Stenosis at the Entrance of the Inferior Vena Cava in an 11-Years-Old Boy with Lupus Nephritis

Case Report

Austin J Nephrol Hypertens. 2014;1(2): 1009.

Stenosis at the Entrance of the Inferior Vena Cava in an 11-Years-Old Boy with Lupus Nephritis

Mengxia Li1, Yunyan Shen1, Xueming Zhu2, Qinying Xu1, Yun Zhu1, Meifeng Zeng1, QiuqinXu3, Xiaozhong Li1, Yanhong Li1,4*

1Department of nephrology, Children’s Hospital affiliated to Soochow University, China

2Department of pathology, Children’s Hospital affiliated to Soochow University, China

3Department of ultrasound, Children’s Hospital affiliated to Soochow University, Chi

4Institute of pediatric research, Children’s Hospital affiliated to Soochow University, Suzhou, China

*Corresponding author: Yanhong Li, Department of nephrology, Institute of pediatric research, Children’s Hospital affiliated to Soochow University, Suzhou, Postal code: 215003, China

Received: June 30, 2014; Accepted: July 31, 2014; Published: Aug 04, 2014

Abstract

This case report describes an 11-year-old boy with systemic lupus erythematosus (SLE) presenting with stenosis at the entrance of the inferior vena cava. The concurrent presence of the stenosis of inferior vena cava and lupus nephritis has rarely been reported in children.

Keywords: Childhood-onset systemic lupus erythematosus; Inferior vena caval stenosis; Lupus nephritis; Proteinuria; Vasculitis

Abbreviations

ANA: Antinuclear Antibody; ANCA: Anti-neutrophil Cytoplasmic Antibodies; APS: Antiphospholipid Syndrome; SLE; Systemic Lupus Erythematosus

Introduction

Childhood-onset systemic lupus erythematosus (SLE) is a multisystem autoimmune disease, having a higher frequency of major organ involvement than adults-onset SLE [1,2]. Lupus nephritis affects more than 80% of children with SLE and remains one of the most severe manifestations of SLE associated with considerable morbidity and mortality [3]. Although vascular manifestations are conditions that can occur in patients with childhood-onset SLE and are associated with lupus nephritis [2], stenosis of the inferior vena cava has rarely been reported in children with SLE and lupus nephritis.

Case Presentation

An 11 years old boy was admitted with a history of periorbital and facial edema and swelling of the ankles for three days. There was no history of gross hematuria, fever, facial rash, joint pains, or drug intake. On admission, physical examination was remarkable for periorbital and facial edema. His weight was 28 kg within normal ranges. Temperature was normal. Blood pressure was 140/90 mm Hg. There were no signs of oral ulcer, ecchymosis, petechiae, erythema, lymphadenopathy or joint swelling. The liver and spleen were not palpable below the costal margin. The laboratory results on admission were as follows: normal hemoglobin, 111 g/L; normal total white blood cell count, 5,680/mm3; lymphocyte count, 3,510/mm3; normal platelet count, 290,000/mm3; high erythrocyte sedimentation rate, 66 mm/h; normal C-reactive protein, 0.36 mg/L; normal prothrombin, partial thromboplastin, and thrombin time; and high D-dimer, 3788.0 ug /L. Urinalysis revealed: pH, 5.0; protein, +3; high red blood cell count, 17.7/ul; high white blood cell count, 26.3/ul; high epithelial cell count, 46.5/ul; no casts. The 24h urine total protein was 2181.2 mg. The serum biochemistry analysis showed: normal sodium, 138.8 mmol/L; normal potassium, 4.2 mmol/L; normal chloride, 111 mmol/L; normal blood urea nitrogen, 4.47 mmol/L; normal creatinine, 35.9 μmol/L; low total protein, 42.7 g/L; low albumin, 19.8 g/L; high total cholesterol, 9.49 mmol/L; high triglycerides, 5.77 mmol/L. Venous pH was 7.46; normal HCO3, 19.0 mmol/L; normal base excess, -3.2 mmol/L. Serum complement levels were low, with a C3 of 0.39 (normal 0.79-1.52) g/L and a C4 of 0.01 (normal 0.16–0.38) g/L. Serum immunoglobulin G (IgG), IgA, and IgM were 5.34 (normal 6.36-10.04), 1.52 (normal 0.63-1.79), and 0.86 (normal 0.29-1.41) g/L, respectively. The direct Coomb’s test was positive. The antinuclear antibody (ANA) titer was 1:320; negative test for double-stranded DNA antibody, proliferating cell nuclear antigen antibody, anti-smith antibody, anti-RNP antibody, and anti-cardiolipin antibody. Anti-neutrophil cytoplasmic antibodies (ANCA) test showed positive perinuclear ANCA (pANCA) and negative for cytoplasmic ANCA (cANCA), myeloperoxidase ANCA (MPO-ANCA), and proteinase 3 ANCA (PR3-ANCA). Blood cultures were negative for bacteria. Epstein - Barr virus DNA was negative. The CD4/CD8 ratio was 0.5 (normal 1.0-1.9). Chest X-ray showed increase in interstitial lung markings, especially on left side. The left diaphragm and costophrenic angle were obscured. An abdominal ultrasound displayed small peritoneal effusion. The stenosis of the entrances of inferior vena cava and hepatic vein was observed on the echocardiogram. The inner diameter of inferior vena cava was 11 mm, the entrance of inferior vena cava into the right atrium was 4.5 mm in diameter, and the maximal blood flow velocity of the inferior vena cava was 1.68 m/s (normal <1.0 m/s). The blood flow velocity was >1.2 m/s, indicating that the entrance was narrow on the echocardiogram. The diameter of hepatic portal vein was 3.8 mm, the entrance of hepatic vein into the inferior vena cava was 4.1 mm, and the mean blood flow velocity was 1.09 m/s (normal <1.0 m/s) (Figure 1).

Citation: Li M, Shen Y, Zhu X, Xu Q, Zhu Y, Zeng M, et al. Stenosis at the Entrance of the Inferior Vena Cava in an 11-Years-Old Boy with Lupus Nephritis. Austin J Nephrol Hypertens. 2014;1(2): 1009. ISSN:964 2381-8