Journal of Postgraduate Medicine, Education and Research
Volume 57 | Issue 1 | Year 2023

A Case of Bilateral Facial Nerve Palsy with Bilateral Cavitary Lung Nodules in a Young Adult

Vikas Marwah1, Robin Choudhary2, Akhil K Ravi3, Deepu Peter4

1,2Department of Pulmonology, Respiratory Critical Care & Sleep Medicine, Army Institute of Cardio-Thoracic Sciences, Pune, Maharashtra, India

3Department of Respiratory Medicine, Army Hospital Research and Referral, New Delhi, India

4Department of Respiratory Medicine, Command Hospital, Udhampur, Jammu and Kashmir, India

Corresponding Author: Robin Choudhary, Department of Pulmonology, Respiratory Critical Care & Sleep Medicine, Army Institute of Cardio-Thoracic Sciences, Pune, Maharashtra, India, Phone: +91 9673300582, e-mail:

Received on: 27 January 2022; Accepted on: 07 March 2022; Published on: 10 April 2023


Granulomatosis with polyangiitis (GPA) mostly involves the upper and lower respiratory tract with renal involvement. It is a rare disease with varied presentation. However, bilateral facial nerve involvement in this disease is an extremely rare entity. We report a case of a young lady who presented with bilateral facial nerve palsy and was diagnosed as a case of GPA with multisystem involvement.

How to cite this article: Marwah V, Choudhary R, Ravi AK, et al. A Case of Bilateral Facial Nerve Palsy with Bilateral Cavitary Lung Nodules in a Young Adult. J Postgrad Med Edu Res 2023;57(1):35-37.

Source of support: Nil

Conflict of interest: None

Patient consent statement: The author(s) have obtained written informed consent from the patient for publication of the case report details and related images.

Keywords: Bilateral facial nerve palsy, Glomerulonephritis, Granulomatosis with polyangiitis, Vasculitides.


Granulomatosis with polyangiitis is an uncommon condition characterized by necrotizing granulomatosis of the upper and lower respiratory tract and glomerulonephritis. The disease commonly affects the kidneys and upper and lower respiratory tracts, and can have a variety of manifestations in the other organ system as well. GPA often presents with cough, hemoptysis, wheezing, sensorineural hearing loss, glomerulonephritis, serous otitis media, and neurological manifestation in the form of cranial nerve palsy and mononeuritis multiplex. Bilateral facial nerves are involved in extremely rare instances during the course of the disease, and initial presentation with this complication is rarely reported. GPA presenting with a neurological symptom such as bilateral facial nerve palsy has never been reported till now.


A 25-year-old lady was admitted to our hospital with fever, productive cough, bilateral red eyes, and diminution of hearing in both ears for 2 weeks. She also gave history of recurrent episodes of otorrhea since childhood. Two weeks prior to admission, she had reported to another center with productive cough with chocolate-colored sputum and streaky hemoptysis. She was diagnosed to have pulmonary tuberculosis on a clinical and radiological basis and was started on antituberculous treatment. However, she did not have any improvement and even developed arthritis in both knee and ankle joints. There was no history of tobacco and alcohol consumption.

At the time of hospital admission, the patient was conscious, there was no fever, pulse (right arm radial) was 96 per minute, respiratory rate was 20 per minute, and blood pressure was 148/94 mm Hg. She was adynamic, pale, upset, and was unable to speak clearly. Bilateral episcleritis (right eye > left eye) was present on ocular examination, and synovitis was present in the right knee. There was no rash, clubbing, pedal edema, lymphadenopathy, cyanosis, and icterus. Examination of the chest revealed bibasal crackles on auscultation. Detailed neurological examination suggested bilateral seventh nerve palsy (Fig. 1), and there was no evidence of lower cranial nerve involvement. Her neck was supple, with no meningeal signs. Her cardiac and vascular system examination was normal. Examination of her abdomen revealed no tenderness, distension, or visceromegaly. Rest of the systemic examination was unremarkable.

Figs 1A and B: (A) Bilateral facial nerve palsy with absent forehead wrinkles and inability to close eyes completely; (B) Saddle nose deformity

On evaluation, she had normochromic normocytic anemia with hemoglobin of 9.0 gm/dL. Total counts were in normal range but had thrombocytosis (5.21 lacs/cumm) and a high erythrocyte sedimentation rate of 122 mm fall in 1st hour. Liver and renal functions were within normal range, and plasma glucose was 113 mg/dL. Rest of the biochemical markers were in normal range. Chest radiograph (Fig. 2A) showed bilateral cavitary nodular lesions in middle and lower zones. Contrast-enhanced computed tomogram (CECT) of chest (Fig. 2B) showed multiple cavitary lesions bilaterally in the upper and middle lobes.

Figs 2A and B: (A) Chest radiograph showing bilateral cavitary nodular lesions in middle and lower zones; (B) CECT chest showing multiple cavitary lesions bilaterally in upper and middle lobes

High-resolution computed tomogram of the temporal region showed bilateral cholesteatoma with coalescent mastoiditis and chronic otitis media. Sputum analysis was, however, negative for acid-fast bacilli and other microorganisms. Immunological workup revealed antineutrophil antibodies negative by indirect immunofluorescence but cytoplasmic-antineutrophil cytoplasmic antibodies (c-ANCA) was positive (76.94 U/L), and perinuclear-anti- neutrophil cytoplasmic antibodies was negative (1.62 U/L). Urine analysis showed proteinuria, hematuria, and few epithelial cells with no evidence of casts. Ultrasonography abdomen was reported to be within normal limits. Fiber optic bronchoscopy was performed which showed yellowish waxy nodules in lower trachea and left main bronchus, and bronchial biopsy was taken which revealed clusters of epithelial cells (granulomas) consistent with Wegener’s granulomatosis (Figs 3A and B).

Figs 3A and B: (A) Photomicrograph showing granulomas (yellow arrows) in bronchial biopsy (H and E, ×100); (B) Photomicrograph of bronchial biopsy showing multiple slipper-shaped epithelial cells (granuloma) consistent with GPA (H and E, ×400)

As the patient had organ-threatening disease, she was started on oral prednisolone at 1 mg/kg and intravenous cyclophosphamide at 15 mg/kg every 2 weeks. She, however, had a torrid course with worsening glomerulonephritis and developed acute kidney injury with metabolic acidosis. Pulse corticosteroids were initiated, but the patient had a bout of massive hemoptysis and later succumbed to the disease.


Initially described by Klinger in 1933, and later by Rossle in 1933, Wegener in 1936 and 1939, and Ringertz in 1947, GPA is an ANCA positive vasculitis afflicting the small- and medium-sized blood vessels.1 With a mean age of occurrence of 40–55 years, GPA is a disease of older adults. However, cases have been diagnosed in patients of all ages, and severe disease is seen in females and young individuals. The most commonly affected are the kidneys and the upper and lower respiratory tract, with the lungs being involved in > 90% of patients.2,3 Pulmonary involvement usually manifests radiologically in the form of bilateral, multiple, nodular infiltrations, and these nodules may show evidence of cavitation. The disease has no gender predilection; however, women generally present with the limited form, wherein the kidneys are spared, and disease involvement is seen mostly in the respiratory system, while in men, renal involvement is predominantly seen throughout the course of the disease, > 80% of patients show pulmonary involvement and in 70–90% of patients sino-nasal and renal involvement are also seen.4 Symptoms and signs of pleural effusion can be seen in 25–30% patients of GPA.5

Facial diplegia is idiopathic in < 20% individuals, unlike unilateral palsy, wherein no underlying cause is evident in > 50% of cases. Lyme disease caused by Borrelia burgdorferi is the most common cause of bilateral facial palsy seen in 30–35% cases. Guillain–Barré syndrome, a postinfectious inflammatory condition, can lead to bilateral facial palsy in around 50% of fatalities. Other causes are trauma (4%), sarcoidosis (0.9%), and AIDS (0.9%). As was seen in our patient, bilateral palsy of the facial nerve is an extremely rare manifestation seen in around 2% of cases and might be associated with hearing loss.6 Our literature review could identify only one case report wherein a patient presents with facial palsy on both sides, like the patient under discussion.7 A dehiscent fallopian canal or vasculitis of its microvasculature can lead to the compression of the facial nerve in the middle ear and hence facial palsy.4-6 However, decompression of the facial nerve has been proven not beneficial and can also lead to aggravation of the problem.

To distinguish GPA from other vasculitides, the American College of Rheumatology (ACR) has established the following criteria: (i) high -power field examination of urine showing sediment containing red blood cell casts or more than five red blood cells, (ii) chest radiograph abnormalities consistent with GPA, (iii) oral ulcers or nasal discharge, and (iv) tissue biopsy with evidence of granulomatous inflammation. When two or more of these criteria are present, the diagnosis of GPA can be confirmed with a sensitivity of 88% and a specificity of 92%.8,9

Despite being an ANCA-associated vasculitis, ACR or Chapel Hill Consensus Conference definition does not include autoantibodies to proteinase 3/c-ANCA as a diagnostic criteria for GPA.10 Occasionally, ANCA may be falsely present in patients with malignancy, infection, inflammatory bowel disease, and rheumatic disease.5

Granulomatosis with polyangiitis is treated with the aim of achieving remission. Therapy depends on the presence of end organ dysfunction. If the patient has evidence of life-threatening organ dysfunction in the form of peripheral or cranial neuropathy, active glomerulonephritis, scleritis, gastrointestinal bleeding, pericarditis and myocarditis, induction with steroids and cyclophosphamide or rituximab followed by maintenance therapy is warranted. Our patient presented with seventh nerve palsy, scleritis, and glomerulonephritis for which she was started on steroids and cyclophosphamide. Combination of cyclophosphamide and glucocorticoids is known to induce remission in 85–90% of cases and complete remission can be seen in 75%.11,12 Rituximab-based regimen was also compared with cyclophosphamide in the rituximab in ANCA-associated vasculitis (RAVE) trial, which revealed similar induction remission rate at 6 months with both the drugs.13 Rituximab was found to be superior to cyclophosphamide in patients with relapsing disease.

A study conducted in 193 patients by Samson et al. has shown that patient of ANCA-associated vasculitis presenting with mononeuritis multiplex has a poor prognosis.14 These patients are also likely to fail initial therapy with glucocorticoids and might require stronger immunosuppressive therapy.


Unilateral facial palsy is mostly idiopathic; however, bilateral facial palsy, when identified should warrant suspicion of systemic disease and requires aggressive evaluation to identify the etiology. The emergency physician should be aware of the various differential diagnosis so as to prevent further complications and even mortality. A patient with GPA, when presenting with facial palsy, has a poor prognosis, and early identification of the disease is necessary to institute the necessary immunosuppressive therapy, which can be life-saving.


Vikas Marwah

Robin Choudhary

Akhil K Ravi

Deepu Peter


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