Indian Journal of Medical Sciences Medknow Publications on behalf of Indian Journal of Medical Sciences Trust ISSN: 0019-5359 EISSN: 1998-3654 Vol. 60, Num. 12, 2006, pp. 520-522

Indian Journal of Medical Sciences, Vol. 60, No. 12, December, 2006, pp. 520-522

Letter To Editor

An unusual case of systemic lupus erythematosus mimic: Disseminated gastric signet ring cell carcinoma

Haroon Nigil, Aggarwal Amita, Garg Naveen, Krishnani Narendra, Kumari Neerja, Agarwal Vikas

Department of Clinical Immunology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow
Correspondence Address:Clinical Immunology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Raebareli Road, Lucknow - 226014, vikasagr@sgpgi.ac.in

Code Number: ms06077

Sir,

A 41-year-old lady presented with acute worsening of dyspnea and orthopnea of one day duration. She had history of irregular fever for 5 months and dyspnea and anasarca for 1.5 months. Two weeks earlier, she underwent pericardiocentesis for cardiac tamponade, elsewhere. Fluid was exudative with lymphocyte predominance and was initiated on rifampicin, isoniazid, ethambutol, and pyrazinamide. Examination revealed temperature 101°F, pallor, tachypnea (36/min), tachycardia (124/min), pedal edema, blood pressure 120/70 mm Hg (pulsus paradoxus 20 mm Hg), raised jugular venous pressure, bilateral pleural effusion, muffled heart sounds, tender hepatomegaly, ascites and 5x6 cm patch of skin thickening in left posterior triangle of the neck. Rest of the examination was unremarkable. Radiograph chest showed bilateral pleural effusion with cardiomegaly. Electrocardiogram showed sinus rhythm with low voltage complexes. 2D-echocardiogram showed large pericardial effusion with early tamponade. Urgent pleuro- (500 ml) and pericardiocentesis (450 ml) were carried out. Laboratory investigations showed positive antinuclear antibodies with low C3 and Coombs positive hemolytic anemia [Table - 1][Table - 2]. With a provisional diagnosis of systemic lupus erythematosus (SLE), she received 3 boluses of methylprednisolone

(1 g/day) followed by oral prednisolone 1mg/kg/day along with antibiotics. Both the effusions resolved but fever continued. Seven days later, she developed recurrent vomiting, upper abdominal discomfort and intermittent dyspnea without hypoxemia. Erect radiograph of the abdomen was normal. Gastroduodenoscopy showed growth at gastric outlet and obstruction in the 1st part of duodenum. Biopsies were taken from the duodenum and thickened skin in the neck. Two days later, patient died following acute respiratory distress following oral contrast intake for computerized tomography. Consent for autopsy was denied. The skin and duodenal biopsy reports (available post-mortem) showed evidence of signet ring carcinoma cells [Figure - 1][Figure - 2].

Polyserositis, immune hemolytic anemia (AIHA), lymphopenia and positive ANA with low C3 favored the diagnosis of SLE, initially. ANA though cardinal to SLE are not exclusive to it and may occur in malignancies, [1],[2] and drugs. We ruled out drug induced lupus on the basis of history. A weak association between malignancies, especially lymphomas and SLE has been reported recently,[3] however, polyserositis and cardiac tamponade are distinctly uncommon presentations of lymphomas. Moreover, we have a cohort of more than 500 SLE patients in our clinic and over last 16 years we have not observed a single case of malignancy (unpublished data). It was only during workup of upper intestinal obstruction symptoms, a growth at the gastric outlet and obstruction in the duodenum suggested a malignant lesion. Gastric malignancies may present with pericardial effusion and AIHA,[4] positive ANA.[2] and cardiac tamponade.[5] A similar case of a 45-year-old man presenting with cardiac tamponade due to disseminated signet-ring cell carcinoma of stomach has been described. He died of respiratory failure. Autopsy revealed pulmonary lymphangitis, pericarditis, and pleuritis carcinomatosa.[4] Possibly, this could have been the cause of death in our patient as well as signet ring cells were seen infiltrating the lymphatics in the duodenum [Figure - 2].

Our case highlights an unusual case of signet ring cell carcinoma of the stomach mimicking SLE.

References

Copyright 2006 - Indian Journal of Medical Sciences

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