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Saudi Journal of Kidney Diseases and Transplantation
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CASE REPORT  
Year : 2021  |  Volume : 32  |  Issue : 6  |  Page : 1804-1808
Acute Kidney Injury and Nephrotic Syndrome in Guillain-Barre Syndrome: A Rare Clinical Scenario


Department of Nephrology, Nizam’s Institute of Medical Sciences, Hyderabad, Telangana, India

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Date of Web Publication27-Jul-2022
 

   Abstract 


Guillain-Barre syndrome (GBS) is an acute polyradiculoneuropathy, caused by dysregulated immune response following an infectious or noninfectious event. Although cardiovascular, respiratory, and gastrointestinal systems are commonly involved secondary to neuromuscular paralysis, renal manifestations are rare. Acute kidney injury (AKI) can develop in GBS due to acute tubular necrosis secondary to dysautonomia. Minimal change nephrotic syndrome in GBS may be due to T-cell dysregulation and cytokine release attributed to molecular mimicry. Here, we report the case of GBS with simultaneously developed AKI and nephrotic syndrome during the course of disease, which recovered completely in parallel with neurological improvement without any immunosuppressive medications.

How to cite this article:
Shamsudheen MP, Vijayan S, Kuchay A, Taduri G, Guditi S, Karthik R, Das U. Acute Kidney Injury and Nephrotic Syndrome in Guillain-Barre Syndrome: A Rare Clinical Scenario. Saudi J Kidney Dis Transpl 2021;32:1804-8

How to cite this URL:
Shamsudheen MP, Vijayan S, Kuchay A, Taduri G, Guditi S, Karthik R, Das U. Acute Kidney Injury and Nephrotic Syndrome in Guillain-Barre Syndrome: A Rare Clinical Scenario. Saudi J Kidney Dis Transpl [serial online] 2021 [cited 2022 Aug 14];32:1804-8. Available from: https://www.sjkdt.org/text.asp?2021/32/6/1804/352445



   Introduction Top


Guillain-Barre syndrome (GBS), acute inflammatory demyelinating polyradiculoneuropathy (AIDP), is the most frequent cause of acute flaccid paralysis in both adults and children. AIDP is the most widely recognized form of GBS, but the axonal variants (acute motor axonal, acute motor-sensory axonal), and Miller−Fisher syndrome are also well recognized. It is usually preceded by infection or other immune stimulation that induces an aberrant autoimmune response targeting peripheral nerves and their spinal roots. The involvement of both sympathetic and parasympathetic fibers in GBS is usually manifested in cardiovascular, gastrointestinal, respiratory, and other systems. However, only a few cases of renal involvement have been reported in the adult and pediatric population.


   Case Report Top


A 62-year-old male, who is diabetic and hypertensive for 15 years and with a history of acute gastroenteritis one month before current hospital admission, noticed paresthesias and weakness of the right lower limb which progressed to the left lower limb over five days. He was admitted in a local hospital with excessive sweating, transient loss of consciousness, and low blood pressure and on evaluation detected to have renal dysfunction and referred to our center. He had swelling of both legs, shortness of breath, and reduced urine output of 3 days’ duration. He was bedridden at the time of admission and also complained paresthesias of both hands. On examination, he was conscious, cooperative, and afebrile. Pulse rate was 86/min and blood pressure recorded 140/90 mm Hg. Pallor and bilateral pedal edema were present. Fundus examination showed no evidence of diabetic retinopathy. Neurological examination revealed hypotonia, Grade 2 power in both lower limbs, Grade 4 - power in both upper limbs, and generalized areflexia with flexor plantar response. The sensory system and cranial nerve examinations were unremarkable.

Initial investigations showed renal dysfunction (serum creatinine - 6.1 mg/dL), severe metabolic acidosis, and normal serum electrolytes. Hence, hemodialysis (HD) was initiated through right jugular catheter. Detailed neurological evaluation revealed normal creatine phosphokinase levels and normal magnetic resonance imaging of the brain and spinal cord. However, nerve conduction study was suggestive of axonal predominant sensory motor polyneuropathy and cerebrospinal fluid analysis showed albuminocytological dissociation, hence GBS was suspected. Meanwhile renal function recovered with five sessions of HD and physiotherapy was continued.

Detailed investigation [Table 1] showed 3+ albumin in dipstick urine test with no active urinary sediments, massive proteinuria (spot urine PCR 19.41, 24 h urine protein 13.69 g), severe hypoalbuminemia, normal lipid/thyroid profile, and normal sized kidneys in imaging. Work up for paraproteinemia, autoimmune disease and malignancy was negative. Percutaneous renal biopsy was performed which showed normal glomeruli with mild tubular injury and focal interstitial inflammation in light microscopy, without evidence of diabetic nephropathy or amyloid deposition [Figure 1]. Immunofluorescence was negative for immunoglobulins and complements. This suggested probable minimal change disease with resolving acute tubular necrosis, but no immunosuppressant drugs were started at that time.
Table 1. Investigation details.
ALP: Alkaline phosphatase, ANCA: Anti-neutrophil cytoplasmic antibodies, ANA: Antinuclear antibody, c-ANCA: Cytoplasmic ANCA, CPK: Creatine phosphokinase, CSF: Cerebrospinal fluid, dsDNA: Doublestranded DNA, GRBS: General random blood sugar, Hb: Hemoglobin, HBsAg: Hepatitis B surface antigen, HCV: Hepatitis C virus, HDL: High-density lipoprotein, HIV: Human immunodeficiency virus, IFE: Immunofixation electrophoresis, K: Potassium, LDH: Lactate dehydrogenase, LDL: Low-density lipoprotein, MRI: Magnetic resonance imaging, NA Sodium, NCCT: Noncontrast computed tomography, p-ANCA: Perinuclear ANCA, PCR: Protein/creatinine ration, RBC: Red blood cell, RBS: Random blood sugar, SCr: Serum creatinine, SGOT: Serum glutamic-oxaloacetic transaminase, SGPT: Serum glutamic-oxaloacetic transaminase, SPEP: Serum protein electrophoresis, TGL: Triglyceride, TSH: Thyroid-stimulating hormone, Urine PE: Urine protein electrophoresis, USG: Ultrasonography, WBC: White blood cell.


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Figure 1. Light microscopy showing normal glomeruli with mild tubular injury and focal interstitial inflammation.


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Around three weeks after hospitalization muscle power started improving and peripheral edema completely disappeared. We rechecked proteinuria which was only 625 mg. After around one month of hospitalization, the patient was discharged when he started walking without support [Figure 2] shows graphical representation of clinical course in hospital).
Figure 2. Graphical representation of clinical course in hospital.

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The authors obtained all appropriate consent forms from the patient for the publication of this case report.


   Discussion Top


The GBS, also known as Landry’s paralysis,[1] is an immune-mediated disorder of the nervous system characterized commonly by acute or subacute onset of fairly symmetric muscle weakness accompanied by absent or depressed deep-tendon reflexes[2] and usually having a spontaneous recovery. GBS occurs worldwide with an overall incidence of 1-2/100,000 population per year[3] and increases linearly with age, and men are about 1.5 times more affected than women.[4]

Molecular mimicry is a dual recognition of a microbial component and an antigen of the host and is the mechanism by which infections trigger cross-reactive antibodies or T-cells resulting in autoimmune disease.[5] This phenomenon of molecular mimicry is proven in GBS.[6] Following a susceptible event leading to GBS, like infection with Campylobacter jejuni infection, activation of humoral and cellular arms of the immune response occurs. This include (i) accumulation of antiganglioside antibodies at the nodes of Ranvier (ii) recruitment of T-cells by antiganglioside antibodies-producing B cells, and (iii) activated T-cells that produce cytokines which damage the blood-nerve barrier.[7]

GBS may affect cardiovascular, respiratory, and gastrointestinal system which is mainly secondary to neurological dysfunction, but a few cases of renal involvement in the form of nephrotic syndrome secondary to immune dysregulation have also been described in the literature. Acute kidney injury (AKI) can also occur in cases with severe GBS, particularly in those with dysautonomia, causing high mortality rate. In a study by Khajehdehi et al, among 30 consecutive cases, six out of seven cases with severe GBS and AKI had dysautonomia and became oliguric while being in a hypotensive state and mortality was higher in such cases. Other causes of AKI include acute interstitial nephritis secondary to preceding infection or related to nonsteroid anti-inflammatory drugs or native medications used during the course of illness.[8]

It is postulated that minimal change disease is a disorder of T-cells, which release a cytokine or permeability factor that injures podocyte foot process. Specific autoantibodies are thought arise from molecular mimicry from a preceding infections and in occasional patients, these antibodies cross react with one or more antigens expressed on podocyte cell membranes. Abnormal cell mediated and humoral immune mechanisms play a role in the pathogenesis of both GBS and nephrotic syndromes including minimal change disease.

Tateyama et al searched for English language literature from 1946 to 2012 and found 33 cases of GBS and chronic inflammatory demyelinating polyneuropathy with nephrotic syndrome. On analysis, they found following features: (i) male preponderance (84.8%); (ii) motor dominant impairments with favorable final outcomes; (iii) major histological diagnoses of renal biopsies were membranous nephropathy, focal segmental glomerulosclerosis and minimal change disease, and (iv) proteinuria occurring simultaneously with neurological impairment and ameliorating along with neurological improvement in the majority of the cases.[9] These findings suggest that GBS and nephrotic syndrome might be a specific clinical entity caused by immune reactions to antigens shared by peripheral nerves and the glomerulus.

In nephrotic syndrome associated with GBS, the renal lesion usually appears to be selflimiting, and resolve along with improvement of the neuropathy. In such cases immunosuppressive therapy is not required for the renal lesion. But GBS should be treated with either IVIG or plasma exchange, if indicated. In few cases associated with the chronic and/or relapsing neuropathies, the renal disease appears to persist as well. The optimal treatment in such cases from nephrology standpoint remains unknown.[10]

Our patient with history of preceding infection, was presented with areflexic flaccid quadri-paresis with dysautonomic symptoms and neurological evaluation was suggestive of axonal variant of GBS. AKI developed at the time of presentation might be acute tubular necrosis secondary to dysautonomia. Further detailed evaluation showed massive proteinuria without active urinary sediments and hypoalbuminemia, hence common secondary causes were ruled and performed percutaneous renal biopsy, which was absolutely normal even without evidence of diabetic nephropathy, hence considered possibility of minimal change disease. Without starting any immunosuppressive medications, renal manifestations were recovered in parallel with improvement of neurological dysfunction.


   Conclusion Top


AKI in GBS can be due to acute tubular necrosis secondary to dysautonomia. Minimal change nephrotic syndrome developed due to molecular mimicry is usually self-limiting and resolve along with improvement of the neuropathy without any immunosuppressive therapy.

Conflict of interest: None declared.



 
   References Top

1.
Ropper AH, Samuels M. Adams and Victor’s Principles of Neurology. 9th ed. New York: McGraw-Hill Medical Publishing Division; 2005. p. 1117-27.  Back to cited text no. 1
    
2.
Gorson KC, Ropper AH. Guillain-Barré syndrome (acute inflammatory demyelinating polyneuro pathy) and related disorders. In: Katirji B, Kaminski HJ, Preston DC, et al, editors. Neuromuscular Disorders in Clinical Practice. Boston Mass: Butterworth-Heinemann; 2002. p. 544-66.  Back to cited text no. 2
    
3.
Yuki N, Hartung HP. Guillain-Barré syndrome. N Engl J Med 2012;366:2294-304.  Back to cited text no. 3
    
4.
van Doom PA, Ruts L, Jacobs BC. Clinical features, pathogenesis, and treatment of Guillain-Barré syndrome. Lancet Neurol 2008;7:939-50.  Back to cited text no. 4
    
5.
Ang CW, Jacobs BC, Laman JD. The Guillain-Barré syndrome: A true case of molecular mimicry. Trends Immunol 2004;25:61-6.  Back to cited text no. 5
    
6.
Shahrizaila N, Yuki N. Guillain-barré syndrome animal model: The first proof of molecular mimicry in human autoimmune disorder. J Biomed Biotechnol 2011 ;2011:829129.  Back to cited text no. 6
    
7.
Nyati KK, Nyati R. Role of Campylobacter jejuni infection in the pathogenesis of Guillain-Barré syndrome: An update. Biomed Res Int 2013; 2013:852195.  Back to cited text no. 7
    
8.
Khajehdehi P, Shariat A, Nikseresht A. Acute renal failure due to severe Landry-Guillain-Barre syndrome. Nephrol Dial Transplant 1998;13: 2388-91.  Back to cited text no. 8
    
9.
Tateyama M, Nakashima I, Suzuki N, et al. Inflammatory demyelinating polyneuropathy with nephrotic syndrome: Report of a case and review of the literature. Clin Exp Neuroimmunol 2013;4:79-88.  Back to cited text no. 9
    
10.
Filippone EJ, Kanzaria M, Bell R, Newman E, L Farber J. Secondary membranous nephropathy associated with Guillain Barré syndrome. Case Rep Nephrol Urol 2013;3:34-9.  Back to cited text no. 10
    

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Correspondence Address:
Gangadhar Taduri
Department of Nephrology, Nizam’s Institute of Medical Sciences, Punjagutta, Hyderabad, Telangana, India.
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1319-2442.352445

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