==== Front Ann Saudi MedAnn Saudi MedAnnals of Saudi Medicine0256-49470975-4466King Faisal Specialist Hospital and Research Centre 2640980410.5144/0256-4947.2015.263asm-3-263Case ReportGuillian-Barre syndrome as the initial presentation of systemic lupus erythematosus – case report and review of literature Nadri Quaid Althaf Mohammed Mahdi Section of Nephrology, Department of Medicine, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi ArabiaCorrespondence: Quaid Johar Nadri, MD, MBC 46 Department of Medicine King Faisal Specialist Hospital and Research Centre, PO Box 3354 Riyadh 11211, Saudi Arabia, T: 966-11-442-7492 F: 966-11-442-7499, qnadri@kfshrc.edu.saMay-Jun 2015 35 3 263 265 Copyright © 2015, Annals of Saudi Medicine2015This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.A number of neurological entities have been associated with systemic lupus erythematosus (SLE). Gullian-Barre syndrome (GBS) as a presenting feature of SLE remains uncommon with just 9 cases reported in the last half-century with the first case reported in 19641–9 (Table 1). We report a young female presenting with GBS in whom SLE and WHO class V lupus nephritis (LN) was subsequently diagnosed. The neurological symptoms partially responded to pulse methylprednisone, intravenous immunoglobulin (IVIG) and plasmapheresis. ==== Body A 23-year-old Saudi female with no prior medical illness presented with a new onset of progressive motor weakness of all four extremities in an ascending pattern associated with increasing difficulty in standing up and walking over a period of 3 days. There were no sensory symptoms. Additionally, there was increasing lower limb swelling. Upon admission there was further deterioration of her weakness to profound quadriparesis with areflexia. Fortunately there was no evidence of respiratory muscle involvement and FEV1 was monitored closely with spirometry. History was negative for any recent gastroenteritis, flu-like symptoms, travel or recent immunizations. Family history was negative for renal disease and connective tissue disease. Physical examination was unremarkable except for motor weakness with a power of 1/5 in all four limbs. Deep tendon reflexes were absent and plantar reflexes were flexor. Sensation was intact. Laboratory investigations revealed hemoglobin 96 g/L, white cell count 8.29×109/L, platelets 322×109/L, ESR 68 mm/hr, CRP 1.0 mg/L. Urinalysis showed +2 protein, no casts, 0–3 red blood cells/hpf. 24-hour proteinuria was 6.05 g/24 hours. Serum creatinine 41 μmol/L, urea 7.1 mmol/L, albumin 28 g/L. Liver function tests were normal. Immunologic tests revealed ANA positive with titer of 1:1280 speckled pattern, double stranded DNA antibody of 28.2 U/mL (normal<20), and a border-line positive anti-Smith antibody. Both C3 and C4 were normal. Serum cryoglobulin was absent. CSF revealed a normal cell count, total protein, and lactate. Oligoclonal immunoglobulin bands were not present. Serology was negative for hepatitis B, hepatitis C, cytomegalovirus and HIV. A renal biopsy was performed given the heavy proteinuria, all the glomeruli showed diffuse thickening of the capillary basement membrane associated with mesangial expansion. Some of the glomeruli showed segmental scaring. There was no increase in cellularity, proliferation, crescents or necrosis. A foci of mild tubular loss with replacement by fibrosis was noted. No significant interstitial inflammation was noted. Immunofluorescence 1 to 2+ glomerular capillary wall and mesangial staining for IgG, IgA, C3, C1q, kappa and lambda gave the characteristic full house immunofluorescence for LN. Fibrinogen and IgM were negative. Electron microscopy was not done as there was inadequate tissue. The findings were consistent with membranous nephropathy, suggesting WHO class V lupus nephritis. MRI brain and whole spine were normal. Electromyogram and nerve conduction revealed characteristic findings of a demyelinating polyneuropathy. GBS was diagnosed on the basis of the Asbury criteria10 and the patient also fulfilled the criteria of the American College of Rheumatology case definitions for GBS.11 The patient received intravenous pulses of methylprednisone 1 g daily for 3 days in addition to 9 cycles of plasmapheresis and IVIG for a total of 2 g/kg. Furthermore, the patient was prescribed prednisone 60 mg daily for 6 weeks and thereafter tapered to a maintenance dose of 5 mg daily. She was discharged on prednisone and physiotherapy. The patient had substantial resolution of weakness regaining motor power to 3 of 5 in all extremities after 6 months, and on the 8-year follow-up her motor power was around 4 of 5. Lupus nephritis was in remission on maintenance mycofenolate mofetil and steroid. DISCUSSION The prevalence of SLE with GBS has been reported to be between 0.6% and 1.7%.12 However, to the best of our knowledge this is the tenth case to be reported in the last 50 years where the initial manifestation of SLE was GBS, thus making it rare and notable. In our patient the diagnosis of SLE was made with the presence of hemolytic anemia, renal involvement, positive ANA and positive ds-DNA antibody, meeting the minimum 4 of 11 criteria for the diagnosis of SLE by the American College of Rheumatology. In addition to these findings renal biopsy consistent with class V LN.13 The pathogenesis of GBS as a manifestation of active SLE is not clear, however both cell-mediated and humoral processes may play a significant role.14 When GBS and LN present concurrently, the efficacy of prednisone as a single agent for therapy is insufficient in about 50% of the cases.4 In the setting of GBS alone plasma exchange and IVIG have been established as standard therapy with satisfactory patient outcomes.15 The mechanism of action of IVIG in autoimmune disease was unclear, but most likely due to binding and neutralization of autoantibodies by anti-idiotypes in the IVIG preparation. IVIG also retards antibody secretion and modifies T cell function.16 Currently, there is no ideal treatment combination that has been proven to treat GBS in the context of SLE. In our patient the neurological symptoms resolved with plasmapheresis, IVIG and corticosteroid treatment. Cyclophosphamide was not administered, although others have reported success when it was employee.4,5 Interestingly, our patient did not have evidence of recent or intercurrent infection that may have been the trigger factor for autoantibody cross-reaction. Our case highlights the importance of early diagnosis and prompt institution of therapy in the form of plasmapheresis, IVIG and corticosteroids in this rare clinical presentation, in which a sustained clinical response was achieved. Table 1 Guillain-Barre syndrome (GBS) as the presenting symptom of systemic lupus erythematosus. Case No./Year Age/Sex Renal Involvement Associated neurological manifestation Treatment Outcome 1/1964 32/F none Anterior radiculopathy CS Full but slow recovery, able to return to premorbid functional state 2/1980 22/F none Bilateral cranial nerve V, IX, X involvement CS Death secondary to tracheostomy 3/1986 23/F none Cranial Nerve Involvement, recurrent GBS ACTH and CS Slow recovery with recurrence at 2 and 3 years 4/1989 40/M Membranous GN Tonic, clonic seizure CS, IV CYP, PE Full recovery, able to walk with frame, persistent bilateral foot drop 5/1999 23/M Membranous lupus Nephritis (WHO class V) none PE, CS, IV-CYP Complete resolution of GBS but recurrence of lupus nephritis and progression to end-stage renal disease 6/2001 20/F Mesangiocapillary lupus nephritis with a membranous component (WHO class IV.C) Cranial Nerve Involvement IVIG, CS, IV CYP Complete resolution of GBS and lupus symptoms 7/2003 33/F none Cranial Nerve Involvement CS Complete resolution 8/2009 28/F none Paresthesia in distal limbs CS, PE Complete resolution 9/2013 20/F Renal biopsy not done but had proteinuria Cranial Nerve Involvement PE, CS, IVIG, IV CYP Complete resolution 10/2013 23/F Membranous lupus Nephritis (WHO class V) none PE, CS, IVIG Near complete resolution CS-Corticosteroid, IV CYP-intravenous Cyclophosphamide, PE-Plasma exchange ==== Refs REFERENCES 1 Gargour G MacGaffey K Locke S Stein MD Anterior Radiculopathy and Lupus Erythematosus Cells: Report of Case British Medical Journal 1964 2 5412 788.4 2 Moreira FP Nascimento O Cinnicinatus D Porto F Freitas M Santos P Guillain-Barré syndrome as a manifestation of systemic lupus erythematosus. Report of a case Arquivos de neuropsiquiatria 1980 38 2 165 3 Millette TJ Subramony SH Wee AS Harisdangkul V Systemic lupus erythematosus presenting with recurrent acute demyelinating polyneuropathy European neurology 1986 25 6 397 402 3024988 4 Chaudhuri KR Taylor IK Niven RM Abbott RJ A case of systemic lupus erythematosus presenting as Guillain-Barre syndrome British journal of rheumatology 1989 28 5 440 2 2790407 5 Vaidya S Jasin HE Logan J Systemic lupus erythematosus and guillain-barre syndrome Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases 1999 5 6 349 53 19078429 6 van Laarhoven HW Rooyer FA van Engelen BG van Dalen R Berden JH Guillain-Barre syndrome as presenting feature in a patient with lupus nephritis, with complete resolution after cyclophosphamide treatment Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association 2001 16 4 840 2 11274284 7 Ait Benhaddou E Birouk N El Alaoui-Faris M Mzalek-Tazi Z Aidi S Belaidi H Acute Guillain-Barre-like polyradiculoneuritis revealing acute systemic lupus erythematosus: two case studies and review of the literature Revue neurologique 2003 159 3 300 6 12703046 8 Hsu TY Wang SH Kuo CF Chiu TF Chang YC Acute inflammatory demyelinating polyneuropathy as the initial presentation of lupus The American journal of emergency medicine 2009 27 7 900e3 5 10.1016/j.ajem.2008.11.006 19683133 9 Santiago-Casas Y Peredo RA Vila LM Efficacy of low-dose intravenous cyclophosphamide in systemic lupus erythematosus presenting with Guillain-Barre syndrome-like acute axonal neuropathies: report of two cases Lupus 2013 22 3 324 7 10.1177/0961203313476358 23439473 10 Asbury AK Cornblath DR Assessment of current diagnostic criteria for Guillain-Barre syndrome Annals of neurology 1990 27 Suppl S21 4 2194422 11 The American College of Rheumatology nomenclature and case definitions for neuropsychiatric lupus syndromes Arthritis and rheumatism 1999 42 4 599 608 10.1002/1529-0131(199904)42:4<599::AIDANR2>3.0.CO;2-F 10211873 12 Korn-Lubetzki I Abramsky O Acute and chronic demyelinating inflammatory polyradiculoneuropathy. Association with autoimmune diseases and lymphocyte response to human neuritogenic protein Archives of neurology 1986 43 6 604 8 2424413 13 Hochberg MC Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus Arthritis and rheumatism 1997 40 9 1725 Epub 1997/10/27 10.1002/1529-0131(199709)40:9<1725::AIDART29>3.0.CO;2-Y 9324032 14 Robson MG Walport MJ Davies KA Systemic lupus erythematosus and acute demyelinating polyneuropathy British journal of rheumatology 1994 33 11 1074 7 7981996 15 Hughes RA Wijdicks EF Barohn R Benson E Cornblath DR Hahn AF Practice parameter: immunotherapy for Guillain-Barre syndrome: report of the Quality Standards Subcommittee of the American Academy of Neurology Neurology 2003 61 6 736 40 14504313 14504313 16 Richter C Schnabel A Csernok E De Groot K Reinhold-Keller E Gross WL Treatment of antineutrophil cytoplasmic antibody (ANCA)-associated systemic vasculitis with high-dose intravenous immunoglobulin Clinical and experimental immunology 1995 101 1 2 7 7621588