UNUSUAL PRESENTATION OF ANCA
VASCULITIS WITH OTITIS MEDIA AND CVA
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FARAH ALNOOR EBRAHIM1 SYLVIA
MBUGUA1 FRED OTIENO1 HANIKA
PATEL2 SHEILA WAA2 |
1 Department of Medicine, Faculty of Health Science, The Aga Khan
University Hospital, Aga Khan University of Medical Collage of East
Africa, Nairobi, Kenya
2 Department of Radiology, Faculty of Health Science, The Aga Khan
University Hospital, Aga Khan University of Medical Collage of East
Africa, Nairobi, Kenya
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ABSTRACT
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A 42-year-old African female (Kenyan in origin), known to have
hypertension that was diagnosed during gestation and persisted;
presented with a history of dysphagia since 2016. Prior to her
admission, she had presented to the surgical clinic with ear fullness
and ear pain with throbbing headaches, and she was diagnosed with
bilateral oto-mastoditis. Her symptoms subsequently, progressed to a
multisystemic disease involving the lungs, brain, kidneys and peripheral
nerves.
ANCA vasculitis is a rare disease, the main target organs in
granulomatous polyangiitis include the ear, nose throat and upper
respiratory tract, glomerulonephritis of the kidney and the lungs.
Localized disease manifestation can include the nose (rhinorrhea, nasal
crusts, septal perforation) and ear (otitis media, hearing loss,
sensorineural deficits). The diagnosis of vasculitis from localized
disease manifestation can be difficult and hard to diagnose.
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CASE
PRESENTATION
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A 42-year-old African female
(Kenyan in origin), known to have hypertension that was diagnosed during
gestation and persisted; presented with a history of dysphagia, to
solids, liquids and semisolids since 2016. Prior to her admission she
had presented to the surgical clinic with ear fullness and ear pain with
throbbing headaches and was diagnosed to have bilateral oto-mastoditis
with a mastoid effusion for which she underwent mastoidectomy after
recurrent episodes of ear infection. Cultures obtained grew
corrynebacterium urealytiucm and staphylococcus aureus for which she
received antibiotics sensitive to gentamicin, cotrimoxazole,
ciprofloxacin, clindamycin, erythromycin, vancomycin and tetracycline,
and multiple tests or tuberculosis had been negative including cultures.
HIV test was negative. A few months later she presented with trigeminal
neuralgia, and received prednisone.
Two weeks prior to her recent admission in 2019, she developed
progressive bilateral lower limb numbness up to the calves. Two days
prior to admission, she experienced sharp bilateral pains radiating to
the buttocks, poorly responsive to analgesia. She also reported
hoarseness of the voice.
On presentation, the blood pressure was 136/97mmHg, pulse 91/min,
respiratory rate 18/min with temperature of 36.6 degrees centigrade.
Chest and abdominal examination were unremarkable. Neurological
examination revealed distal lower limb length dependent polyneuropathy
bilaterally (with altered sensation to pain, light touch and vibration
sense).
She was thereafter noted to have right sided hemiparesis, with brisk
right bicep and knee deep tendon reflexes. A repeat MRI done showed a
6mm acute right pontine infarct, and a remonstration of the
oto-mastoidistis and left mastoid effusion (Figure 1, Figure 2). She was
commenced on aspirin and clopidogrel and atorvastatin. A vasculitis
screen was carried out and C-ANCA was positive; this entertained the
diagnosis of Anti-neutrophilic associated vasculitis.
Her neuralgia was treated with analgesia, for the laryngeal infection
she was started on high dose cotrimoxazole, based on the need for later
prophylaxis against opportunistic infections once treatment for
anti-neutrophilic associated vasculitis was commenced, for which she
received rituximab.
In 2020 she presented with tracheitis (figure 3, figure 4) and sputum
cultures ad been positive for Klebsiella oxycota for which she was
treated with antimicrobial therapy and continued her maintenance therapy
of prednisone, mycophenolate sodium, cotrimazole prophylaxis,
alendronate and her antihypertensive medications. Unfortunately, she
developed massive upper gastrointestinal bleed secondary to multiple
vasculitis gastric ulcers evidenced by an upper endoscopy. She kept
having persistent upper gastrointestinal bleed and in view of the
vasculitis, she underwent plasma exchange. Over the next couple of days,
she experienced persistent upper gastrointestinal haemorrhage for which
she was transfused and a surgical opinion was sought. However, surgical
interventions could not be instituted in view of her hemodynamic
instability. Her condition persistently deteriorated and she suffered a
cardiopulmonary arrest for which she was resuscitated as per the ACLS
protocol. She was regrettably pronounced dead.
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IMAGING
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FIGURE 1 There is a 6mm FLAIR hyperintense focus with associated true
restricted diffusion in the right hemi-pons as shows below:
Axial FLAIR demonstrating the 6mm hyperintense focus in the right
hemi-pons. Also seen are hyperintense signals within the right mastoid
air cells in keeping with otomastoiditis.
FIGURE 2 Axial DWI and ADC maps demonstrating the restricted diffusion
in the right hemi-pons (dark on ADC and bright on DWI).
FIGURE 3 Sagittal contrast enhanced CT of the neck demonstrates soft
tissue thickening of the posterior wall of the upper trachea (inferior
to the posterior cricoid), slightly eccentric to the left, and with a
craniocaudal extent of approximately 12.2 mm as shown below:
FIGURE 4 Axial images at the same level demonstrates narrowing of the
adjacent airway by approximately 50%.No invasion into the surrounding
soft tissues or cartilaginous structures was seen. The rest of the neck
spaces are intact. No enlarged or abnormal neck nodes.
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