Medical Case Reports

Introduction

Otologic symptoms, such as pain and hearing loss, may be experienced by some people with autoimmune disease. We describe a patient with ANCA-associated vasculitis who needed surgery to remove a suspicious mastoid lesion. Necrotizing vasculitis characterized by autoantibodies directed against neutrophil-specific proteins is known as Antineutrophil Cytoplasmic Antibody (ANCA) Associated Vasculitis (AAV). However, patients may maintain an AAV diagnosis despite negative serologic testing for ANCA. Patients with ANCA-negative AAV have had fewer reports of otologic manifestations than those with AAV. To reduce the condition's morbidity as much as possible, early diagnosis is essential. A mastoid lesion in the context of ANCA-negative small vessel vasculitis is the subject of this case report. In 2020, a 58-year-old woman with a history of bipolar disorder, hypertension, and hypothyroidism was admitted to the hospital with a number of medical issues, including pericarditis, pericardial effusion, headache, nodular lung disease, ground glass opacities of the lung, and recurrent sinusitis.

Otorhinolaryngology Abnormalities

She also had attempted suicide multiple times. The ESR, ferritin, and CRP were all found to be significantly elevated in her. The ANCA was negative, and the temporal artery biopsy did not reveal any particular pathologic changes. She began treatment with methotrexate and prednisone, and her lung opacities on imaging improved; the patient was in this manner determined to have ANCA-negative little vessel vasculitis. The patient stopped taking these medications herself, and in 2021, she overdosed on Seroquel and ended up in the hospital. She began to complain of blurry vision after being admitted. She was given rituximab and re-started taking steroids due to concerns about an exacerbation of vasculitis. She underwent additional workup with Computed Tomography (CT) imaging, which coincidentally revealed a left mastoid lesion. Except for a mild hearing impairment, the patient had no symptoms in the left ear. She had no set of experiences of otitis media, ear torment or ear waste. CT of the inward hear-able channel showed fiery changes in the mastoid air cells respectively with highlights reminiscent of coalescent mastoiditis with broad disintegration of the mastoid piece of the left transient bone. CT head imaging one year earlier shown no mastoid anomalies. The tegmen and cortex both showed signs of bone erosion. MRI revealed uniform enhancement and associated pachymeningeal enhancement in the adjacent temporal lobe, as well as homogenous T1 isointense signal within the bilateral mastoid air cells. There were no signs of an abscess or cholesteatoma. She did not have any other otorhinolaryngology abnormalities or disease symptoms. The patient was recommended for mastoidectomy and biopsy due to the aggressive appearance of the otologic lesions and their uncertain etiology. The left partial mastoidectomy revealed large bony erosion and a soft tissue lesion in the mastoid. The bony ear canal's posterior-superior aspect was severely eroded, but the skin remained intact. Spindle cells and inflammation were found in a frozen section biopsy, but there was no evidence of an acute infection. Minuscule assessment of the mass on conclusive pathology showed a fibrohistiocytic sore made out of macrophages, mature lymphocytes, eosinophils and neutrophils implanted in a fibrocollagenous foundation. There was no unmistakable plasma cell part to recommend IgG4 related infection or granuloma development to propose granulomatosis with polyangiitis. Similarly as with her past fleeting supply route biopsy, no proof of vasculitis was noted. Entities like Inflammatory Myofibroblastic Tumor (IMT), peripheral nerve sheath tumors, B cell lymphoproliferative disorders, and Langerhans cell histiocytosis were ruled out by immunostaining. Special staining was used to rule out a mycobacterial spindle cell tumor. The lesion's immunochemistry and histology were found to be most indicative of an inflammatory fibrohistiocytic lesion. To rule out fibromatosis and solitary fibrous tumors, additional stains were performed. The case was sent to a different institution to be looked at, and the consultants agreed that the diagnosis was correct. The necrotizing vasculitis known as Antineutrophil Cytoplasmic Antibody (ANCA) Associated Vasculitis (AAV) primarily affects the small blood vessels. Autoantibodies for neutrophilic proteins, myeloperoxidase (MPO-ANCA) and proteinase 3 (PR3-ANCA), are associated with AAV. Granulomatosis with Polyangiitis (GPA), eosinophilic Granulomatosis with Polyangiitis (eGPA), and Microscopic Polyangiitis (MPA) are major clinicopathologic variants of AAV. ANCA-negative AAV depicts cases in which ANCA serology is negative, yet that in any case satisfy the meaning of AAV. Up to 10% of patients might be seronegative.

Systemic Glucocorticoids

Atypical features and seronegative GPA were suspected in our patient. Up to 70% of the time, GPA is associated with otolaryngologic symptoms, particularly rhinologic and sinus manifestations. Otologic manifestations with ANCA-positive GPA have been reported, though they are uncommon. Otalgia, otorrhea, hearing loss, tinnitus, middle ear disease, and mastoiditis are some of the symptoms. However, only one other case of ANCA-negative GPA with otologic manifestations has been reported. The depicted patient's mastoid infection was an accidental finding during workup for immune system sickness. A fibrohistiocytic lesion made up of mature lymphocytes, macrophages, eosinophils, and neutrophils embedded in a fibrocollagenous background was discovered in her mastectomy case. In AAV, tests from impacted locales can yield vague intense and constant irritation in up to half of biopsies. The lack of distinct vasculitis in surgical biopsy may also be due to the mastoid's limited vasculature. Immunosuppressive medications and systemic glucocorticoids are used to treat small vessel vasculitis. When treated with rituximab, patients who have otolaryngologic manifestations of small vessel vasculitis are less likely to have active ENT disease. It has been demonstrated that surgical management of acute mastoiditis in the context of AAV should be carefully considered because it frequently has limited success in eliminating disease and the prognosis may worsen postoperatively. However, obtaining a tissue biopsy may help direct and support the right medical treatment. After surgery, our patient continued to receive rituximab treatment. Given the uncommonness of this element, made to order audit ought to be led with interdisciplinary clinical and careful groups to decide ideal administration of this condition. Patients infected with a seronegative AAV may present with autoimmune disease-like otologic manifestations.