UNUSUAL PRESENTATION OF ANCA VASCULITIS WITH OTITIS MEDIA AND CVA

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

ABSTRACT

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.

CASE PRESENTATION

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.

IMAGING

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.