Eur J Pediatr (1992) 151 : 581-582

EuropeanJournalof

Pediatrics

9 Springer-Verlag 1992

Systemic lupus erythematosus: a case report with unusual manifestations and favourable outcome after plasmapheresis J. Garcia-Consuegra 1, R. Merino l, A. Alonso 2, and F. Goded 3 1Unit of Paediatric Rheumatotogy, ;Section of Paediatric Nephrology, 3Intensive Care Unit, La Paz Children's Hospital, E-28046 Madrid, Spain Received August 13, 1991 / Accepted after revision November 21, 1991

Abstract. W e r e p o r t a case o f s y s t e m i c lupus e r y t h e m a tosus ( S L E ) in a 1 5 - y e a r - o l d girl with s e v e r e n e u r o l o g i c a l disease, platelet function disorder and pulmonary haemorrhage, which remitted after plasmapheresis. The patient developed protein-losing enteropathy shrinking lung, a n d a c u t e p a n c r e a t i t i s with p s e u d o c y s t f o r m a t i o n . T h e s e i n f r e q u e n t c o m p l i c a t i o n o f S L E a r e discussed.

Key words: S y s t e m i c l u p u s e r y t h e m a t o s u s - P l a s m a p h e r esis - P a n c r e a t i t i s - P r o t e i n - l o s i n g e n t e r o p a t h y - S h r i n k ing lung

Introduction S y s t e m i c lupus e r y t h e m a t o s u s ( S L E ) is a m u l t i s y s t e m d i s e a s e t h a t can i n v o l v e m o s t o r g a n systems. W e r e p o r t t h e o c c u r r e n c e o f m u l t i p l e a n d u n u s u a l m a n i f e s t a t i o n s in o n e p a t i e n t , a n d h e r f a v o u r a b l e o u t c o m e with p l a s m a pheresis.

DNA and anti-RO test were negative. Antibodies to total histones (12920 IU/1) and IgM cardiolipin antibody titre were elevated. Serum complement and repeated urinalyses were normal. Hypoalbuminaemia of 2,4g/1 was detected. Stool examinations disclosed fat malabsorption (faecal fat 14,6g/24h) and a proteinlosing enteropathy (alpha-l-antitrypsin 4.5 mg/g dry stools; normal = < 0.75). Small intestine biopsy was normal. Chest roentgenogram showed decreased lung volumes (Fig. 1), and tung function test revealed a pattern of restrictive disease and a diminished CO diffusion, suggesting a condition called ,,shrinking lung". Echocardiography showed a mildly thickened pericardium without effusion. Over the next month she developed thrombophlebitis of the left superficial femoral vein associated with a transient fall in antithrombin III activity. Oral prednisone was increased to 70 mg/day, monthly i.v. cyclophosphamide was continued and 125mg/day of acetylsalicyclicacid was added. Mild improvement occurred. One month later, she was readmitted with generalized seizures and coma. Cerebrospinal fluid and CT scan were normal, and the EEG showed generalized slowing. Multiple severe haematomas were observed. Platelet adherence and aggregation tests were defective, with Ivy bleeding time longer than 3 days. Total platelet count was normal, as well as the remaining coagulation parameters. Mechanical ventilation was instituted. She was treated with i.v. methylprednisolone bolus for 4 days, without improvement. Plasmapheresis was started with an 80% blood volume exchange, using 62% of plasma for restitution, and continued for 7 days. Her clinical course improved dramatically with remission of neurologi-

Case report SLE was diagnosed in a 15-year-old girl in February 1989 at a local hospital, on the basis of fever, dyspnoea, haemolytic anaemia, pleuropericarditis, arthritis and positive antinuclear antibody test. No drug ingestion was reported. She was treated with prednisone, 60 mg daily and i.v. cyclophosphamide. Because fever, progressive dyspnoea and weight loss persisted, she was transferred to our institution. On admission she presented with malaise, dyspnoea, and leg oedema. Chest examination showed decreased breath sounds in both lower lungs. Cardiac auscultation was normal. Elbow and hand joints were swollen. No rash was observed. Laboratory studies showed anaemia (Hb 7,5 g/dl); leucopenia (2.750 leucocytes/ram3); and lymphopenia (500 lymphocytes/mm3). Clotting tests were normal and no lupus anticoagulant was detected. Antinuclear antibodies were positive at a titre of 1/2560; antiOffprint requests to: J. Garcia-Consuegra Abbreviation: SLE = systemic lupus erythematosus

Hg. 1, Chest roentgenogram showing reduction in lung volume

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Fig. 2. Ultrasound examination showing pancreatic pseudocyst cal dysfunction and clotting disorder. Staphylococcus aureus and then Candida septicemia occurred and were treated appropriately. In November 1989 she developed severe pancreatitis requiring total parenteral nutrition for 2 months. Prednisone was increased to 100 mg/day. She improved but a pancreatic pseudocyst developed (Fig. 2) and was drained by ultrasound-guided needle aspiration. In December she developed a severe lung haemorrhage. Steroids were given by bolus without response and plasmapheresis was reinstituted. Improvement became evident after the 2nd of a total of 12 sessions with gradual recovery. One month later a left tibial nerve palsy was detected, which spontaneously regressed. She was discharged in February 1990. At present, 2 years after her first admission, she remains free of symptoms, with decreasing steroid doses, and oral cyclophosphamide. Cutaneous rash, urinary abnormalities or hypertension have not been observed.

Discussion Protein-losing e n t e r o p a t h y associated with S L E has been described in relation to pericarditis, intestinal l y m p h a n giectasia and vasculitis [4, 10], although in m a n y instances the p a t h o g e n i c m e c h a n i s m remains u n k n o w n . In o u r patient the excessive intestinal protein loss o c c u r r e d when pericarditis i m p r o v e d . Pancreatitis is infrequently seen in S L E but m a y occur in an acutely ill patient with active multisystem disease [6]. A n u m b e r of p a t h o g e n i c m e c h a n i s m s have b e e n prop o s e d including vasculitis with ischaemia, a u t o - i m m u n -

ity and drug toxicity. T h e role of corticosteroids in the precipitating and t r e a t m e n t of acute pancreatitis is unclear [9]. T h e shrinking lung has b e e n ascribed to p r i m a r y pulm o n a r y disease, diaphragmatic dysfunction, and to recurring pleuritis [2, 8]. I m p a i r e d platelet a d h e r e n c e and aggregation p r e s e n t in o u r patient is rare [1, 5]. Plasmapheresis diminishes the circulation of i m m u n e complexes, antibodies and active c o m p l e m e n t c o m p o nents. It has b e e n r e c o m m e n d e d in e x t r e m e l y ill patients w h o have not r e s p o n d e d to conventional t h e r a p y [3, 7], and this view is strongly s u p p o r t e d by the f a v o u r a b l e course of our patient. T h e only possibly related complications were Staphylococcus aureus septicaemia and candidiasis.

References 1. Byron MA (1982) The clotting defect in SLE. Clin Rheum Dis 8 : 137-151 2. Delgado EA, Malleson PN, Pirie GE, Petty RE (1990) The pulmonary manifestations of childhood onset systemic lupus erythematosus. Semin Arthritis Rheum 19: 285-293 3. Lieberman JD, Schatten S (1988) Systemic lupus erythematosus: Treatment. Disease-modifying therapies. Rheum Dis Clin North Am 14 : 223-243 4. Monballyu J, Hauglustaine D, Geboes K, Desmet V, Michielsen P (1985) Protein-losing enteropathy in systemic lupus erythematosus. Digestion 31 : 243-246 5. Regan MG, Lackner H, Karpatkin S (1974) Platelet function and coagulation profile in lupus erythematosus. Ann Intern Med 81 : 462-468 6. Reynolds JC, Inman RD, Kimberly RP, Chuong JH, Kovacs JE, Walsh MB (1982) Acute pancreatitis in systemic lupus erythematosus: report of twenty cases and a review of the literature. Medicine 61 : 25-32 7. Schur PH (1985) The clinical management of systemic lupus erythematosus. Grune and Stratton, New York London Paris, pp 272-273 8. Thompson P J, Dhillon DP, Ledingham J, Turner-Warwick M (1985) Shrinking lungs, diaphragmatic dysfunction, and systemic lupus erythematosus. Am Rev Respir Dis 132 : 926-928 9. Watts RA, Isenberg DA (1989) Pancreatic disease in the autoimmune rheumatic disorders. Semin Arthritis Rheum 19 : 158-165 10. Weiser MM, Andres GA, Brentjens JR, Evans JT, Reichlin M (1981) Systemic lupus erythematosus and intestinal venulitis. Gastroenterology 81 : 570-579

Systemic lupus erythematosus: a case report with unusual manifestations and favourable outcome after plasmapheresis.

We report a case of systemic lupus erythematosus (SLE) in a 15-year-old girl with severe neurological disease, platelet function disorder and pulmonar...
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