Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0042384 (vasculitis)
 20,525 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We observed the change of hearing for 1 year in a patient with Wegener's granulomatosis. We also studied anti-human neutrophil cytoplasm antibody (ANCA), which is believed to be specific for this disease and to be associated with disease activity. From these progresses, changes in hearing were considered to be generally related with the general condition and with the ANCA titer level. Because hearing loss in our case was improved by steroids and immunosuppressants, and because ANCA is the autoantibody observed in angiitis syndrome, hearing loss in this case of Wegener's granulomatosis was suspected of being caused by angiitis of the middle and inner ear induced by autoimmune reaction.
Auris Nasus Larynx 1992
PMID:The hearing loss in Wegener's granulomatosis: relationship between hearing loss and serum ANCA. 151 41

Intact Eustachian tube and tympanic cavity functions are essential for normal middle ear physiology. Type II collagen is an essential component of ear tissue. Autoimmune response to this type II collagen produces sensorineural hearing loss, vestibular dysfunction, endolymphatic hydrops, otospongiosis-like lesions, Eustachian tube inflammation and Eustachian tube chondritis. In addition, these autoimmune responses occasionally produce otitis media with effusion (not infectious). Tympanosclerosis, which is a frequent sequelae of otitis media with effusions, was also induced by type II collagen immunization together with surgical incision of the tympanic membrane. The exact mechanisms of this type II collagen autoimmune mediated middle ear disease are not clear. However, the animals thus induced have higher antibody titers and cell mediated immune responses to type II collagen. The tympanic membrane showing tympanosclerosis also had C3 and Ig deposits. All the animals with otitis media with effusion induced by type II collagen immunization also had Eustachian tube disease. This could be due to a malfunction of the Eustachian tube. Thus, this model might provide a rational approach for the study of otitis media with effusion associated with vasculitis. Further studies are needed to elucidate the immunologic mechanism involved in the pathogenesis of otitis media with effusion.
Auris Nasus Larynx 1985
PMID:Type II collagen mediated autoimmune middle ear disease: eustachian tube disease, otitis media with effusion and tympanosclerosis. 383 48

A 36-year-old female with hyperthyroidism that had been treated with propilthiouracil (PTU) complained of tinnitus and hearing loss in both ears. She was treated with steroid administration by an otolaryngologist; however, hearing continued to fluctuate when the steroids were tapered. Laboratory evaluation revealed a decreased complement level and elevated levels of immunoglobulin M (IgM) and myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA). With the withdrawal of PTU and high-dose methylprednisolone, she had excellent return of right-sided hearing. In recent years, there have been many reports about MPO-ANCA-associated small vessel vasculitis. Although any organ may be affected by this disease, there are no reports about MPO-ANCA-associated progressive hearing loss without any other organ involvement. The present case suggests the possibility that inner ear blood flow impairment due to ANCA-associated small vessel vasculitis induces the so-called autoimmune sensorineural hearing loss.
Auris Nasus Larynx 2001 May
PMID:Myeloperoxidase-antineutrophil cytoplasmic antibody-associated sensorineural hearing loss. 1168 26

Churg-Strauss syndrome (CSS) is a rare syndrome of unknown cause, which is characterized by bronchial asthma and hypereosinophilia followed by systemic symptoms of angiitis. In 1951, Churg and Strauss first distinguished this disease from periarteritis nodosa. Our patient in particular was also complicated with otorhinological symptoms, which have less frequently been reported in CSS cases. We have detailed her clinical symptoms.
Auris Nasus Larynx 2001 May
PMID:Churg-Strauss syndrome with pachymeningitis refractory to steroid therapy atone--a case report. 1168 30

Eosinophilic angiocentric fibrosis (EAF) is a rare benign condition of unknown aetiology and is most commonly found in the nasal septum and sinus mucosa. We report a case of EAF and present a review of the available literature. A 51-year-old man with progressive nasal obstruction was referred to our hospital. CT and MRI scans revealed a mass on the nasal septum; this was surgically excised. Histological analysis of the resected tumour showed an inflammatory infiltrate with a predominance of eosinophils present in fibrous matrix and absence of eosinophilic vasculitis and onion skinning. Histologically, it resembled granuloma faciale. However, our case was considered to be an EAF although eosinophilic vasculitis and onion skinning were not observed. This was because a cutaneous lesion was absent and the lesion was limited to the nasal septal mucosa.
Auris Nasus Larynx 2006 Sep
PMID:Atypical eosinophilic angiocentric fibrosis on nasal septum. 1652 99

Wegener's granulomatosis (WG) is a multi-system disease characterized by granuloma formation and widespread necrotizing vasculitis. It classically presents with involvement of the upper and lower respiratory tracts and renal system. Facial nerve palsy and otologic manifestations have been reported during the course of the disease but it is extremely rare as the presenting features. In Literature only one case of bilateral facial palsy as presenting sign of WG is reported. The testing of anticytoplasmic antibodies versus neutrophil polymorphonucleate granules (c-ANCA) are highly specific for the diagnosis of WG, being positive in 97% of the cases. The early diagnosis and the timely medical treatment lead to high rates of remission of an otherwise lethal disease. A case of WG presenting with bilateral facial paralysis and bilateral serous otitis media is reported.
Auris Nasus Larynx 2007 Sep
PMID:Early diagnosis of Wegener's granulomatosis presenting with bilateral facial paralysis and bilateral serous otitis media. 1735 Jan 98

Patients with Wegener's granulomatosis (WG) often suffer from hearing loss, but its precise mechanisms have not been well understood. We experienced 3 WG cases whose initial symptoms were bilateral progressive mixed (both conductive and sensorineural) hearing loss, followed by systemic symptoms one year later. They were diagnosed as WG based on positive serology of anti-neutrophil cytoplasmic antibodies (ANCAs) and pathologic findings of affected lesions in addition to systemic symptoms. Although they were different in the type of ANCAs and systemic lesions, all showed considerably reversible cochlear disorders with normal vestibular functions. Moreover, their initial otologic manifestations shared same characteristic features, (1) thick ear drums with pulsatile serous intratympanic effusion, (2) poor speech discrimination ability, and (3) steroid-dependent changes of hearing levels (HLs). They exhibited no significant vestibular abnormalities in chair vestibule-ocular reflex (VOR) testing and cold air caloric tests even when they had severe hearing loss. On the basis of these results, we hypothesized that vasculitis of stria vascularis which generates endocochlear potential might cause these reversible cochlear-specific dysfunctions.
Auris Nasus Larynx 2012 Apr
PMID:Reversible cochlear disorders with normal vestibular functions in three cases with Wegener's granulomatosis. 2160 91

Subclavian steal syndrome (SSS) is usually caused by unilateral subclavian artery (SA) occlusion, and bilateral SSS is very rare. Takayasu's arteritis (TA) is a chronic granulomatous form of vasculitis that affects the SA, most commonly in women aged 15-40 years. We report a rare case of bilateral SSS due to TA in a 52-year-old woman, who exhibited severe vertigo. Although her blood pressure was within the normal range and did not differ between her arms, Doppler ultrasonography revealed low antegrade blood flow in the right SA and retrograde flow in the left SA. Computed tomography angiography demonstrated complete obstruction of the bilateral SA proximal to the vertebral artery origin. The more marked decrease in the blood flow of the vertebrobasilar artery experienced in bilateral SSS compared with unilateral SSS is considered to have caused the severe rotatory vertigo in the present patient. Since the vascular conditions of TA progressively deteriorate, delayed diagnosis and treatment could result in poor outcomes and unfavorable prognosis. We suggest that SSS with TA might require an early detection and treatment as well as careful follow-up for preventing vertigo and other neurological deficits in the vertebrobasilar arterial region.
Auris Nasus Larynx 2014 Jun
PMID:Bilateral subclavian steal syndrome with vertigo. 2420 29

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a form of necrotizing vasculitis with few or no immune deposits. It primarily affects small and medium blood vessels. AAV is classified into three categories, granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangitis (EGPA), and two major ANCAs, proteinase 3 (PR3)-ANCA or myeloperoxidase (MPO)-ANCA are involved in their pathogenesis. Intractable otitis media frequently occurs in patients with GPA, MPA or EGPA, although all patients show similar clinical features, regardless of the type of AAV. Furthermore, approximately 15% patients with otitis media caused by AAV do not show ANCA positivity, histopathological evidence, or any other AAV-related lesions at the initial visit; therefore, these patients do not fulfill the ordinary diagnostic criteria for systemic AAV. Thus, we first proposed that this condition could be categorized as "otitis media with AAV (OMAAV)". Subsequently, the Japanese Otological Society (JOS) conducted a nationwide survey between December 2013 and February 2014 and identified 297 patients with OMAAV. The survey revealed that OMAAV is a disease that initially occurs in the middle ear and subsequently spreads to other organs such as the lungs and kidneys, with eventual involvement of all body organs. Severe sequelae such as facial palsy, hypertrophic pachymeningitis, complete deafness, and subarachnoid hemorrhage resulting in death can also occur. In this review, we introduce the clinical features, diagnostic criteria, and treatment strategies recommended by JOS for early diagnosis and treatment of OMAAV.
Auris Nasus Larynx 2020 Aug 04
PMID:Clinical characteristics, the diagnostic criteria and management recommendation of otitis media with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (OMAAV) proposed by Japan otological society. 3276 13