A 26-year-old cabin crew member presented with a two-month history of dry cough and dyspnea, leading to a diagnosis of systemic lupus erythematosus and pseudo-pseudo Meigs’ syndrome. Diagnostic evaluation confirmed a rare presentation involving pleural effusion, ascites, and an elevated cancer antigen 125 level, which resolved with medical therapy.
Autoimmune conditions often present with overlapping clinical features that challenge standard diagnostic frameworks. When unusual combinations of symptoms appear, clinicians must carefully differentiate between primary autoimmune manifestations, concurrent organ pathology, and malignancy. Recent case documentation highlights the complex diagnostic pathways required when dealing with rare systemic lupus erythematosus manifestations and overlap syndromes.
Case Presentation and Diagnostic Workup of Pseudo-Pseudo Meigs’ Syndrome
The patient, a young South African woman employed as a cabin crew member, sought medical attention for a two-month history of dry cough, exertional dyspnea, and left-sided pleuritic chest pain. She also reported hair loss, recurrent mouth ulcers, and morning stiffness, alongside recent travel history to Japan and Indonesia. Physical examination revealed dullness to percussion and reduced breath sounds over the lower left lung fields.
Initial chest radiography demonstrated a moderate left pleural effusion. Diagnostic pleural tap findings showed an exudative, lymphocyte-predominant effusion with a glucose level of 5.8 mmol/L, a lactate dehydrogenase level of 125 U/L against a serum level of 144 U/L, and a protein level of 47.8 g/L against a serum level of 66 g/L. Cultures showed no growth, and cytology revealed no atypical or malignant cells.
Extensive evaluations, including tumor marker assessments, pleural biopsies, and cytology, successfully excluded malignancy. The single significant tumor marker finding was an elevated cancer antigen 125 level of 156.0 U/mL. Subsequent autoimmune workup revealed positive antinuclear antibodies with a titer of 1:1280 in a homogeneous pattern, elevated anti-double-stranded DNA antibody levels, and decreased complement components C3 at 0.50 gm/L and C4 at 0.05 gm/L. Further testing identified positive anti-Ro and anti-La antibodies.
Imaging Findings and Diagnostic Criteria
Advanced imaging clarified the extent of internal fluid accumulation and structural findings. Contrast-enhanced computed tomography of the thorax demonstrated a moderate left pleural effusion accompanied by underlying lung collapse and air bronchograms resulting from passive compressive atelectasis.
Pseudo-pseudo Meigs’ syndrome represents a variant occurring in systemic lupus erythematosus where pleural effusion and ascites develop due to serositis rather than an ovarian tumor, and the condition typically resolves with management of the underlying autoimmune disease. The patient achieved complete resolution following treatment with prednisolone and hydroxychloroquine.
Overlapping Autoimmune Pathologies and Mechanistic Considerations
Autoimmune overlap syndromes present additional clinical complexities. In a separate case report published in Internal Medicine examined granulomatosis with polyangiitis overlapping with systemic lupus erythematosus in a 74-year-old Japanese woman. That patient had a 39-year history of systemic lupus erythematosus and a history of diffuse large B-cell lymphoma in remission, and she later developed acute renal function decline, hematuria, and elevated anti-myeloperoxidase antineutrophil cytoplasmic antibodies.
Researchers note that while both systemic lupus erythematosus and antineutrophil cytoplasmic antibody-associated vasculitis involve autoantibody formation, their underlying immune mechanisms differ. Systemic lupus erythematosus features antibodies against nuclear antigens depositing in tissues, whereas antineutrophil cytoplasmic antibodies activate neutrophils to cause small vessel inflammation. Impaired clearance of neutrophil extracellular traps containing proteinase-3 and myeloperoxidase may contribute to small vessel injury and offer a mechanistic basis for overlap syndromes.
Therapeutic Approaches and Disease Management
Management strategies for systemic lupus erythematosus rely on induction therapies to control acute severe manifestations, followed by long-term maintenance protocols. Corticosteroids remain the first-line intervention, often supplemented by immunosuppressive agents such as cyclophosphamide or mycophenolate mofetil for severe disease manifestations detailed in professional rheumatology guidance. Biological therapies, including belimumab and voclosporin, are also utilized to improve renal outcomes.
Clinical management requires careful attribution of symptoms, particularly when neuropsychiatric or serosal manifestations occur. As outlined in broad epidemiological and clinical reviews compiled across international research platforms, understanding symptom chronology and identifying early prodromal signs help clinicians detect flares and apply targeted immunomodulatory therapies to mitigate long-term patient disability.
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