Laura Zavota

Rituximab ameliorated severe hearing loss in Cogan's syndrome: a case report

BackgroundRituximab is a monoclonal antibody inducing depletion of B lymphocytes and presently approved for the treatment of non-Hodgkins lymphoma and rheumatoid arthritis. Here is the first report of the use of this drug in a case of Cogans syndrome (CS).Case Presentationa 25-year-old Italian woman was referred with conjunctival hyperaemia, interstitial keratitis, moderate bilateral sensorineural hearing loss accompanied by tinnitus, dizziness, nausea and vertigo, poorly responsive to oral and topical steroidal therapy. Diagnosis of typical CS was made. The administration of a combined immunosuppressive treatment resolved ocular inflammation, dizziness, nausea, and vertigo but gave little results in controlling progressive hearing loss. A noticeable improvement in hearing function was documented by pure tone audiometry after infusion of Rituximab.Discussionin CS, hearing function is often the most difficult parameter to control with therapy. A positive effect of Rituximab on was observed in our case. The drug also allowed to significantly reduce the number of adjuvant immunosuppressive medications.

Anti-68 kDa antibodies in autoimmune sensorineural hearing loss: are these autoantibodies really a diagnostic tool?

Objectives: Autoimmune sensorineural hearing loss (ASNHL) is a relatively rare disorder which can lead to total deafness. At present, no specific laboratory test with adequate sensitivity and specificity is available to confirm the clinical suspicion of ASNHL. The aim of this study was to identify if evaluation of anti-hsp70 antibodies is an accurate diagnostic tool in patients affected by ASNHL. Study design: Prospective study. Methods: During 4-year (2001–2005), all patients with SNHL who were referred to the Eye, Ear, Nose and Throat Department of Parma University, Italy, underwent specific tests to determine the autoimmune origin of the disease. Patients with a consistent suspicion of ASNHL underwent the routine serologic tests and a test for determination of anti-hsp70 antibodies. The same patients were divided into three groups: (1) idiopathic ASNHL; (2) ASNHL associated with ocular inflammation, i.e. Cogans Syndrome; (3) ASNHL associated with a systemic autoimmune disease (SAD). The control group included: (1) healthy subjects; and (2) patients affected by SAD, without any ocular or audiovestibular disease. Results: 88 subjects (67 patients, defined as “study group”, and 21 controls) were evaluated. Anti-hsp70 antibodies were isolated in 52% of the study group patients, and in 4% of the control group (χ2 = 13.009, p < 0.01). In the idiopathic ASNHL patients, 59.5% were found positive for anti-hsp70 antibodies. About 50% of patients affected by CS and 37.5% of patients affected by SAD with SNHL were found positive. In the control group, anti-hsp70 antibodies were found in 8.3% of healthy subjects and in none of the patients with SAD and no hearing loss. Conclusions: The present study confirms the value of the anti-hsp70 test in the serological diagnosis of autoimmune hearing loss. It is still the only available diagnostic marker that identifies an autoimmune origin of hearing loss.

Autoimmune uveitis in children: clinical correlation between antinuclear antibody positivity and ocular recurrences.

Abstract.Objective. The aim of this study was to identify the correlation between antinuclear antibody (ANA) titre and the onset and clinical course of uveitis in children with juvenile idiopathic arthritis (JIA) or without any other systemic autoimmune disease, i.e., idiopathic uveitis (IU). Methods. Twenty-two patients affected by uveitis were examined. Ten had JIA-associated uveitis, 12 had IU. Follow-up ranged from 7 to 101 months. The ANA were titrated three times per year and additionally in case of ocular recurrences. All patients were treated with immunosuppressive drug combination therapy (IDCT). Results. JIA-associated uveitis: ocular recurrences were noted in three ANA-positive patients and in one ANA-negative patient. IU uveitis: ocular recurrences were noted in one ANA-positive and in one ANA-negative patient. No significant rise in ANA titre was noted in either group during uveitis recurrence. Conclusions. (1) ANA had no value in predicting the recurrence of uveitis. (2) IDCT does not influence ANA production.

Syphilitic interstitial keratitis: treatment with immunosuppressive drug combination therapy.

Objective: The following is a case presentation of congenital syphilitic keratitis in a boy 6 years of age who was successfully treated with an immunosuppressive drug combination therapy. Methods: Congenital syphilitic keratitis was diagnosed by clinical findings and laboratory tests. The child was unresponsive to traditional treatment; thus, systemic immunosuppressive therapy, which consisted of oral cyclosporine 4 mg/kg/d, 6 days per week, and oral low-dose steroids (fluocortolone 0.8 mg/kg a week, given every other day), was initiated. Results: Corneal disease showed great improvement with this therapy, with progressive healing of lesions in the first month of treatment and no signs of toxic renal, hepatic, or growth abnormalities. Recurrences of uveitis have not occurred, and corneal interstitial keratitis episodes have been limited to 3 in an 8-year period. After 6 months with no recurrences, a tapering off of the systemic therapy was initiated, and the child is still asymptomatic and without flare-ups. Conclusions: Congenital syphilitic keratitis is usually treated with topical steroids and cycloplegic drugs, which not only can be ineffective but can also lead to complications such as cataract and glaucoma. In the present case report, a pediatric patient affected by syphilitic interstitial keratitis was treated successfully with an immunosuppressive drug combination therapy.

Discontinuous drug combination therapy in autoimmune ocular disorders

Purpose:  This study aimed to assess the effectiveness of a steroid‐sparing immunosuppressive treatment (IST) protocol in the control of severe or steroid‐resistant autoimmune ocular inflammatory diseases.

Autoimmune disorders: a concept of treatment based on mechanisms of disease

The history of autoimmunity is almost as old as the history of immunology. In 1890 Behring and Kitisato [1], working in Koch’s institute, demonstrated that the serum of animals immunized against attenuated diphtheria toxins can be used as a preventive or therapeutic inoculation against diphtheria in other animals through a specific neutralization of the toxin of the disease. This discovery initiated the long history of the elucidation of the immune response. Just 10 years later Metalnikof [2] challenged the possibility of an immune response against self-constituents of the organism on the basis of his studies on guinea pigs immunized with spermatozoids. A few years later Chauffard and Vincent [3] reported the first case of autoimmune hemolytic anemia (AIHA) and Fiessinger [4] described anti-liver antibodies in patients suffering from chronic active hepatitis (CAH). Nevertheless, in the 1950s the idea of the immune apparatus being used against self-antigens was still considered absurd. The very existence of autoimmune disorders was questioned. Until recently, multiple sclerosis (MS) was considered a viral disease, and rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE) to be caused by infectious agents, especially of the mycoplasma family. Interest in autoimmune diseases grew slowly but constantly in view of the intensive research being carried out in experimental animals as well the clinical research being carried out on thyroid disorders, RA, SLE, and MS. It is now known that autoimmunity is not a rare event as was thought 50 years ago but is a frequent condition with over 40 recognized autoimmune diseases affecting more than

Factitious Pseudo-Membranous Conjunctivitis in an Adolescent Boy

Background/Aims: Ocular factitious lesions involving the conjunctiva alone represent a challenging diagnosis for the ophthalmologist; corneal integrity, in fact, allows maintenance of good visual acuity and precludes the pain subsequent to trigeminal stimulation. Conjunctival biopsy is crucial to make a diagnosis and to focus on possible peculiarities in the patient’s behavior. A psychiatrist has to confirm the diagnosis. In this case report, images of a bilateral pseudo-membranous conjunctivitis sparing the cornea in an anorexic adolescent boy are shown. Methods: Photographically documented case report. Results: A fourteen-year-old Italian boy was referred with a diagnosis of bilateral chronic conjunctivitis unresponsive to systemic and topical antibiotic and steroidal treatment. It had lasted for 4 months and was concomitant with an 8-kg weight loss. Conjunctival biopsy revealed cotton wool fragments. The patient admitted an unsafe behaviour lasting for months. A diagnosis of factitious conjunctivitis was made, and confirmed by a psychiatric assessment. Conclusion: Factitious lesions of the eye involve not only anatomical structures situated on the visual axis causing a reduction of visual acuity, but may also involve the conjunctiva alone. A thorough clinical history should identify the source of the patient’s anxiety. Moreover, close cooperation between ophthalmologists and a psychiatrist can further clarify the diagnosis.

Staphylococcus aureus and autoimmune uveitis reactivation in childhood: a possible correlation?

The role of infectious agents in autoimmune diseases has been the subject of several studies and is still under investigation. Here a paediatric case series of autoimmune uveitis is reported. An exacerbation of the ocular inflammation occurred in concomitance with nasal colonisation by Staphylococcus aureus.

Effect of a drug combination treatment on ocular perfusion in recurrent idiopathic intermediate uveitis

Purpose: To test the effect of a drug combination therapy on ocular perfusion in human eyes affected by idiopathic intermediate uveitis. Methods: Seven patients (12 eyes) showing active signs of intermediate uveitis, with at least two more similar episodes reported within the previous 12 months, were enrolled in a prospective case series. Two fellow healthy eyes of two of the enrolled patients were studied as internal controls. Color Doppler imaging of the central retinal artery (CRA), the ophthalmic artery (OA), and the posterior ciliary arteries (PCAs) was performed at the time of enrollment, and at 6 and 12 months after starting treatment with oral fluorocortolone, cyclosporine, and parenteral methotrexate. The best-corrected visual acuity was concurrently measured as a second parameter. Results: In the 12 affected eyes, the mean visual acuity (±SD) improved from 0.15(±0.12) to 0.04(±0.18) LogMAR (paired samples Student’s t -test: p = 0.015). The resistivity index (RI ± SD) of the CRA decreased from 0.81(±0.13) to 0.71(±0.13)(p = 0.0091). Further, the variation of the RI in the PCAs reached a borderline significance (p = 0.062), decreasing from 0.71(±0.12) to 0.61(±0.12). No significant changes were observed in the OA. Moreover, eyes showing a visual improvement of =.01 (LogMAR) were more likely to show a =10% improvement of the RI for the CRA (Fisher’s exact test: p = 0.018; power = 90%; alpha probability = 5%; odds ratio = 2,4). Conclusions: In eyes affected by idiopathic intermediate uveitis, treated with a systemic drug combination therapy, the improvement of the visual acuity seems to correlate with a proportional improvement of the retrobulbar circulation.