Letter to the Editor Nephron 1998;80:361–362 A Case of Guillain-Barré Syndrome Complicated by Nephrotic Syndrome and p-ANCA Positivity Kenan Keven Gökhan Nergisog ˇlu S ¸ ehsuvar Ertürk Mustafa KÈlÈçkap Kenan Ates ¸ Harun Akar Bülent Erbay Oktay Karatan Neval Duman A. Ergün Ertug ˇ University of Ankara, Faculty of Medicine, Ibni Sina Hospital, Department of Nephrology, Ankara, Turkey Kenan Keven 1. Mesa BatÈ Sitesi A3 Blok No 5 TR–06370 BatÈkent, Ankara (Turkey) Tel. +90 312 2553914, E-Mail tp920076@dialup.ankara.edu.tr ABC Fax + 41 61 306 12 34 E-Mail karger@karger.ch www.karger.com © 1998 S. Karger AG, Basel 0028–2766/98/0803–0361$15.00/0 Accessible online at: http://BioMedNet.com/karger Dear Sir, Acute inflammatory demyelinating poly- radiculoneuropathy, Guillain-Barré syn- drome (GBS), is characterized clinically by weakness or ascending paralysis affecting more than one limb due to demyelination. Sixty percent of the patients with GBS have a history of antecedent infectious disease such as upper respiratory infection, common viral infections including Epstein-Barr virus (EBV), cytomegalovirus (CMV), hepatitis B or C [1]. Although the pathogenesis remains obscure, it is generally held that GBS is an autoimmune disorder [2]. GBS may be asso- ciated with glomerulonephritis and the clini- cal presentation can vary from asymptomat- ic urinary abnormalities to full blown ne- phrotic syndrome. An extensive review of the literature showed that renal involve- ment, most commonly membranous glomer- ulonephritis, has repeatedly been described in GBS [3]. However, to the best of our knowledge, perinuclear antineutrophil cyto- plasmic antibody (p-ANCA) has never been reported in association with GBS and ne- phrotic syndrome due to membranous glo- merulonephritis. A 57-year-old man was referred to our hospital with a history of progressive weak- ness of the limbs and inability to walk for a week. Two days before admission, ankle ede- ma had appeared. There was no history of kidney disease, exposure to any toxic sub- stance, or recently observed infectious dis- ease. Vital signs were as follows: temperature 36.8 ° C; pulse rate 76/min, and blood pres- sure 110/70 mm Hg. There was no patholog- ic finding but 4+ bilateral pretibial edema on physical examination. On neurologic exami- nation, the patient’s motor power was graded as 4–5/5 on the upper extremities, and 1/5 and 3/5 on the proximal and distal lower extremities, respectively. Deep tendon reflexes were absent on the lower and intact on the upper extremities. No pathologic re- flexes and sensorial disturbances were found. Nerve conduction studies revealed marked slowing of motor nerve conduction, distal motor latency prolongation, and mul- tifocal conduction blocks compatible with a demyelinating polyneuropathy of the lower extremities. Urinalysis showed 3+ protein- uria, and 3–4 erythrocytes and 5–6 granular casts on the sediment. Proteinuria was 4 g/ day. Serum biochemistry revealed the fol- lowing: blood urea nitrogen 55 mg/dl; creati- nine 2.0 mg/dl; total protein 4.1 g/dl; albu- min 1.4 g/dl; total cholesterol 368 mg/dl, and triglyceride 613 mg/dl. The sedimentation rate was 100 mm/h, white blood cell count 6,500/mm 3 and hemoglobin level 12.7 g/dl. No abnormalities were found on abdominal ultrasonography, thoracic computerized to- mography and cervical, thoracic, and lum- bar magnetic resonance imaging examina- tions. Renal venous Doppler ultrasonogra- phy was normal. Hepatitis B, C, CMV-IgM, EBV-IgM were negative and hepatitis B anti- body, CMV-IgG, EBV-IgG were positive. Fecal occult blood was negative on three consecutive screenings. Double contrast co- lonography and sigmoidoscopy were normal. Prostate examination was normal and pros- tate-specific antigen was 0.62 ng/ml. Gastro- duodenoscopy and biopsies from the antrum and corpus showed chronic atrophic gastri- tis. Vitamin B 12 and folic acid levels were normal in range. Antinuclear antibody, anti- double-stranded DNA, glomerular basal membrane antibody and c-ANCA were neg- ative, but p-ANCA was positive. Serum im- munoglobulin levels (IgG, IgA, IgM) and complement levels (C3, C4) were normal in range. Lumbar puncture was done and to- tal protein was 30.4 mg/dl, albumin was 14.5 mg/dl, no cell or microorganism was obtained from the cerebrospinal fluid. Mem- branous glomerulonephritis was diagnosed by renal biopsy on the basis of light and immune fluorescein microscopy findings. The patient was followed for 4 weeks with supportive treatment in the hospital. Substantial improvement in muscle strength was noted. Proteinuria disappeared. Serum total protein, albumin, lipid levels, creati- nine, and blood urea nitrogen returned to normal levels before the patient was dis- charged from hospital. At the same time, p-ANCA was also retested and was negative. At the last visit performed 6 months later, neurological findings were all normal, and the nephrotic syndrome was in remission. In this case, p-ANCA positivity could be an important sign of vasculitic disorders such as microscopic polyangitis, Churg- Strauss syndrome, but less probably classic polyarteritis nodosa and Wegener’s granulo- matosis. However, there was no finding con- sistent with the presence of these diseases. Downloaded by: University of Illinois at Chicago 128.248.155.225 - 1/10/2017 8:24:08 AM