Is salt-wasting the long awaited answer to the hyperuricaemia seen in uromodulin storage diseases?

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). 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.