Infliximab as successful treatment option in a case of adenosine deaminase 2 deficiency.

Deficiency of adenosine deaminase 2 (DADA2) is a recessively inherited autoinflammatory disease characterized by systemic inflammation and immunodeficiency. Infliximab proved to be favorable in the treatment of this condition. This case report is concerned with a DADA2 deficient patient treated with infliximab. This is a rare case of DADA2 in a 32-year-old female patient. The patient was admitted with a clinical presentation of erythema, ulcers, and pruritus on both legs and ankles, accompanied by red ulcerative oral lesions, fatigue, malaise, and dizziness. The patient's genetic analysis was positive for DADA2. Treatment based on TNF-α inhibition was highly effective for this patient. We used laboratory testing and punch biopsy as differential diagnostic tools, where antinuclear antibody positivity, high prolactin levels, and high serum C-reactive protein were observed. The punch biopsy revealed both orthohyperkeratosis and parahyperkeratosis of the dermis, diffuse core fragments, plasma in the stratum corneum, and hypergranulous acanthosis. DADA2 treatment is centered on tumor necrosis factor α suppression. Although high-dose systemic glucocorticoids can reduce inflammation in the initial stages of the disease, most patients have a resistant or relapsing response to tapering attempts. The prevalence of undiagnosed cases of autoinflammatory diseases is anticipated to diminish with the growing awareness of them.

Juvenile and adult-onset scleroderma: Different clinical phenotypes.

OBJECTIVES: Systemic sclerosis (SSc) represents extremely rare disease with majority of data coming from adults. Studies comparing juvenile- (jSSc) and adult-onset (aSSc) patients are limited. We aimed to compare clinical features, treatment modalities and survival rates of jSSc and aSSc patients. METHODS: A retrospective study among pediatric and adult Scl patients has been performed. Demographic characteristics, clinical features, autoantibody profiles, and treatment data were retrieved from the databases. Survival analysis was done using Kaplan-Meier plot and factors associated with mortality were identified with multiple regression analysis. RESULTS: A total of 158 adults and 58 juvenile Scl patients were identified. The mean age at the disease onset was 37±14.7 vs. 8.8 ± 4.1 years, mean age at diagnosis 42±15.2 vs. 10.4 ± 3.8 years and mean follow-up duration was 6.3 ± 4.9 years vs. 6.6 ± 4.9 years for aSSc and jSSc patients, respectively. The frequency of interstitial lung disease (ILD) (50.9% vs 30%, p<0.001) and systemic hypertension (17.9% vs 0, p = 0.009) was significantly higher among aSSc. While aSSc patients had presented mostly with limited cutaneous subset (74.1%), diffuse cutaneous subset was the dominant subset among jSSc (76.7%), (p<0.001). The mortality rate was significantly higher among adults (p = 0.005). The ILD (p = 0.03) and cardiac insufficiency (p = 0.05) were independent risk factors of mortality in both aSSc and jSSc patients. CONCLUSION: Juvenile and adult-onset Scl represent rarely seen conditions with different clinical phenotypes. Pediatric patients with LS are more commonly seen by pediatric rheumatologists, in contrary to adults. Diffuse disease subset is the dominant form among juvenile patients, whereas limited form is the main disease subset among adults. On the other hand, juvenile-onset patients have a better survival than those with adult-onset.

Comparison of vision-related quality of life in primary open-angle glaucoma and dry-type age-related macular degeneration.

PurposeTo compare quality of life (QoL) in patients with primary open-angle glaucoma (POAG) and dry-type age-related macular degeneration (AMD) with similar best-corrected visual acuity.MethodsAge-, sex-, and visual acuity-matched POAG and dry AMD patients were included in the study. Each patient performed 24-2 and 10-2 SITA standard visual field tests. Contrast sensitivity was evaluated with CSV-1000 HGT instrument. The 25 item National Eye Institute Visual Function Questionnaire (NEI-VFQ-25) was used to analyze QoL. Overall and subscale scores were converted to scores between 0 and 100, the higher scores indicating better vision-related QoL.ResultsOverall NEI-VFQ-25 scores were 86.44 and 84.66 in glaucoma and AMD groups, respectively (P=0.244). The highest scores were obtained in 'vision-related dependency' subgroup in glaucoma and 'color and peripheral vision' in AMD group, whereas the lowest scores were noted 'in peripheral vision' in both glaucoma and AMD patients. Glaucoma patients had significantly lower scores in ocular pain, color vision, and peripheral vision subgroups compared with the AMD group, whereas AMD patients had lower scores in near and distance vision activities, vision-related social activity, and dependency subgroups. Contrast sensitivity results and mean defect values showed correlation with NEI-VFQ-25 scores in both groups.ConclusionsGlaucoma and AMD patients with similar visual acuity experienced similar overall impairment in QoL. However, glaucoma patients described more difficulty with peripheral vision and ocular pain, whereas AMD patients complained more about near and distance vision and dependency items.

Frequent oral ulceration during early disease may predict a severe disease course in males with Behçet's syndrome.

OBJECTIVES: The numbers and recurrence rates of mucocutaneous manifestations can be highly variable among patients with Behçet's syndrome (BS) but it is not known whether these differences influence the disease course at the long-term. METHODS: We evaluated the outcome of 30 patients that made up the placebo arm of a 6 months controlled trial of thalidomide and looked at the relation between the frequencies of mucocutaneous manifestations during the trial and the development of major organ involvement necessitating immunosuppressives during the post-trial period. RESULTS: Fifteen (50%) patients had received immunosuppresives for major organ involvement during the post-trial period. Patients receiving immunosuppressive treatment were significantly younger at the onset of BS compared to those who did not (24.5±5 vs. 29.7±3.8 SD years; p=0.003). The mean number of oral ulcers recorded throughout the trial was significantly higher among patients using immunosuppressives compared to those who did not (2.09±0.96 vs. 1.43±0.8; p=0.029). This significance disappeared when adjusted for age of onset of BS (p=0.16). ROC curve analysis showed that having 10 or more ulcers during 6 months has a sensitivity of 86.7% and a specificity of 53% for the subsequent necessity of immunosuppressive use. The same association was not true for genital ulcers, follicular lesions and erythema nodosum. CONCLUSIONS: These findings on a limited number of patients suggest that frequent occurrence of oral ulceration during the initial years of the disease may predict the development of major organ involvement in men with BS.

Behçet syndrome: is it one condition?

Behçet's syndrome (BS) is a disease of unknown etiology, and as such, there have been efforts to classify BS within the popular nosological identities of the times such as seronegative spondarthritides, autoimmune, and more recently autoinflammatory diseases. Current evidence suggests that BS does not easily fit into any one of these lumps, while on occasion, it might be impossible to tell BS from Crohn's disease, especially when the main clinical presentation is intestinal ulceration. There are distinct regional differences in disease expression of BS with fewer cases of intestinal disease in the Mediterranean basin and less severe eye disease and less frequent skin pathergy among patients reported from northern Europe or America. The clustering of symptoms, especially with the recently described increased frequency of the acne/arthritis cluster in familial cases, suggests that more than one pathological pathway is involved in what we call BS today. Supportive evidence for this contention also comes from the observations that (a) the genetic component is very complex with perhaps different genetic modes of inheritance in the adult and in the pediatric patients; and (b) there are differing organ responses to one same drug. For example, the anti-TNF agents successfully control the oral ulcers while they have no effect on the pathergy reaction.

Anti-Saccharomyces cerevisiae (ASCA) and anti-neutrophil cytoplasmic (ANCA) antibodies are not increased in Takayasu arteritis.

OBJECTIVES: Takayasu arteritis (TA) may be associated with inflammatory bowel disease (IBD). As there is such an overlap and since both diseases show granulomatous histopathological lesions we reasoned similar biological pathways might be implicated in both conditions. Therefore, we investigated the prevalence of anti-Saccharomyces cerevisiae (ASCA) and anti-neutrophil cytoplasmic (ANCA) antibodies - serological markers for IBD-among patients with TA. METHODS: Thirty-two patients with TA, 21 with Crohn's disease (CD), 17 with ulcerative colitis (UC) and 34 healthy controls were studied. Among 32 patients with TA, 2 had CD and one had UC concomitantly. ASCA Ig A and Ig G antibodies were analyzed using a commercial ELISA kit. Immune fluorescence analysis (IFA) was used to assess the presence of ANCA antibodies. RESULTS: Only patients with CD had significantly higher levels of both ASCA Ig A and Ig G, compared to patients with TA and healthy controls. Similarly, the frequency of ASCA positive patients was higher only among patients with CD. ASCA Ig A and Ig G antibodies were found in 16 % (5/32) of patients with TA. Among 3 patients, in whom TA and IBD co-existed, only one (one with CD) had positive ASCA Ig G and A antibodies. The p-ANCA antibodies were present among patients with UC (35 %) and CD (10 %). CONCLUSION: ASCA positivity in TA was similar to that found in UC and healthy controls. No ANCA antibodies were detected among patients with TA.

Plasma viscosity, an early cardiovascular risk factor in women with subclinical hypothyroidism.

OBJECTIVE: It is controversial, if subclinical hypothyroidism increases cardiovascular risk. Plasma viscosity is a hemorheological parameter, which is accepted as an early cardiovascular risk factor. We investigated the alterations in plasma viscosity in women with subclinical hypothyroidism. DESIGN: 40 female patients with subclinical hypothyroidism and 31 age- and weight-matched healthy women were included. Free thyroxine (FT4), thyroid stimulating hormone (TSH), lipid parameters, fibrinogen, C-reactive protein (CRP) levels, hematocrit and plasma viscosity were measured in all subjects. MAIN OUTCOME: Plasma viscosity, total cholesterol and low density lipoprotein were significantly increased and high density lipoprotein was significantly decreased in patients with subclinical hypothyroidism. No significant correlation was found among the parameters. CONCLUSION: Increased plasma viscosity in patients' group suggests that cardiovascular risk might be increased in patients with subclinical hypothyroidism. As far as we could reach, this is the first study concerning plasma viscosity in subclinical hypothyroidism.

Prevalence of angina, myocardial infarction and intermittent claudication assessed by Rose Questionnaire among patients with Behcet's syndrome.

OBJECTIVE: To determine the risk of clinical cardiovascular disease in middle-aged patients with Behcet's syndrome (BS) compared with gender-matched non-BS subjects. METHODS: The prevalence of angina, myocardial infarction (MI), doctor diagnosed ischaemic heart disease (IHD) and intermittent claudication were sought by the Rose Angina Questionnaire in 225 (141 M/84 F) BS patients (mean age: 52 +/- 8) with BS and 117 (74 M/43 F) controls (mean age: 50 +/- 5). Information on atherosclerotic risk factors was also collected. RESULTS: The prevalence of angina, MI and doctor-diagnosed IHD were not different between BS patients and non-BS controls in the whole study population and when males and females were separately analysed. Angina tended to be more common among females compared with males among both patients and controls. Intermittent claudication was found to be significantly more common among BS patients, especially in males with venous disease. CONCLUSIONS: The findings in this cross-sectional clinical study are in line with previous observations not indicating accelerated atherosclerosis in BS. Intermittent claudication might not be a suitable tool for the detection of peripheral atherosclerotic disease especially among BS patients having venous disease.

Anti-Scl-70 antibodies in autoimmune hypothyroidism.

The relationship between autoimmune thyroiditis and systemic sclerosis is controversial. Data exist on the presence of thyroid autoantibodies in patients with systemic sclerosis but, as far as we could ascertain, anti-Scl-70 antibodies, which are highly specific for systemic sclerosis, have not been investigated in autoimmune hypothyroidism. This study compares the presence of anti-Scl-70 in females with autoimmune hypothyroidism (n = 24) and in healthy age-matched female controls (n = 26). Free thyroxine levels were similar in both groups. Thyroid stimulating hormone (TSH), antithyroid peroxidase (anti-TPO), antithyroglobulin (anti-Tg) and index values for anti-Scl-70 levels were significantly higher in patients with autoimmune hypothyroidism compared with controls, although the anti-Scl-70 test was negative in both groups. Anti-TPO, anti-Tg and TSH significantly correlated with anti-Scl-70. In conclusion, autoimmune hypothyroidism seems to be associated with a higher index level of anti-Scl-70, yet a negative anti-Scl-70 antibody test. This suggests that autoimmune hypothyroidism might have common aetiological factors with systemic sclerosis.

Treatment options in colchicine resistant familial Mediterranean fever patients: thalidomide and etanercept as adjunctive agents.

OBJECTIVE: Regular colchicine treatment cannot control the typical febrile attacks of FMF in about 5-10% of the compliant patients. Here we report the effect of thalidomide and etanercept in 5 colchicine-resistant cases. METHODS: Five (4M/ 1F) FMF patients between April 2005 and March 2006, who were experiencing at least 2 attacks per month, despite regular colchicine were included to the study. Four male patients were given thalidomide 100 mg/d initially. Two of these patients unresponsive to thalidomide were prescribed subcutaneous injections of etanercept 25 mg, twice a week. The female patient received etanercept as the first choice due to potential side effects. She then had to be converted to thalidomide due to a severe injection site reaction. RESULTS: The median follow up period with thalidomide and etanercept was 8 months. Both thalidomide and etanercept lowered the number of the abdominal attacks. CONCLUSION: Thalidomide and etanercept might be effective as additional treatment in colchicine-resistant cases of FMF.

Atherosclerosis in Takayasu arteritis.

OBJECTIVE: Chronic inflammatory diseases such as systemic lupus erythematosus (SLE) and rheumatoid arthritis are associated with accelerated atherosclerosis. We hypothesised that atherosclerosis may also be increased in Takayasu arteritis. METHODS: The frequency of atherosclerotic plaques and the intima-media thickness (IMT) were investigated in 30 female patients with Takayasu arteritis (mean age (standard deviation), 35.4 (8.0) years), along with 45 sex-matched and age-matched patients with SLE (37.4 (6.8)) and 50 healthy controls (38.2 (5.7)). Plaques were scanned and IMT was measured at both sides of the common carotids, carotid bulb, and internal and external carotid arteries by B-mode ultrasonography. Traditional risk factors for atherosclerosis were also assessed. RESULTS: Most of the atherosclerotic risk factors were comparable between patients with Takayasu arteritis and SLE. More atherosclerotic plaques were observed among patients with Takayasu arteritis (8/30; 27%) and those with SLE (8/45; 18%) than among the healthy controls (1/50; 2%; p = 0.005). Logistic regression analyses showed that the presence of a plaque was associated only with age in both Takayasu arteritis and SLE (p = 0.04 and 0.02, respectively). The mean overall IMT was significantly higher among patients with Takayasu arteritis (0.95+/-0.31 mm) than among the patients with SLE (0.58+/-0.10 mm) and the healthy controls (0.59+/-0.08 mm; p<0.001). CONCLUSION: Patients with Takayasu arteritis have a high rate of atherosclerotic plaques, at least as frequent as that observed among patients with SLE.

Anti-Saccharomyces cerevisiae antibodies (ASCA) in Behçet's syndrome.

OBJECTIVES: Anti-Saccharomyces cerevisiae antibodies (ASCA) are found in 50-60% of patients with Crohn's disease. Increased as well as normal levels have been reported in Behçet's syndrome (BS). We reassessed the level of IgG and IgA ASCA antibodies in BS and in a group of diseased and healthy controls. METHODS: Eighty-five patients with BS were studied along with 20 patients with ankylosing spondylitis (AS), 24 with Crohn's disease (CD), 25 with ulcerative colitis (UC) and 21 healthy volunteers. A commercial ELISA kit was used (Inova Diagnostics). RESULTS: It was only the patients with CD who had significantly higher levels of antibodies compared with the rest of the group (ANOVA: ASCA IgG, p = 0.0001; ASCA IgA, p = 0.0001). 42% of CD, 4% of BS, 4% of UC and 15% of AS patients had a positive IgG+IgA ASCA. There was a significant trend for patients with gastrointestinal (GI) involvement with BS (n = 8) to be more positive for IgG and IgG+IgA ASCA compared to the rest of the patients with BS (n = 77) (Chi-square, IgG, p = 0.02, IgG+IgA, p = 0.001). CONCLUSION: The rate of positivity of ASCA in BS is comparable to that observed among patients with UC and AS. Patients with BS who have GI involvement may have higher levels of ASCA and this needs to be further studied.

Aspergillus niger infection of exenterated orbit.

PURPOSE: To present two immunocompetent patients with Aspergillus niger infection of the exenterated orbit. METHODS: Case reports. RESULTS: The patients were treated with oral itraconazole and socket irrigation with amphotericin B. The sockets cleared completely; no growth was observed on reculturing. CONCLUSIONS: A niger infection of an exenterated socket can be effectively treated with oral itraconazole and topical amphotericin B in immunocompetent patients.

Relationship between structural abnormalities and short-wavelength perimetric defects in eyes at risk of glaucoma.

PURPOSE: To determine the relative prevalence of blue-yellow perimetric defects and structural abnormalities of the optic nerve and nerve fiber layer in eyes at risk of glaucoma. METHODS: Seventy-two eyes (of 72 patients) at risk of glaucoma, with normal white-on-white full threshold perimetry, were examined prospectively with blue-yellow full-threshold perimetry (Humphrey). Structural evaluations were conducted with qualitative assessment of stereoscopic color optic disk photographs and monochromatic nerve fiber layer photographs performed independently by three masked examiners (a glaucoma specialist and two glaucoma fellows), and statistical analysis of summary parameters was obtained with scanning confocal laser tomography (abnormal defined as values outside 95% confidence limits established in normal control subjects). RESULTS: Kappa values for interobserver agreement were 0.64, 0.88, and 0.79 for optic disk evaluation and 0.59, 0.60, and 0.61 for nerve fiber layer evaluation. Thirteen (18%) of 72 eyes had blue-yellow abnormalities. A total of 30 eyes (42%) were identified as having a structural abnormality; 29 (40%) had qualitatively determined optic disk abnormalities, 21 (29%) had qualitatively determined nerve fiber layer defects, and 26 (36%) had statistically significant structural abnormalities. Twelve of 13 eyes with blue-yellow defects had a detectable structural abnormality; all 12 had abnormalities identified with disk photography, nine with nerve fiber layer photography, and 12 by scanning laser tomography. CONCLUSIONS: Clinically detectable structural abnormalities frequently coexist with blue-yellow perimetric defects in patients with ocular hypertension. A substantial proportion of patients with ocular hypertension with normal blue-yellow perimetry has early detectable glaucomatous structural abnormalities.

Necrobiotic xanthogranuloma: long-term outcome of ocular and systemic involvement.

PURPOSE: To report long-term outcomes of patients with necrobiotic xanthogranuloma, to investigate the propriety of therapeutic surgical excision or debulking, and to study tissue specimens by immunoperoxidase staining and in situ hybridization. METHODS: Medical records of all patients at the Mayo Clinic, Rochester, Minnesota, with necrobiotic xanthogranuloma between 1980 and 1997 were reviewed. A follow-up letter was sent to each patient inquiring about the current status of the lesions, the treatment regimen, and associated systemic diseases. RESULTS: The average age (+/- standard deviation) of the 15 men and 11 women was 56.8 +/- 14.8 years. Of the 26 patients, 21 (81%) had lesions of the ocular adnexa. Ulceration of the lesions occurred in 11 patients (42%). The lesions recurred after surgical removal in 11 patients (42%) and on prior incision sites from unrelated operations in three patients (12%). The average duration of follow-up from the appearance of characteristic skin lesions was 10 +/- 6.1 years. Four patients had multiple myeloma, five had a plasma cell dyscrasia, and one had a lymphoproliferative disorder during this period. Time to development of associated malignancy ranged from 8 years before the skin lesions to 11 years after the skin lesions. Overall survival was 100% at 10 years and 90% at 15 years (95% confidence limit, 0.73 to 1.00). Immunoperoxidase stains demonstrated that most histiocytes are not of Langerhans cell lineage. Monoclonal immune globulins were not identified in tissue specimens. CONCLUSION: Care of patients with necrobiotic xanthogranuloma should include avoidance of surgical removal, if possible, and lifelong follow-up to detect the development of associated malignancy.

Arterial blood flow characteristics in central retinal vein occlusion and effects of panretinal photocoagulation treatment: an investigation by colour Doppler imaging.

AIMS: To determine whether an increase in vascular resistance in the central retinal and ophthalmic arterial circulations contributes to the development of central retinal vein occlusion (CRVO), or haemodynamic alterations in central retinal and ophthalmic arteries occur secondary to the vein occlusion as increased intravascular pressure is transferred through the capillary bed to the arterial side and the effect of panretinal photocoagulation treatment on these circulations in ischaemic cases. METHODS: The ophthalmic and central retinal arteries of the affected and non-affected eyes of 20 patients with non-ischaemic CRVO, 13 patients with ischaemic CRVO, and 22 control subjects were investigated by colour Doppler imaging. Panretinal photocoagulation (PRP) treatment was applied to the eyes with ischaemic CRVO. Maximum and minimum blood flow velocities, and resistivity indexes were calculated in the affected and healthy eyes of patients and in the control eyes. RESULTS: Average blood flow velocity in the central retinal and ophthalmic arteries of patients with non-ischaemic CRVO did not differ from their fellow eyes, but a significantly lower average blood flow velocity was found in the ophthalmic and central retinal arteries of the patients with ischaemic CRVO compared with their fellow eyes. Patients with ischaemic CRVO had significantly lower blood flow velocities in their ophthalmic and central retinal arteries than non-ischaemic cases that were further reduced following PRP treatment. CONCLUSION: This study suggests that impaired arterial blood flow observed in patients with CRVO may be partly related to secondary changes in the retrobulbar arterial circulation as a result of enhanced arterial resistance following CRVO. These data also demonstrate that PRP treatment decreases retinal and ophthalmic blood flow velocities in patients with ischaemic CRVO.

Factitious disease of periocular and facial skin.

PURPOSE: To describe the clinical appearance of factitious (or self-inflicted) lesions on periocular skin and face. METHODS: All patients with factitious cutaneous disease who were examined at Mayo Clinic, Rochester, Minnesota, between 1985 and 1997 were identified. For patients with lesions on the face and periocular skin, the demographic features, clinical descriptive characteristics of their lesions, associated psychopathology, and treatments were ascertained. RESULTS: Of 38 patients with factitious dermatitis, 18 (47%) had facial lesions. Of these 18 patients, 15 (83%) were female. The mean age (+/- SD) of the patients with facial lesions was 35.2 +/- 15.7 years (range, 9 to 66 years). Eight patients (44%) had neurotic excoriations, nine (50%) had dermatitis artefacta, and one (6%) had trichotillomania. The working diagnoses of five patients cared for initially in the Department of Ophthalmology were corneal epithelial and facial desquamation associated with severe pain of unknown cause, medial cicatricial ectropion of probable vasculitic cause, basal cell carcinoma of the nasojugal fold, recurrent preseptal cellulitis resistant to medical treatment, and madarosis of the upper eyelids of unknown cause. CONCLUSION: Cutaneous factitious disease may masquerade as numerous clinical entities and should be included in the differential diagnosis of lesions of the periocular skin.

Slope of the peripapillary nerve fiber layer surface in glaucoma.

PURPOSE: To develop structural markers of early glaucomatous optic nerve damage with confocal scanning laser ophthalmoscopy. METHODS: Custom software was developed to analyze the images of 53 patients with open-angle glaucoma and 43 healthy subjects (matched for age, race, and refractive error), with images acquired with a confocal scanning laser ophthalmoscope. Height values were obtained along radial profiles of the peripapillary nerve fiber layer surface at 5-degree intervals around the disc edge. Two new parameters were derived: mean height and mean slope of the peripapillary nerve fiber layer surface. Mean slope was tested for its independence from a retinal reference plane. A logistic regression analysis was used to determine functions of disease probability. Receiver-operating characteristic (ROC) curves were used to evaluate sensitivity and specificity of peripapillary nerve fiber layer slope and height to discriminate normal subjects from glaucoma patients. RESULTS: Mean (+/-SD) visual field mean deviation in the glaucoma group was -4.8 +/- 3.3 dB. Mean slope (+/-SD) of the peripapillary nerve fiber layer was significantly (P < 0.001) steeper (0.30 +/- 0.12) in glaucoma patients than in healthy subjects (0.11 +/- 0.12). Mean slope values were identical with or without the retinal reference plane. Mean height (+/-SD) values with respect to a retinal reference plane were 45.2 +/- 103 microm in healthy subjects and -65.2 +/- 105 microm in glaucoma patients, which were significantly different (P < 0.001). The differences for mean slope and for mean height between the healthy subjects and the glaucoma patients were greatest inferiorly. The diagnostic precision, sensitivity, and specificity of mean slope were 83%, 85%, and 80%, respectively. The diagnostic precision, sensitivity, and specificity of mean height were 75%, 69%, and 83%, respectively. CONCLUSIONS: Mean peripapillary slope of the nerve fiber layer surface can be used to discriminate between healthy subjects and glaucoma patients with clinically useful diagnostic precision. This parameter is independent of a retinal reference plane and may be particularly useful to detect progressive glaucoma damage.

Acquired pits of the optic nerve in glaucoma: prevalence and associated visual field loss.

PURPOSE: This study was performed to determine the prevalence of acquired pits of the optic nerve (APON) in glaucoma and the characteristics of associated visual field loss. SUBJECTS AND METHODS: Stereoscopic optic disc photos from 235 patients with a low-tension variety of primary open-angle glaucoma (LTG) and 251 primary open-angle glaucoma patients with intraocular pressures higher than 21 mmHg (HTG) were examined for the presence of typical APON as defined by a standard photograph (Fig 1). Associated visual field loss was evaluated with automated perimetry and patterns of field loss were identified. RESULTS: Out of a total of 486 patients, 63 APON were found in 51 eyes of 44 glaucoma patients. APONs were more prevalent in women. Acquired pits were more frequently (p<0.001) located inferiorly (70%) than superiorly (30%). There was a significantly higher prevalence (p<0.001) of APON in LTG patients (12.7%) than in HTG patients (5.6%). Dense visual field defects within five degrees of fixation occurred in 96% of patients with APON. CONCLUSION: Acquired pits of the optic nerve are more often located at the inferior optic disc than at the superior disc and are nearly always associated with visual field loss close to fixation. In a glaucoma referral center setting, APON are more prevalent in lower pressure glaucoma than in higher pressure glaucoma.

Increasing peripapillary atrophy is associated with progressive glaucoma.

OBJECTIVE: This study aimed to determine the incidence and degree of progression of peripapillary atrophy in progressive and nonprogressive glaucoma. STUDY DESIGN: A retrospective cohort study. PARTICIPANTS: A total of 75 eyes of 75 patients were examined. MAIN OUTCOME MEASURES: Qualitative assessment of optic disc, peripapillary atrophy,and visual field change was performed by three experienced, masked, independent observers. METHODS: Rim-disc area ratio and peripapillary atrophy-disc area ratio were measured at baseline and follow-up with computer-aided planimetry. RESULTS: Among 75 eyes studied with an average duration of follow-up of 8 years (range, 4-19 years), 33 (44%) showed progressive optic disc damage. Twenty-one (64%) of 33 eyes with progressive disc damage showed peripapillary atrophy progression, and 7 (17%) of 42 eyes without progressive disc damage showed peripapillary atrophy progression; this difference was significant (P < 0.01). In groups with and without peripapillary atrophy, no statistically significant differences were found for mean intraocular pressure, baseline rim-disc area ratio, or baseline peripapillary atrophy-disc area ratio. However, optic disc progression and visual field progression were statistically more frequent in the group with peripapillary atrophy progression (75% and 54%, respectively) than in the group without peripapillary atrophy progression (26% and 11%, respectively) (P < 0.01). There was a statistically significant correlation between measurements of peripapillary atrophy area increase and disc rim loss over time (r = -0.35, P = 0.002). CONCLUSION: Progression of peripapillary atrophy is associated with progressive optic disc damage and progressive visual field loss in glaucoma and may be used as a marker for progressive glaucomatous damage.