A 47-year-old female, previously known to have type 2 diabetes mellitus, was admitted to a tertiary-care hospital in Al Ain city, with complains of severe headache, dizziness, and right-sided facial weakness. She has been complaining of headache and dizziness for a duration of 2 months, which has been ongoing intermittently, but has increased in the past 5 days prior to admission, associated with right ear pain. She described headache as of pulsatile in quality, more on the right side, with a severity of 7/10, increasing with bending forward, relieved with low light condition and analgesics. Her dizziness was in the form of vertigo, increased with head movements, more on the right side. She had right lower motor neuron (LMN) type facial palsy, which developed around two months prior to current presentation and has been improving with a course of steroid treatment. In addition, two weeks prior to her development of right lower motor neuron facial palsy, she had a history of left lower motor neuron facial palsy, which was treated with a course of systemic steroids. She has a long-term history of chronic maxillary sinusitis and bilateral otitis media, complicated with conductive hearing loss, which was treated with bilateral ear grommet tube insertion, three months prior and is on regular follow-up with the ENT team in a private hospital. She had no other symptoms or signs of vasculitis.
Along with recurrent history of otitis media and sinusitis, she also had history of fever and cough, which was treated as for pneumonia in another hospital facility, following which, on admission to our hospital, her chest X-ray revealed a patchy opacity in the right mid zone, which was further investigated via computed tomography (CT) thorax (Figures 1-3) and biopsy. CT thorax revealed a cavitary lesion in the right upper lobe measuring about 2.5 x 2.5 x 1.9 cm, which has thick irregular outline with nearby interstitial changes. Magnetic resonance imaging (MRI) of the brain revealed significant sinusitis of both the maxillary sinuses and mastoid air cells of both sides, with normal findings of the brain (Figure 4).
Further investigation including autoimmune screening showed that she had raised erythrocyte sedimentation rate (ESR) level (62 mm/hr), raised C-reactive protein (CRP) levels (54 mg/L), positive C-antineutrophil cytoplasmic antibody (ANCA) level (106RU/mL), mild lymphopenia (1.15 x 10 9/L), mild iron deficiency anemia (hemoglobin 95 g/L), elevated lactate dehydrogenase (LDH) level (322 IU/L), and raised HbA1c 6.8%. Other parameters for urea, creatinine, electrolytes, and urine tests were normal, and there was no evidence suggestive of any form of renal involvement. She tested negative for blood and urine cultures.
Workup was negative for p ANCA, antinuclear antibody (ANA), rheumatoid factor (RF), cyclic citrullinated peptide (CCP), and quantiferon-TB, and she also had normal angiotensin-converting enzyme (ACE) levels (25 microliters). Her HIV and hepatitis screen was also negative. The patient was advised to undergo lumbar puncture for further investigation for headache, to which she refused. Facial nerve palsy was diagnosed clinically, and no additional tests were performed to confirm the diagnosis at this instance.
The histopathological report from the CT-guided biopsy of the lung lesion revealed findings of extensive coagulative necrosis and moderate interstitial inflammation comprising lymphocytes, neutrophils, histiocytes, plasma cells, and eosinophils. There were also scattered multinucleated giant cells of langhans type and vague granulomatous and focal neutrophilic infiltration consistent with leucocytic vasculitis. It was negative for acid-fast bacillus and fungal strains. The histological features were compatible with the diagnosis of granulomatosis with polyangiitis.
Given the histopathological findings and positive C-ANCA levels, a diagnosis of granulomatosis with polyangiitis was made and it was decided to start treatment with immunosuppressants. The involvement of the cranial nerves causing bilateral facial palsy is presumed to be due to the autoimmune inflammatory process from granulomatosis with polyangiitis, as it is unusual to have bilateral Bell's palsy apart from diseases such as sarcoidosis, Lyme disease, Guillain-Barre syndrome, meningitis (neoplastic or infectious), and rarely congenital.
The patient was started on treatment with analgesics for headaches and beta-histine for peripheral vertigo as per the ENT team recommendations initially. After multidisciplinary team discussion, the patient was started on intravenous (IV) pulse steroid with methylprednisolone 1 gram/day for 3 days, followed by oral prednisolone 60 mg per day. Following completion of pulse steroid treatment, she was started on IV rituximab 1 gram as per rheumatology recommendations and also carefully taking into account the patient's denial to receive IV cyclophosphamide due to fear of the side effects and renal impairment in the long run. This approach was supported by the fact that she had normal renal function and as per the literature, and both immunosuppressants showed equal efficacy in treatment for GPA. The patient had significant improvement after starting the treatment, and she was followed up regularly in the rheumatology clinics.
Prior to starting of the immunosuppressants, the calculated Birmingham Vasculitis Activity Score/Wegener's granulomatosis (BVAS/WG) was 27. She received her second dose of rituximab 1 gram after 2 weeks, following the first dose, and her symptoms showed significant improvement. After 3 weeks, her BVAS score came down to 15 and she had almost 90% improvement in her headache, dizziness, facial palsy, and numbness. Her thiopurine methyltransferase (TPMT) enzyme level activity was slightly low towards the borderline, but not deficient and had a normal genotype. Considering this, she was started on a slightly lower dose of azathioprine 50 mg daily, later increased to 100 mg daily and was kept on close monitoring. Steroid treatment was continued on a tapering dose, and she was also kept on bone protection agents such as alendronate, vitamin D, and calcium supplements. She was kept on prophylaxis treatment for pneumocystis jeroveci infection with co-trimoxazole and also supplemented with ferrous fumarate for anemia.
Following two months of treatment with regular follow-up, her inflammatory marker had improved to normal with C-ANCA level 7 RU/mL, CRP level 6.4 mg/L, and ESR 10 mm/hr. Her complete blood count showed normal hemoglobin, leukocytes, lymphocytes, neutrophils, and platelets. Steroid treatment was tapered slowly. She was kept on azathioprine 100 mg per day, calcium, vitamin D, and co-trimoxazole along with her diabetic and hypertension medications. She continued to be followed up regularly in the ENT, diabetes, and rheumatology clinic to be monitored for any further complications.