|Year : 2019 | Volume
| Issue : 4 | Page : 286-288
Guillain–Barre syndrome in an ABO-incompatible renal allograft recipient during pregnancy treated with plasma exchange
Praveen Kumar Etta1, Sreepada Subhramanyam1, Vivek Narain Mathur2, Karopadi Shivanand Nayak1
1 Department of Nephrology and Renal Transplantation, Virinchi Hospitals, Hyderabad, Telangana, India
2 Department of Neurology, Virinchi Hospitals, Hyderabad, Telangana, India
|Date of Submission||17-Jun-2019|
|Date of Acceptance||26-Nov-2019|
|Date of Web Publication||31-Dec-2019|
Dr. Praveen Kumar Etta
Department of Nephrology and Renal Transplantation, Virinchi Hospitals, Hyderabad, Telangana
Source of Support: None, Conflict of Interest: None
Guillain–Barre syndrome (GBS) is an extremely rare complication after solid organ transplantation (SOT) and its clinical course can be more severe in them. Most of the cases of GBS in SOT have been associated with Cytomegalovirus infection. GBS presenting after an ABO-incompatible (ABOi) transplant and during pregnancy has never been reported in the past among SOT recipients. We report a case of a 27-year-old female ABOi renal transplant recipient who presented with acute flaccid paralysis all four limbs during the second trimester of pregnancy, diagnosed to have GBS. Pregnancy and vaccination might have played a role in precipitating GBS in our patient. She was treated with plasma exchange and experienced GBS treatment-related fluctuations but finally recovered completely without any adverse pregnancy outcome.
Keywords: ABO-incompatible, Guillain–Barre syndrome, pregnancy, renal transplantation
|How to cite this article:|
Etta PK, Subhramanyam S, Mathur VN, Nayak KS. Guillain–Barre syndrome in an ABO-incompatible renal allograft recipient during pregnancy treated with plasma exchange. Indian J Transplant 2019;13:286-8
|How to cite this URL:|
Etta PK, Subhramanyam S, Mathur VN, Nayak KS. Guillain–Barre syndrome in an ABO-incompatible renal allograft recipient during pregnancy treated with plasma exchange. Indian J Transplant [serial online] 2019 [cited 2020 Jan 18];13:286-8. Available from: http://www.ijtonline.in/text.asp?2019/13/4/286/274601
| Introduction|| |
Guillain–Barre syndrome (GBS) is an acute ascending form of polyradiculoneuropathy characterized by symmetrical flaccid areflexic motor paralysis with or without sensory disturbances. If not treated promptly, it may progress to respiratory muscle paralysis or autonomic dysfunction leading sometimes to death. GBS is extremely rare after solid organ transplantation (SOT), probably due to their immunocompromised state. GBS presenting after an ABO-incompatible (ABOi) transplant and during pregnancy has never been reported in the past among SOT recipients. We herein report a case of GBS diagnosed during pregnancy in an ABOi renal transplant (RT) recipient who recovered completely with plasma exchange (PLEX).
| Case Report|| |
A 27-year-old primigravida with 20 weeks of amenorrhea who had received a live-related (one haplomatch) ABOi RT in April 2014 presented with symmetric weakness of both lower limbs with progression to upper limbs over 5 days. She also had significant sensory symptoms such as tingling and numbness of both lower limbs. There was no bowel and bladder involvement. She received tetanus toxoid (TT) vaccine about 4 weeks before her illness. She denied fever, prodromal illness, cough, or diarrhea. She was on triple immunosuppressive therapy with prednisolone, azathioprine, and tacrolimus and having normal graft function. Her basic kidney disease was presumed as chronic interstitial nephritis. She received a single dose of rituximab (375 mg/m2), four sessions of PLEX, and intravenous immunoglobulins (IVIGs) as desensitization protocol and low-dose antithymocyte globulin (ATG: 1 mg/kg) as induction at the time of RT with target anti-ABO titer of 1:8 (baseline titer: 1:64). Her post-RT period was uneventful till the recent past.
On examination, she had severe loss of muscle power in both lower and upper limbs (2/5 and 3/5, respectively). There was generalized areflexia, and sensory examination was normal. Plantar reflexes were flexor, and cranial nerves were intact. The routine laboratory evaluation was normal except for mild anemia.
The workup for triggering causes of GBS such as infections and immunological diseases was negative. Serology or nucleic acid tests for hepatitis B and C, human immunodeficiency virus (HIV), Cytomegalovirus (CMV), Epstein–Barr virus (EBV), and antinuclear antibody were negative. Nerve conduction studies (NCSs) confirmed the diagnosis of acute inflammatory demyelinating polyradiculoneuropathy (AIDP) which is the most common variant of GBS. Cerebrospinal fluid (CSF) analysis was not done. The whole-blood tacrolimus trough level was optimal.
She was treated with four sessions of PLEX (1.5 times the plasma volume, with human albumin as replacement fluid) on alternate days judiciously as she was pregnant with improvement in her neurological status, but again after 10 days, she had a recurrence of symptoms with worsening weakness of limbs. She was diagnosed to have “GBS-treatment-related fluctuations (GBS-TRF)” rather than a true relapse. She was retreated with another two sessions of PLEX on alternate days, with significant neurological recovery. Physiotherapy and supportive care were continued. Immunosuppression was not changed. She was discharged after 3 weeks with completely recovered motor function, but her sensory symptoms persisted for another 3 weeks after discharge. At 3 months of follow-up, she was having normal renal allograft function without any neurological symptoms and finished 34 weeks of amenorrhea without any adverse pregnancy outcome.
| Discussion|| |
GBS occurs relatively frequently in patients after bone marrow transplantation but has been a rare complication in SOT. In general population, almost two-third cases of GBS are preceded by gastrointestinal or respiratory infection; important microbial triggers include Campylobacter jejuni, CMV, EBV, varicella, influenza virus, HIV, and Mycoplasma. Other noninfectious precipitating factors include autoimmune disorders (systemic lupus erythematosus, chronic active hepatitis, hypothyroidism, sarcoidosis, Wegener's granulomatosis, and ulcerative colitis), vaccinations (influenza A, rabies, polio, TT, and meningococcal and pneumococcal vaccines), drugs, pregnancy, surgery, and malignancy. Almost all cases of GBS in SOT have been associated with CMV before or at the time of onset, and the majority of cases have occurred within 6 months to 1 year of SOT. Recently, Ostman and Chacko have reviewed 17 cases of GBS in RT patients and identified CMV as the most common trigger for GBS in the post-RT setting. The majority of cases were in males (81%) and in deceased donor RTs (87%). The time between RT and onset of symptoms ranged from 2 days to 10 years. GBS was associated with antecedent viral (CMV-12 and EBV-1) or diarrheal (2) illness while two cases were attributed to calcineurin inhibitor (CNI) use. All patients recovered fully or partially after treatment. We could not identify antecedent infection in our case. Few case reports have noted pathogenic roles of rituximab, ATG, and CNIs in triggering GBS both in nontransplant and post-SOT patients.,, CNIs probably could not have played a role in GBS occurrence in our case as the dose of tacrolimus used was minimal, taking it for last several years, and was continued post-GBS without triggering a relapse; also, rituximab and ATG due to their remote exposure might not have played a role. Pregnancy itself may trigger GBS, especially during the third trimester, and postpartum period may be due to imbalance in activity of helper T (Th1, Th2, and Treg) cells. During pregnancy, Th2 (anti-inflammatory) cytokines predominate over Th1 (pro-inflammatory) cytokines, and Treg (induce tolerance) activity increases during the first and second trimesters; these changes are regulated by hormones. Although there is little evidence to support a causal association with most vaccines including TT vaccination, their effect on the immune system may be associated with subsequent GBS.
Although GBS is considered as a monophasic disease, recurrences are reported in up to 2%–5% of patients. These could be due to the actual recurrence of the disease or due to the wearing-off effect of immunomodulatory therapy. However, it is important to distinguish recurrent GBS from GBS-TRF. Recurrent GBS is defined as two or more episodes with either a minimum interval of >4 months between the episodes if the patient did not recover completely or >2 months when there was a complete or near-complete recovery. GBS-TRF is defined as worsening of disability within the first 2 months after the disease onset. Hence, our patient was diagnosed to have GBS-TRF and retreated with additional sessions of PLEX.
CSF study may reveal albuminocytologic dissociation (isolated elevation in CSF protein level with normal white blood cell count) in the majority of patients with GBS, especially after the 1st week of onset of symptoms. Electrodiagnostic studies (NCS and electromyography) are especially useful for confirming the diagnosis and prognostication and to classify the variants of GBS. AIDP is characterized by features of demyelination such as decreased motor nerve conduction velocity, prolonged distal motor latency, increased F-wave latency, absent H-reflexes, conduction blocks, and temporal dispersion. Axonal forms of GBS are supported by decreased distal motor or sensory amplitudes. Although PLEX and IVIG are equally effective therapies in general population, optimal therapy for GBS in SOT is still unknown. Antimicrobial/antiviral agents and reduction of immunosuppression are required in postinfectious cases.
Our patient has several peculiarities to mention: GBS occurred following transplantation that to ABOi RT; GBS was diagnosed during second trimester of pregnancy; there was no evidence of antecedent infection; GBS resulted more than 5 years after RT; she also experienced GBS-TRF; it was successfully managed with PLEX though she is pregnant; she recovered completely and is maintaining a stable graft function. Pregnancy and vaccination might have played a role in precipitating GBS in our patient. High index of suspicion and early and aggressive therapy are important for rapid recovery and to prevent adverse sequelae as the clinical course of GBS can be more severe in a SOT recipient.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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