NDT Advance Access originally published online on February 20, 2006
Nephrology Dialysis Transplantation 2006 21(7):2027-2028; doi:10.1093/ndt/gfl043
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Protein-losing gastroenteropathy in a patient with systemic lupus erythematosus and antiphospholipid antibody syndrome simulating nephrotic syndrome
Email: jameshung{at}yahoo.comSir,
Systemic lupus erythematosus (SLE) is an autoimmune disorder which can affect multiple organs [1]. Protein-losing enteropathy (PLE) in patients with SLE is uncommon [2], typically occurring in young women, and characterized by the onset of profound oedema and hypoalbuminaemia [3], clinically indistinguishable from nephrotic syndrome. In many cases, it is the first obvious manifestation of SLE [3]. Diarrhoea is present in about 50% of cases, but steatorrhoea is absent [3]. We report a case of PLE associated with SLE and antiphospholipid antibody syndrome (APS).
A 34-year-old white woman was referred to our service to investigate a nephrotic syndrome. Three months previously, she presented with abdominal cramps, nausea and oedema of the lower limbs that evolved to generalized oedema. She also had malar rash, photosensitivity, hypothyroidism and a sister with SLE. She denied miscarriage or oral contraceptives use.
Clinical examination revealed normal vital signs, generalized oedema and ascites. Laboratory data included: normal complete blood count/LDH/sodium/potassium/creatinine/liver enzymes/thyroid function/glucose/prothrombin time. Albumin was 0.8 g/dl, globulin 2.4 g/dl, cholesterol 284 mg/dl, C3 79 mg/dl (normal 84–167), C4 15 mg/dl (16–30), ANA>1/200, anti-DNA 49 UI (<30), anti-Ro/La/Ena/Sm were negative, C-reactive protein 49 mcg/ml (<5).
Surprisingly, the urinalysis was normal with negative proteinuria. Investigation of the oedema showed no renal or liver disorder, nor malnutrition. Nuclear imaging study employing chromium-labelled albumin revealed an enteric loss of 1878 ml of protein per day (normal <14.6 ml/day). Endoscopy was unrevealing. Colonoscopy with biopsies showed unspecific ileitis and colitis. Abdominal CT scan showed portal vein thrombosis with progression to superior and inferior mesenteric vein and splenic vein (Figure 1). Antithrombin III, protein C and S were normal; lupus anticoagulant 1.19 (<1.2); anticardiolipin antibody was positive (IgG 30.5/IgM 50: <10).
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Our patient meets the criteria for SLE and for APS [1,4]. APS is characterized by lupus anticoagulant, anticardiolipin antibodies, vascular thrombosis, thrombocytopaenia and recurrent foetal losses [4]. APS occurs frequently in patients with SLE [4].
Hirohata et al. [5] described a patient with portal vein thrombosis associated with APS. In our patient, the loss of protein may be explained by the increased intestinal congestion caused by portal vein thrombosis. The increase of the portal vein pressure causes increased intestinal congestion and lymph production and subsequent protein leakage [6]. A normal lymphocyte count, elevated serum cholesterol and absence of lymphangiectasia on intestinal biopsy help distinguish lupus-associated PLE from PLE due to direct or indirect lymphatic obstruction [3]. Normal endoscopy and mucosal biopsy can rule out protein loss due to mucosal disruption [3].
Corticosteroids have been demonstrated to dramatically improve the course of PLE, particularly when signs of an inflammatory disease are present [3,6]. Another therapeutic option is octreotide, which reduces hepatic and splanchnic blood flow, lowering portal pressure [6,7]. The patient's symptoms improved with daily corticosteroid, octreotide and anticoagulation with warfarin.
Conflict of interest statement. None declared.
Department of Nephrology Hospital das Clínicas University of São Paulo São Paulo Brazil
References
- Gill JM, Quisel AM, Rocca PV et al. Diagnosis of systemic lupus erythematosus. Am Fam Physician 2003; 68: 2179–2186[Web of Science][Medline]
- Mills JA. Systemic lupus erythematosus. N Engl J Med 1994; 330: 1871–1879
[Free Full Text] - Perednia DA, Curosh NA. Lupus associated protein-losing enteropathy. Arch Intern Med 1990; 150: 1806–1810
[Abstract/Free Full Text] - Levine JS, Branch DW, Rauch J. The antiphospholipid syndrome. N Engl J Med 2002; 346: 752–763
[Free Full Text] - Hirohata Y, Murata A, Abe S, Otsuki M. Portal vein trombosis associated with antiphospholipid syndrome. J Gastroenterol 2001; 36: 574–578[CrossRef][Web of Science][Medline]
- Landzberg BR, Pochapin MB. Protein-losing enteropathy and gastropathy. Current Treatment Options in Gastroenterology 2001; 4: 39–49[Medline]
- McQuaid KR. Alimentary tract. In: Tierney Jr, LM, McPhee SJ, Papadakis MA. Current Medical Diagnosis and Treatment, 40th edn. McGraw-Hill, San Francisco, California, USA, 2001; 559–661
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