Discrete arcs of increased fundus autofluorescence in retinal dystrophies and functional correlate on microperimetry.

PURPOSE: To describe the occurrence of discrete arcs of increased fundus autofluorescence (FAF) associated with various retinal dystrophies and to assess their functional significance by fundus-controlled microperimetry. METHODS: Seven patients, three with pigmented paravenous retinochoroidal atrophy (PPRCA), one with sector retinitis pigmentosa (RP), one with typical RP, and two with macular dystrophy were assessed by retinal imaging including FAF imaging. Serial images were obtained within a review period of 6 and 10 years in a patient with PPRCA and macular dystrophy, respectively. Fundus-controlled microperimetry was performed in eight eyes of five patients to determine light increment sensitivity. RESULTS: A discrete arched line of increased FAF was observed without obvious correlate on fundus biomicroscopy. The orientation of this line differed from ring shape in RP and macular dystrophy, a semi-circle structure in sector RP to crescent shape with tiplike extensions towards branching retinal veins in PPRCA. Longitudinal investigation revealed slight migration of the arc in PPRCA and peripheral extension of the ring diameter in macular dystrophy. Microperimetry revealed that the arc of increased FAF sharply delineated areas of severely impaired retinal sensitivity. CONCLUSIONS: The findings indicate that arcs of increased FAF in PPRCA and other retinal dystrophies demarcate areas of impaired retinal function and may migrate over time. FAF imaging may therefore reveal the exact extent of neurosensory dysfunction that may exceed the dimensions anticipated by conventional examinations.

[Validity of anterior chamber depth measurements for the evaluation of accommodation after implantation of an accommodative Humanoptics 1CU intraocular lens]. / Validität der Vorderkammertiefenmessung zur Akkommodationsevaluierung nach Implantation einer akkommodativen Intraokularlinse (Modell Humanoptics 1CU).

BACKGROUND: Changes in the anterior chamber depth (ACD) after pilocarpin application are generally regarded as reflecting an accommodative effect of accommodative intraocular lenses (IOLs) METHODS: We implanted Humanoptics 1CU accommodative IOLs into 25 eyes of 20 patients aged 53.2+/-14.7 years (range: 30-83 years). In seven of these patients, we were able to measure anterior chamber depth changes after the application of pilocarpine (2%) eye drops 3 months postoperatively using a Zeiss IOLMaster, an Orbscan II topography system, as well as by ultrasound biomicroscopy (20 MHz-US-head). RESULTS: Uncorrected near acuity was on average 0.4+/-0.23 (0.1-0.8) and uncorrected distance acuity was 0.76+/-0.23 (0.3-1). Before pilocarpine, ACD was 3.19+/-2.11 mm (IOL-Master), 3.35+/-2.22 mm (Orbscan II) and 3.35+/-2.21 mm (US-biomicroscopy) ( P=0.96). After pilocarpine, ACD was 2.61+/-1.71 mm (IOL-Master), 2.63+/-1.78 mm (Orbscan II) and 3.15+/-2.08 mm (US-biomicroscopy) ( P=0.002). The average individual ACD change before/after pilocarpine was 0.83+/-0.25 mm with the IOL-Master, 1.04+/-0.39 mm with the Orbscan II System and 0.28+/-0.14 with US-biomicroscopy ( P=0.0004). DISCUSSION: The 1CU Humanoptics accommodative IOL presented with potential accommodative capabilities in clinical evaluation. Drug induced accommodation by pilocarpine does not reflect real accommodative effects. The differences in ACD measurements between three different methods were statistically significant. Evaluation methods for accommodative IOLs should be carefully analysed and further development of objective means for evaluation is needed.

Cardiac tamponade: a life-threatening complication of Still's disease.

Pericarditis, a common feature of adult-onset and juvenile-onset Still's disease, is rarely complicated by cardiac tamponade. We report one patient with juvenile-onset and another with adult-onset Still's disease presenting with pericardial tamponade. On the basis of our experience and analysis of literature, we suggest early identification of pericarditis and the life-threatening complication of tamponade, with institution of aggressive intervention with corticosteroids and, if necessary, pericardiocentesis and pericardial window formation. These cases remind physicians about the need to include Still's disease in the differential diagnosis of pericarditis and tamponade and to not neglect the potential of severe systemic inflammation to lead to fatal complications in this group of patients.

Early radiographic joint space narrowing and erosion and later malalignment in rheumatoid arthritis: a longitudinal analysis.

OBJECTIVE: To analyze the time course of development of radiographic erosion, joint space narrowing, and malalignment in a longitudinal study of radiographs of the hands and wrists of 58 patients with rheumatoid arthritis (RA) taken over 18 years. METHODS: Among 210 consecutive patients in a reported cohort, 58 had at least one prior available radiograph 2-18 years earlier. A total of 141 hand and wrist radiographs in the 58 patients were scored for joint space narrowing, erosion, and malalignment in individual joints. RESULTS: Of the 58 patients studied, all developed joint space narrowing, 56 (96.5%) developed erosions, while only 24 (41.4%) developed malalignment. In radiographs of 22 patient studied within the first 5 years of disease, joint space narrowing was seen in 17 (77.3%), erosion in 16 (72.7%), and malalignment in only 3 (13.6%). Radiographic progression was seen in all 36 patients in whom the interval between radiographs was longer than 2 years. CONCLUSION: Joint space narrowing and erosion are seen in hand radiographs of most patients with RA seen in treatment centers within the first 5 years of disease. By contrast, malalignment develops in fewer patients, and generally only after 5 years of disease.

Quantitative analysis of hand radiographs in rheumatoid arthritis: time course of radiographic changes, relation to joint examination measures, and comparison of different scoring methods.

Quantitative studies of hand radiographs in patients with rheumatoid arthritis (RA) indicate that radiographic joint space narrowing and erosion are seen in more than 67% of patients within the 1st 2 years of disease, and progresses most rapidly during the 1st 5 years of disease, according to currently used scoring methods. Radiographic malalignment is rarely seen until after 5 years of disease. In cross sectional studies, correlations of radiographic scores with physical examination scores are minimally significant for joint tenderness, modestly significant for joint swelling, and highly significant for joint deformity and limited motion. In cross sectional studies, 3 quantitative methods, the Steinbrocker radiographic stage, modified Sharp method, and Larsen method, are highly significantly correlated and yield similar results in comparisons with other clinical measures.

Joint counts and physical measures.

The joint examination is the major source of information to determine clinical status of rheumatoid arthritis in the clinic or in a clinical trial. Quantitative methods may evaluate tenderness, pain on motion, swelling, deformity, and limitation of motion. Use of self-report, simplified, reduced, and binary (normal/abnormal) evaluations are presented in this article with emphasis on using binary evaluations in clinical settings. The uses of grip strength, button test, and walk time are also discussed.

Rapid exacerbation of scleroderma in a patient treated with interleukin 2 and lymphokine activated killer cells for renal cell carcinoma.

A patient with longstanding scleroderma with myositis was treated with interleukin 2 (IL-2) and lymphokine activated killer (LAK) cells for locally metastatic renal cell carcinoma. A rapid progression of truncal skin thickening and muscle weakness occurred within weeks of the initial infusion. Studies using supernatants from peripheral T lymphocytes of patients with scleroderma have shown increased levels of IL-2, IL-2 receptor, IL-4 and B cell growth factors, indications of activation of immune mechanisms. The rapid progression of our patient's illness during immunotherapy suggests a primary role for IL-2 and LAK cells in this disorder. Patients with scleroderma who receive IL-2 and LAK cells should be monitored prospectively for exacerbation of their illness.

Reduced joint counts in controlled clinical trials in rheumatoid arthritis.

OBJECTIVE: To determine if quantitative assessment of a reduced number of joints provides information equivalent to that obtained by the traditional 60-joint evaluation in detecting changes in patients participating in clinical trials of rheumatoid arthritis (RA). METHODS: The changes in quantitative joint scores of patients from 3 previously reported clinical trials were compiled and compared with changes in quantitative scores derived using a reduced number of joints. Effect sizes were calculated (mean change in joint score/standard deviation of joint score) and compared for the different joint indices. RESULTS: The effect sizes of the joint scores derived using a reduced number of joints were similar to those of the original 60-joint score. The reduced joint count scores revealed significant changes for clinical trials involving as few as 15 patients. CONCLUSION: Reduced joint count scores may be used to evaluate the results of clinical trials without decreasing the ability to detect change over time. Quantitative assessment of a reduced number of joints may also facilitate assessment of responses to treatment in the routine care of patients with RA.

The use of the disease activity score in the analysis of clinical trials in rheumatoid arthritis.

OBJECTIVE: To ascertain how well the disease activity score discriminates drug from placebo treated patients. METHODS: Three placebo controlled trials in rheumatoid arthritis (RA) were reanalyzed using the disease activity score: DAS = 0.53938 x SQRT (Ritchie index) + 0.06465 x (# swollen joints) + 0.330 x 1n (erythrocyte sedimentation rate) + 0.224. RESULTS: Patient groups receiving methotrexate, high dose D-penicillamine and sulfasalazine had the statistically greatest improvement vs placebo treated groups; patient groups receiving gold sodium thiomalate (GSTM) and low dose D-penicillamine also showed statistically significant improvement versus placebo treated groups. Patients receiving sulfasalazine or GSTM were deemed to benefit compared to placebo treated patients in this analysis, unlike the results presented in the initial analyses of this trial. CONCLUSION: The disease activity score is a simple and effective measure of inflammation that can discriminate between active drug and placebo treated patient groups. Use of this composite measure may improve analysis of clinical trials and also be applicable to clinical care.

Polyarticular pseudosepsis in rheumatoid arthritis.

Three patients with longstanding seropositive rheumatoid arthritis had fever and marked synovial fluid leukocytosis in multiple joints. No infectious agents were found in synovial fluid or blood, and the patients recovered uneventfully without prolonged antibiotic therapy. Acute, severe exacerbations of synovitis in patients with rheumatoid arthritis may simulate a septic process and involve multiple joints.

Problems with the Steinbrocker staging system for radiographic assessment of the rheumatoid hand and wrist.

Hand and wrist radiographs of 202 patients with rheumatoid arthritis were graded with the classical Steinbrocker Staging System, and were also assigned quantitative scores for joint space narrowing, erosion, and malalignment according to a detailed scoring method. Steinbrocker Stage scores were correlated significantly with total detailed scores (r = 0.60 to 0.66, P less than 0.001). However, the ranges of detailed total and subtotal scores among the various Steinbrocker Stage scores were broad, with considerable overlap. Practical problems in applying the Steinbrocker staging method were identified in 26.5% of the hands graded with the Steinbrocker method. Additionally, analysis of scoring data showed discrepancies between the scoring methods in 25.2% of the hands examined; one-half of these discrepancies were felt to result from the global or overall approach of the Steinbrocker method in contrast to individual joint analysis. Asymmetry in assigned stages between right and left hands was found in 15.8% of patients. The detailed scoring method, although more time-consuming, appears superior to the Steinbrocker Stage for quantitative assessment of patient radiographic status in rheumatoid arthritis.

A simplified twenty-eight-joint quantitative articular index in rheumatoid arthritis.

We describe a joint index that includes only 28 joints: 10 proximal interphalangeal joints of the fingers, 10 metacarpophalangeal joints, and the wrists, elbows, shoulders, and knees. These joints are evaluated for swelling, tenderness, and limited motion, with the findings scored as abnormal or normal. The 28-joint index is considerably easier to use than traditional methods, and appears to yield as much information in terms of correlation with other measures of clinical status, including hand radiograph scores, American Rheumatism Association functional class, grip strength, walking time, and activities of daily living questionnaire scores (r = 0.25-0.53, P less than 0.001). Simplified joint counts might facilitate acquisition of quantitative articular data in research and clinical rheumatologic settings.

Evidence of significant radiographic damage in rheumatoid arthritis within the first 2 years of disease.

Quantitative radiographic scores for joint space narrowing erosion, and malalignment in the hands and wrists of 200 patients with rheumatoid arthritis (RA) were significantly correlated with duration of disease. Nonetheless, most of the 42 patients who reported disease for less than 2 years had evidence of radiographic damage, including 35 with joint space narrowing, 28 with erosions but only one with malalignment. Significant radiographic damage in the absence of malalignment may be seen in many patients within the first 2 years after presentation of RA.