Case Report 201 A PREGNANT FEMALE WITH FIBRILLARY GLOMERULONEPHRITIS AND LIVER CIRRHOSIS Chi-Yuan Hung*, Chi-Jen Wu*, Han-Hsiang Chen*, Jui-Chi Yeh*, Tsang-En Wang**, Jeffrey Tzen***, Yi-Chou Chen* Fibrillary glomerulonephritis is a morphologic diagnosis in which fibrillar aggregates resemble amyloid but are negative for Congo red stain. Extrarenal aggregates are reported but uncommon. We describe a 29-year-old female with nephrotic syndrome diagnosed at 22 weeks of pregnancy. After a premature delivery, she had a renal was interpreted as showing fibrillary glomerulonephritis. She had also had a partial hepatectomy to remove a 2 cm lesion in right hepatic lobe. The liver parenchyma had a particular pattern of cirrhosis. In addition, there were Congo-red-negative microfibrils in the fibrotic areas of the liver similar to those seen in the renal glomeruli. In both organs, the fibrils were about 13.5 to 16.5 nm in diameter and haphazardly arranged. The patient had hypocomplementemia, which is rare in previous reports of fibrillary glomerulopathy. Hypocomplementemia is known to be associated with poor clearance of immune complexes, but it is unclear whether this might have played a role in our patient’s fibrillary glomerulopathy. (Acta Nephrologica 2006; 20: 201-206) Key words: fibrillary glomerulonephritis; nephrotic syndrome; liver cirrhosis; hypocomplementemia Division of Nephrology* and Gastroenterology**, Department of Internal Medicine; Division of Pathology***, Mackay Memorial Hospital, Taipei, Taiwan Received: December, 2005 Revised: March, 2006 Accepted: March, 2006 Address reprint request to: Dr. Yi-Chou Chen, No. 92, Section 2, Chung-Shan North Road, Taipei, Taiwan Tel: 886-2-25433535 Fax: 886-2-25433535 ext. 3572 E-mail: [email protected] INTRODUCTION Fibrillary glomerulonephritis (FGN) is a morphologic diagnosis in which immune aggregates in the glomeruli are organized into fibrils which resemble amyloid on hematoxylin and eosin or periodic acid–Schiff staining but are Congo red- and thioflavin T-negative. Rosenmann and Eliakim first described this form of glomerulonephritis in 1977.1 Two morphological patterns have been described (Iskandar et al. 1992;2 Alpers 1993,3 1994;4 Fogo et al. 19935). A random arrangement of fibrils with a diameter of 15 to 30 nm is designated as fibrillary nephropathy. When fibrils are arranged in an ordered parallel array with 30 to 60 nm in diameter, the diagnosis is immunotactoid glomerulopathy (ITG). The difference of FGN and ITG is based on electron microscopic findings, the former show randomly deposited fibrils, whereas the latter presents parallel arranged bundles with microtubular substructure.6 There is considerable overlap between these two forms. The mechanism of fibrillogenesis in immunoglobin deposits in general and particularly deposition in these two forms is unknown.7 We report a patient with FGN who also had fibrillary deposits in the liver. The liver parenchyma had a particular of pattern cirrhosis and there were Congored- negative microfibrils in the fibrotic areas of the liver similar to those seen in the renal glomeruli. In both organs, the fibrils were about 13.5 to 16.5 nm in diameter and haphazardly arranged. The patient had hypocomplementemia, which is rare in previous reports of FGN. Hypocomplementemia is known to be associated with poor clearance of immune complexes, but it is unclear whether this might have played a role in our patient’s fibrillary glomerulopathy. CASE A 29-year-old female had a past history of irregular menstrual period which had been treated with conjugated estrogen and medroxyprogesterone acetate for one year. She came to our hospital complaining of ankle edema and frothy urine for 6 weeks. She was found to be pregnant at 16 weeks of gestation. Blood pressure was 148/92 mmHg. She had no medical history of hypertension before. The ankle edema was attributed 202 C. Y. HUNG, C. J. WU, H. H. CHEN, et al. Vol. 20, No. 3, 2006 to pregnancy but worsened gradually during the recent weeks. Pre-eclampsia was not considered because there was an elevated blood pressure in the first 20 weeks of pregnancy. She returned 6 weeks later with a fever of 39°C and complaining of chills and worsening edema. Blood pressure was 201/112 mmHg. Laboratory data (Table 1) included a serum albumin of 1.6 gm/dl, total protein 2.9 gm/dl, serum cholesterol 296 mg/dl, triglycerides 76 mg/dl, and urinary protein 24.7 g/day. Serum and urine protein electrophoresis were evaluated and no monoclonal gammopathy was found. Serum glucose was 76 mg/dl, blood urea nitrogen 16 mg/dl, creatine 0.5 mg/dl, AST 42 u/l, ALT 25 u/l, and bilirubin was mildly elevated at 0.5/1.7 mg/dl (direct/total). Hemoglobin was 10.1 gm/dl and hematocrit 28.6%. In her urine sediment there were 5 RBC/HPF and 26 WBC/HPF. Bacteria were also present. No mention was made of casts. The immunologic findings (Table 1) included a negative anti-nuclear antibody (ANA) and anti-double stranded DNA. C3 (35 mg/dl), C4 (
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