1

The prognosis is favorable with appropriate immunosuppressive therapy, with complete resolution of effusions and normalization of CA-125 typically occurring within 2-3 months. 3

Key Clinical Pearls

• Tjalma syndrome can be the initial presenting manifestation of juvenile-onset SLE, even in adolescents 1
• The syndrome represents a diagnosis of exclusion requiring absence of ovarian pathology 2
• Migrated surgical clips (such as Filshie clips) embedded in peritoneum can mimic this syndrome and should be considered in patients with prior tubal ligation 3
• Gynecologists must maintain high clinical suspicion to avoid unnecessary surgical exploration in SLE patients presenting with this triad 5
• The mechanism underlying this syndrome remains unclear, but it responds predictably to immunosuppression targeting SLE activity 5