Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0409974 (lupus)
 22,386 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In view of the presence of autoantibodies against inner ear antigens, the pathogenesis of sudden deafness (SD) and progressive sensorineural hearing loss (PSNHL) is suggested to be of an autoimmune nature. However, microthrombosis of the inner ear may result from pathogenic anti-cardiolipin antibody (aCL) activity. We studied 30 patients (17 females and 13 males, age range 20-52 y), of whom 11 suffered from SD and 19 from PSNHL. All were clinically and serologically evaluated for association with autoimmune disorders (serological examination included: aCL, ANA, ENA, ANCA, proteinelectrophoresis, and complement levels). Twenty healthy matched subjects served as controls. None of the control group were aCL positive, whereas 8 out of 30 (27%) patients demonstrated low-moderate titers (P < 0.02), of whom 5 out of 8 suffered from SD. In addition, 2 aCL negative patients with PSNHL demonstrated hypergammaglubolinemia accompanied by hypocomplementemia, whereas none with SD had such abnormalities. Our data suggests that aCL is detected in patients with sudden sensorineural hearing loss and therefore may play an important role in the pathogenesis of this disability. If sustained by additional studies, these findings would warrant the consideration of anticoagulant therapy.
Lupus 1997
PMID:Autoimmune aberration in sudden sensorineural hearing loss: association with anti-cardiolipin antibodies. 925 13

Although the pathogenesis of sudden sensorineural hearing loss (SNHL) in patients with systemic lupus erythematosus (SLE) is not clear, several reports suggest an association with the antiphospholipid antibody (aPL). We describe 6 patients with SLE or a lupus-like syndrome, who had sudden SNHL and had elevated levels of anticardiolipin antibodies (aCL) or the lupus anticoagulant. In a literature search, of 5 additional reported cases, 2 were not tested for aPL; the remaining 3 had elevated aCL levels. Thus, acute SNHL in patients with SLE who have aPL may be a manifestation of the antiphospholipid syndrome. We recommend anticoagulation treatment of these patients.
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PMID:Sudden sensorineural hearing loss in patients with systemic lupus erythematosus or lupus-like syndromes and antiphospholipid antibodies. 1049 96

In order to clarify the possible mechanism of hearing loss in immune-mediated sensorineural hearing loss, basic research needed includes animal model studies. In the present investigation, we examined hearing thresholds and cochlear histologies of the MRL/lpr mouse which is now well-known as a model for pathology consistent with systemic lupus erythematosis (SLE). Present findings demonstrated that there were no statistically significant differences in auditory brainstem response (ABR) thresholds between 4- to 6-week-old "young" and 20- to 25-week-old "old" MRL mice. These differences were not sex-dependent. Under light microscopy, there were no abnormal morphological findings in the cochleas of either young or old MRL mice. With immunohistochemistry, mouse IgG was detected around the capillary walls in the stria vascularis in both young and old MRL mice. Serum IgG level of the MRL mice significantly decreased after predonisolone (PSL) administration. However, expression of mouse IgG in the stria vascularis was not observed in the MRL mice after PSL administration. From these results, we speculate that the hearing of the MRL mouse does not always deteriorate, and the deposition of mouse IgG on the capillary wall in the stria vascularis is not a sufficient factor to induce hearing loss. At this point, we conclude that the MRL mouse should not be considered a useful model for immune-mediated sensorineural hearing loss.
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PMID:Hearing in the MRL/lpr mouse as a possible model of immune-mediated sensorineural hearing loss. 963 65

Sensorineural hearing loss may occur in SLE, but aortic insufficiency has been very rarely reported. We are describing two patients with well-established SLE who developed bilateral hearing loss and aortic insufficiency, associated with serological evidence of active lupus. Neither patient had evidence of keratitis, and thus did not satisfy criteria for Cogan's syndrome. The aortic insufficiency in one patient stabilized after treatment with high doses of steroids while in the second patient, who refused medical treatment, it progressed requiring surgical valve replacement. Our observations suggest that the aortic valve and the inner ear may share some antigenic crossreactivity not shared by the cornea. In SLE patients, with sensorineural hearing loss, echocardiography should be performed looking for evidence of aortic insufficiency, which may be steroid responsive.
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PMID:Sensorineural hearing loss in conjunction with aortic insufficiency in systemic lupus erythematosus. 1125 92

Hydroxychloroquine, a quinoline compound, rarely causes ototoxicity. According to the few reports in existence, hydroxychloroquine-induced ototoxicity occurred following prolonged therapy in adult patients with rheumatoid arthritis and lupus erythematosus. We report a case of unilateral sensorineural hearing loss in a 7-year-old girl with idiopathic pulmonary haemosiderosis, after 2 years of hydroxychloroquine treatment. Sensorineural hearing loss has previously been reported with hydroxychloroquine treatment, but this is the first report in a child and associated to idiopathic pulmonary haemosiderosis and has the characteristic of being unilateral.
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PMID:Hydroxychloroquine ototoxicity in a child with idiopathic pulmonary haemosiderosis. 1173 95

Systemic lupus erythematosus is a disease of unknown cause that manifests with tissue and cellular alterations due to the deposition of antibodies and pathogenic immune complexes. The disease can be associated with anticardiolipin antibody syndrome, a disorder of recurrent vascular thrombosis and thrombocytopenia associated with a persistent anticardiolipin test positivity. In this report, we describe the case of a young woman affected by lupus erythematosus and positive for anticardiolipin antibodies who was brought to our observation for a sudden sensorineural hearing loss in the right ear. The patient was successfully treated with osmotic therapy and, 13 months after the initial symptom, is now in good clinical condition with no recurrence of the hearing loss and normal bilateral hearing. Together with a critical review of the pertinent literature, this rare clinical case led us to formulate some original comments.
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PMID:Sudden sensorineural hearing loss in lupus erythematosus associated with antiphospholipid syndrome: case report and review. 1582 71

The objective of this study was to establish the frequency of involvement of the auditory apparatus in 45 female patients with systemic lupus erythematosus (SLE) submitted to general clinical and laboratory assessments, and tone and vocal audiometry accompanied by a questionnaire evaluating auditory symptoms. The control group consisted of 45 healthy women, matched by age. Auditory symptoms were present in 25 (55.5%) patients, with a diagnosis of sensorineural hearing loss in seven (15.6%) patients. A significant correlation with hypoacusis (P < 0.001), ear fullness (P = 0.012) and tinnitus (P = 0.017) was observed in patients with hearing loss. None of the clinical or laboratory parameters showed an association with sensorineural hearing loss. In the control group, three women (6.7%) presented audiometric alterations, including two with altered tympanometry results and one with mild sensorineural hearing loss. In conclusion, an adequate investigation of auditory symptoms is important during the follow-up of patients with SLE, since manifestations of the auditory apparatus and sensorineural hearing loss can affect a significant proportion of patients.
Lupus 2007
PMID:Sensorineural dysacusis in patients with systemic lupus erythematosus. 1804 93

Immune-mediated sensorineural hearing loss may complicate systemic autoimmune diseases. We have previously reported the presence of antibodies directed against inner ear antigens in patients with Cogan syndrome, a disease characterized by sudden hearing loss and interstitial keratitis. Such autoantibodies cross-react with an epitope of SSA/Ro60 protein. Anti-Ro/SSA antibodies in pregnant women cross the placenta and reach the fetal tissues inducing an immune-mediated damage of the cardiac conduction system. We wanted to evaluate whether mothers with anti-Ro/SSA antibodies who gave birth to children with congenital heart block have antibodies directed against inner ear antigens and whether these antibodies are connected with the presence of immune-mediated sensorineural hearing loss. We did not find anti-inner ear antibodies in the majority of the mothers. On the contrary a 13-year-old boy with congenital heart block and sensorineural hearing loss was positive for the presence of anti-inner ear antigens antibodies. Moreover his serum was positive for the presence of anti-Ro60 peptide antibodies but did not recognize the entire protein Ro60 (TROVE2), a behaviour similar to that of sera from patients with Cogan syndrome. In conclusion the data obtained so far show that anti-inner ear antibodies do not recognize the entire protein TROVE2 and do not support the hypothesis that such antibodies may be involved in the pathogenesis of congenital heart block.
Lupus 2017 Jul
PMID:Congenital heart block and immune mediated sensorineural hearing loss: possible cross reactivity of immune response. 2791 50

Rowell's syndrome (RS) is recognized since 1963 as a presentation of lupus erythematosus (LE) with erythema multiforme-like lesions and characteristic immunological changes. Antiphospholipid syndrome (APS) encompasses antibodies to phospholipids/phospholipid-binding cofactor proteins and/or circulating lupus anticoagulant with clinical manifestations of thrombosis such as recurrent spontaneous abortions, etc. A 32-year-old female with primary infertility since marriage (7 years) and sequential appearance, for the past 6 months, of various lupus-specific cutaneous lesions, "targetoid" lesions on palms, musculoskeletal, ocular and auditory (sensorineural hearing loss [SNHL]) complaints with positive serological profile for SMD-1, SS-A/Ro, SS-B/La, U1-snRNP, Ku, antiphospholipid and anticardiolipin antibodies, and histopathologically confirmed LE is presented as a case of concurrent overlap syndrome, RS and APS, primary infertility, and SNHL. A brief review of RS including analysis of data of cases reported from India hereto vis-a-vis that of cases worldwide up to 2012 has also been done.
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PMID:Overlap Syndrome with Rowell's Syndrome, Antiphospholipid Syndrome, Primary Sterility, and Sensorineural Hearing Loss: A Case Report, Brief Review, and Analysis of Cases of Rowell's Syndrome Reported from India and Abroad. 3021 Jan 66