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 Table of Contents  
Year : 2021  |  Volume : 2  |  Issue : 2  |  Page : 185-186

Microscopic polyangiitis presenting as acute flaccid paralysis mimicking Guillain–Barre syndrome

1 Department of Geriatric Medicine, Yenepoya Medical College, Mangalore, Karnataka, India
2 Department of Geriatric Medicine, All India Institute of Medical Sciences, New Delhi, India

Date of Submission10-Jan-2021
Date of Decision03-Feb-2021
Date of Acceptance06-Feb-2021
Date of Web Publication30-Aug-2021

Correspondence Address:
Dr. Abhijith Rajaram Rao
Department of Geriatric Medicine, Yenepoya Medical College, Mangalore, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/JME.JME_3_21

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How to cite this article:
Rao AR, Thakral M, Rao A, Chakrawarty A. Microscopic polyangiitis presenting as acute flaccid paralysis mimicking Guillain–Barre syndrome. J Med Evid 2021;2:185-6

How to cite this URL:
Rao AR, Thakral M, Rao A, Chakrawarty A. Microscopic polyangiitis presenting as acute flaccid paralysis mimicking Guillain–Barre syndrome. J Med Evid [serial online] 2021 [cited 2022 Aug 10];2:185-6. Available from: http://www.journaljme.org/text.asp?2021/2/2/185/324960

  Introduction Top

Antineutrophil cytoplasmic antibody-associated vasculitides (AAV) is an umbrella term which includes eosinophilic granulomatosis with polyangiitis (EGPA), microscopic polyangiitis (MPA) and granulomatosis with polyangiitis. The involvement of the peripheral nervous system ranges from 25% to 80%.[1] These commonly cause subacute neuropathy in the form of mononeuropathy, mononeuritis multiplex and radiculopathy.[2] Guillain–Barre syndrome (GBS) is a clinical syndrome characterised by ascending, flaccid weakness with hyporeflexia or areflexia, which progresses up to 4 weeks. Here, we present a case of MPA presenting with acute flaccid paralysis mimicking GBS in on older adult.

  Case Report Top

This 75-year-old female was a case of hypertension, diabetes and hypothyroidism. She presented with fever, shortness of breath and cough for 2 months' duration. Her initial symptoms were mild and improved in 10–14 days. After 1 week, her symptoms reappeared and worsened gradually. The dyspnoea was insidious in onset and had gradually progressed to Modified Medical Research Council Grade IV. The cough was associated with scanty amount of mucoid sputum.

Physical examination revealed pallor and bilateral basal fine crepitation; rest of the examination was unremarkable. Initial investigations revealed anaemia, with haemogram of 10.6 g/dL (12–15 g/dL), white blood cell counts of 14,170/μL (4000–11,000/μL), neutrophil count of 11,817/μL, urea of 15 mg/dL (10–40 mg/dL) and creatinine of 0.5 mg/dL (0.05–1.00 mg/dL). Peripheral smear revealed normocytic normochromic red cells. She was started on intravenous antibiotics. The differential diagnoses considered were pulmonary tuberculosis, chronic pulmonary aspergillosis, initial viral bronchitis complicated by a secondary bacterial infection, atypical pneumonia, carcinoma lung, sarcoidosis and EGPA. Three sputum samples for acid-fast bacilli were negative.

On day 7 of admission, she developed weakness in her bilateral lower limbs, which progressed to involve upper limbs in 2 days. On examination, she was found to have symmetrical bilateral weakness in the lower and upper limbs with absent deep tendon reflexes. Her sensory examination was normal. Nerve conduction study showed pure motor neuropathy affecting all four limbs. A diagnosis of postinfectious GBS was considered, and intravenous immunoglobulin (IVIG) was started. She received IVIG 2 g/kg over 5 days. There was no improvement in the weakness 1 week after IVIG.

Meanwhile, her shortness of breath had worsened, and she developed orthopnoea. Her echocardiography was normal, with no regional wall motion abnormalities. Contrast-enhanced computed tomography (CT) revealed bilateral asymmetrical (right more than left) areas of consolidation with peri-bronchovascular, sub-pleural and peri-fissural distribution, suggestive of organising pneumonia. Five days after CT, she developed acute kidney injury, and her urea was 45 mg/dL and creatinine was 1.2 mg/dL. A diagnosis of contrast-induced nephropathy was considered, but her serum creatinine levels were on a rising tread even after 10 days of the procedure (urea 156 mg/dL and creatinine 2.7 mg/dL). Considering her chest CT findings, worsening renal function and acute flaccid paralysis not responding to IVIG, a sural nerve biopsy was performed. Her sural nerve biopsy showed marked demyelination and axonolysis with no fibrinoid necrosis. Her antinuclear antibody titre (by indirect immunofluorescence method) was 1:100, rheumatoid factor was 256 IU/mL (0–20 IU/mL), serum angiotensin-converting enzyme was 44.20 U/L (7.0–56.0 U/L), anti-myeloperoxidase (MPO) was 55.0 U/mL (0–9.0 U/mL) and anti-proteinase 3 was 1.2 U/mL (0–3.5 U. mL).

A diagnosis of MPA was made, and she was started on intravenous rituximab 375 mg/m2 on weeks 0, 1, 2, and 3 with oral prednisolone 0.5 mg/kg, which was tapered over 4 weeks. The patient's renal function improved (urea 41 mg/dL and creatinine 1.4 mg/dL), and her shortness of breath and weakness also improved. With physical rehabilitation, she was mobilised with a walker. A final diagnosis of MPA with acute flaccid paralysis was made.

  Discussion Top

MPA was described by Wohlwill in 1923 and classified by the Chapel Hill Consensus Conference as a necrotising small-vessel vasculitis.[3] It is known that most of the patients have renal involvement, 30% present with alveolar haemorrhage, 50% of individuals have cutaneous lesions, musculoskeletal involvement is present in two-thirds, 30%–50% have gastrointestinal involvement and a third present with peripheral neuropathy.[4]

Typically, the nerve lesion in vasculitis is axonal. Superficial peroneal, deep peroneal, cubital and median nerves are affected in decreasing frequency. In two-thirds of the patients, mononeuritis multiplex is the presenting feature, followed by subacute distal symmetrical polyneuropathy. MPA is not known to present with acute flaccid paralysis, though there are reports of EGPA mimicking GBS.[1],[5] For MPA diagnosis, demonstration of nerve ischemia, even in the absence of fibrinoid necrosis of epineural artery, is sufficient. Histological confirmation remains the gold standard for the diagnosis of MPA. CT reveals ground-glass attenuation, chronic interstitial inflammation of alveolar septa and capillaritis (94%), seen in our patient.

MPA affects older individuals compared to Wegener's granulomatosis (61 vs. 50 years),[6] with a higher mortality rate of 27.5%. Relapses are frequent with MPA though they respond well to treatment. For patients presenting with glomerulonephritis, remission induction is done by glucocorticoids and cytotoxic agents, such as cyclophosphamide. Moreover, remission is achieved in 90% of the patients.[7] Other options include plasma exchange, IVIGs, methotrexate, azathioprine and rituximab.

Our patient presented to us with chronic cough, with normal renal function, which was followed by the onset of acute flaccid paralysis, which led to the consideration of GBS. The lack of response to IVIG, the onset of acute kidney injury and lung parenchymal changes in CT supported by the anti-MPO and the nerve biopsy finding pointed toward the diagnosis of AAV. After 1 year, the patient can carry out her activities of daily living and walk with the help of a walking stick.

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.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Camara-Lemarroy CR, Infante-Valenzuela A, Villareal-Montemayor HJ, Soto-Rincon CA, Davila-Olalde JA, Villareal-Velazquez HJ. Eosinophilic granulomatosis with polyangiitis presenting as acute polyneuropathy mimicking Guillain-Barre syndrome. Case Rep Neurol Med 2015;2015:981439.  Back to cited text no. 1
Graf J, Imboden J. Vasculitis and peripheral neuropathy. Curr Opin Rheumatol 2019;31:40-5.  Back to cited text no. 2
Jennette JC, Falk RJ, Andrassy K, Bacon PA, Churg J, Gross WL, et al. Nomenclature of systemic vasculitides. Proposal of an International Consensus Conference. Arthritis Rheum 1994;37:187-92.  Back to cited text no. 3
Villiger PM, Guillevin L. Microscopic polyangiitis: Clinical presentation. Autoimmun Rev 2010;9:812-9.  Back to cited text no. 4
Praveen Y. Eosinophilic granulomatosis with polyangitis (Churg Strauss Syndrome) presenting as Guillain Barre syndrome (GBS)- A case report. J Med Sci Clin Res 2017;5:30820-3.  Back to cited text no. 5
Guillevin L, Pagnoux C, Seror R, Mahr A, Mouthon L, Toumelin PL, et al. The five-factor score revisited: Assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) cohort. Medicine (Baltimore) 2011;90:19-27.  Back to cited text no. 6
Greco A, De Virgilio A, Rizzo MI, Gallo A, Magliulo G, Fusconi M, et al. Microscopic polyangiitis: Advances in diagnostic and therapeutic approaches. Autoimmun Rev 2015;14:837-44.  Back to cited text no. 7


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