Tjalma Syndrome as the Rare Initial Presentation of Systemic Lupus Erythematosus

To the Editor,

Meig’s syndrome is characterised by pleural effusion and ascites linked to an ovarian fibroma, whereas similar findings associated with other benign pelvic tumours are referred to as pseudo-Meig’s syndrome. Tjalma syndrome or pseudo-Meig’s syndrome is characterised by ascites, pleural or pericardial effusion with elevated CA125 levels without any malignancy or tumour in a case of systemic lupus erythematosus (SLE). ¹ Since the initial description, ² there have been <25 cases which have been reported so far.

Here we present the case of a 38-year-old woman who developed progressive ascites and anasarca having partial relief with diuretics. She also developed a photosensitive malar rash. Three months later, she experienced proximal muscle weakness affecting both her upper and lower limbs. There was no history suggestive of jaundice, upper gastrointestinal (GI) bleeding, blood transfusions, oliguria, haematuria, chest pain, orthopnoea and chronic cough. There was no history of prolonged fever; however, she experienced significant unintentional weight loss of approximately 20 kg, along with anorexia and fatigue. She had secondary amenorrhea for the past 6 months and also had hypothyroidism.

Upon examination, she was found to have anasarca with decreased air entry in bilateral lung fields. She had proximal muscle, neck and truncal weakness with bilateral shoulders having 3/5, hips 2/5 and knees 4/5 power. The gynaecological examination was normal. She was evaluated initially under gastroenterology and investigations showed anaemia (Hb – 8.5 g/dL), lymphopenia (total leukocyte count 3500/cumm, N53L33) and hypoalbuminemia (1.3 g/dL). Her renal, liver and coagulation profiles, as well as urinalysis, were within normal limits. She had elevated muscle enzymes (SGOT/SGPT/LDH/CPK- 90/57/1034/124 U/L).

Ultrasound and computed tomography abdomen with pelvis were suggestive of moderate ascites, bilateral pleural effusion and mild pericardial effusion without any evidence of malignancy. Liver showed fatty infiltration, portal vein and bilateral kidneys were normal. Enterography showed hypertrophic gastric folds and jejunisation of the ileum. There was no evidence of enteritis, the work-up celiac disease was negative and the upper GI endoscopy showed only mild hypertrophic gastric folds. Echocardiography was normal.

Since she was a young woman with unexplained ascites and significant weight loss, malignancy was still a strong concern and tumour markers were sent which showed isolated CA125 elevation (1863 U/mL). Ascitic fluid analysis showed lymphocytic predominant, low-protein ascites, and was negative for malignant cells (Table 1). Workup for tuberculosis was negative.

Table 1.

Ascitic Fluid Analysis.

Parameter	value
Total count	65cells/cu mm
Differential count	Lymphocytes 98, neutrophil 2"
Sugar	100 mg/Dl (blood – 146 mg/dL)
Protein	0.8 g/dL (serum – 3.9)
Albumin	0.2 g/dL (serum – 1.3)
LDH	29 U/L (1034)
Triglycerides	11 mg/dL
Cholesterol	10 mg/dL
ADA	10.3 U/L
Malignant cells/cultures	Negative

Considering her symptom complex and no definite diagnosis so far, an immunology consult was sought and a work-up for lupus was sent which showed positive antinuclear antibodies (4+ coarse speckled by IIF) with low complements (C3/C4-40.2/13.1 mg/dL) and elevated double-stranded DNA (74.2 IU). Antiphospholipid antibody profile and lupus anticoagulant were negative. There was no evidence of active nephritis (urinalysis normal, 24-hour urine protein 116 mg).

She was managed with large-volume paracentesis, diuretics and albumin infusion. She was started on 0.5 mg/kg/day steroids, followed by azathioprine. She had significant improvement in muscle weakness and ascites. However, her repeat urinalysis after 2 months showed 24-hour urine protein of 470 mg (creatinine – 170 mg) and estimated glomerular filtration rate was estimated to be 73 mL/min/1.73 m ² . A renal biopsy showed Class II lupus nephritis with activity index 0 and chronicity index 2, immunofluorescence-positive for immunoglobulin G, Immunoglobulin M, C3, C1q, λ and κ. After 1 year, she is off glucocorticoids, on daily hydroxychloroquine and mycophenolate and is in complete remission. She has gained weight and her CA125 levels normalised to 21.2 U/mL (normal <35 U/mL).

The occurrence of massive ascites in SLE is rare, and polyserositis without nephrotic syndrome is observed in approximately 10%-15% of SLE cases. A review of 21 previously published cases^3,4 revealed that all patients exhibited ascites, and most of them had pleural effusions with over 90% showing an excellent response to immunosuppressive therapy. Notably, a fraction of these patients did not present with nephrotic syndrome and therapy resulted in a reduction in CA125 levels in the majority of them. CA 125 has a specificity of 78% as a marker for ovarian malignancies ⁵ and can be elevated in various other conditions that affect pancreas, breast tissue and mesothelial cells. One possible explanation for elevated CA125 levels is the stimulation of mesothelial cells by ascitic fluid ⁶ which can be the likely cause in our patient too.

Lupus presenting as massive ascites and anasarca without nephritis or enteritis is rare and when associated with elevated CA 125 levels, it is not necessary that it is always associated with malignancy. Our case enlightens the physicians about such a rare presentation of lupus which may help in avoiding unwarranted anxiety and invasive investigations.

Author Contributions

L. M. Rahulan helped in writing – original draft preparation. All authors helped in writing – review and editing. N. C. Gowda, K. Chandwar, R. Chatterjee, A. Lawrence and A. Aggarwal helped in the supervision of the article.