Renal Vein Thrombosis With Pulmonary Embolism: First Manifestation of Lupus Nephritis

Abstract

References RENAL VEIN THROMBOSIS WITH PULMONARY EMBOLISM: FIRST MANIFESTATION OF LUPUS NEPHRITISSystemic lupus erythematosus (SLE) is a chronic autoimmune disease that can affect almost any organ system, with renal involvement occurring in 90% of patients, while 50% of patients already have renal involvement at diagnosis. 1,2 Venous or arterial thrombosis can occur in SLE either as a result of anti-phospholipid syndrome or as a result of hypercoagulable state, related to nephrotic syndrome. 1,[3][4][5][6][7] The risk factors for thromboembolic complications include hypoalbuminaemia, proteinuria, low plasma anti-thrombin-III (AT-III), increased fibrinogen level, hypofibrinolysis, decreased functional activity of protein C and its cofactor protein S, high haematocrit, thrombocytosis, increased platelet aggregation, hyperlipidaemia and reduced plasma volume.1,3-7 An 11-year-old girl was admitted for a 2 months' history of fatigue and shortness of breath, and exercise-induced dyspnoea, headache and myalgia that occurred within the last 2 weeks. Three months prior to admission, there was a transient oedema of lower extremities. Her height was 141 cm (-1.2 standard deviation (SD) ), weight was 29 kg (-1.4 SD) and body mass index was 14.6 (-1.2 SD); she presented with normal respiratory rate of 20/min, tachycardia (heart rate 111/min), elevated blood pressure (141/88 mm Hg), pallor, oedema of eyelids and lower extremities, and left flank pain with loin tenderness on palpation. Laboratory findings revealed anaemia: red blood cells 2.89 ¥ 10 12 /L (normal 4.10-5.10 ¥ 10 12 /L) and haemoglobin 7.6 g/dL (normal 11.5-15.5 g/ dL . Anti-phospholipid antibodies were negative. Concerning hepatitis B and C status, hepatitis B surface antigen (HBsAg) and antibodies against hepatitis C (anti H-CV) antibodies were negative. There was mild hyperimmunoglobulinaemia (immunoglobulin (IgG) 22.9 g/L; normal 8.4-22.4 g/L) with elevated circulating immune complexes of 63 IU/L (normal <50 IU/L). C3 complement was in the normal range; C4 complement was low (0.13 g/L; normal 0.15-0.4 g/ L). Renal biopsy was performed on day 4, resulting in the histological diagnosis of membranous nephropathy. Immunofluorescence on renal biopsy was positive in all IgG classes and C3 at the basal membrane with negative fibrinogen. Arthritis was diagnosed on magnetic resonance imaging of the joints. Therefore, the girl had severe nephrotic syndrome with left RVT and bilateral pulmonary embolism, and she fulfilled 6 criteria for the diagnosis of SLE (polyserositis, nephropathy, arthritis, haemolytic anaemia and leukopenia, positivity of anti-Smith antibodies and positivity of anti-nuclear antibodies) of 11. 2,3Anti-thrombotic, immunosupressive and supportive therapy were started. She received low-molecular weight heparin (LMWH) 150 IU/kg/day for 3 weeks, three intravenous methylprednisolone pulses (30 mg/kg) followed by seven intravenous cyclophosphamide pulses (750-1000 mg/m 2 ) every 3-4 weeks, and further maintenance therapy with azathioprine (2 mg/kg/day) and...

Fatal embolic events in childhood

“…18 The associated antiphospholipid syndrome has been responsible for pulmonary thromboembolism as the first manifestation of SLE in a 10-year-old boy 19 and postmortem screening for these disorders in children who are found to have thromboemboli at autopsy has been proposed. 16 Pulmonary thromboembolism may also be the first manifestation of SLE due instead to lupus nephritis with nephrotic syndrome, as occurred in an 11-year-old girl with renal vein thrombosis, 20 Patients with severe proteinuria from nephrotic syndrome have a 3.4 times risk of thromboembolism. 21 Increased levels of plasma homocysteine and lipoprotein may also occur in inherited prothrombotic conditions, and increased plasma levels of factors VII, IX and XI coagulant have been associated with thromboses in children.…”

Venous Thrombosis in Rare or Unusual Sites: A Diagnostic Challenge

“…Congenital thrombophilia may also play a role in some instances. [31][32][33][34][35][36][37][38] The picture is insidious with lumbar area pain, renal colic, left side varicocele with or without thrombosis and scrotal swelling and pain. Pulmonary embolism may also occur.…”

“…Pulmonary embolism may also occur. 34,37 Proteinuria, enlarged kidney on sonography, variable renal insufficiency are also present. Renal scintiscan reveals a normal uptake but no or limited discharge curve.…”