Older-onset Patients Research Articles

Young onset of primary Sjögren's syndrome: clinical and immunological characteristics.

The objective of our study was to determine the clinical and immunological characteristics of primary Sjögren's syndrome (SS) in patients with a young onset of the disease. We included 144 consecutive patients (134 female and 10 male; mean age at onset 53 y; range 20-87 y) visited in our Units. All patients were white and fulfilled four or more of the diagnostic criteria for SS, proposed by the European Community Study Group in 1993. Disease onset was determined on the basis of the appearance of symptoms strongly suggestive of SS. In 13 (9%) patients, disease onset occurred before the age of 35. All were female and the disease onset occurred between 20-34 y (mean, 28 y). When compared with patients with older onset, patients with a young onset of the primary SS presented a higher prevalence of lymphadenopathy (54% vs 6%, P < 0.001), rheumatoid factor (70% vs 39%, P=0.034), anti-Ro/SS-A antibodies (70% vs 28%, P=0.004) and monoclonal immunoglobulins (23% vs 4%, P=0.02) in their sera. From the initial diagnosis of SS, three patients with a young-onset of the primary SS have developed lymphoproliferative disease at the time of the study, compared with one patient of the older-onset group (23% vs 1%, P=0.002). Our study shows several differences between younger and older onset patients, including a higher incidence of lymphomas in the younger, thus conferring to the age at onset of the disease a prognostic value.

The importance of early diagnosis of treatable diabetic retinopathy for the four-year visual outcome in older-onset diabetes mellitus.

The four-year visual outcome was retrospectively studied in patients with older-onset diabetes mellitus and diabetic retinopathy in need of laser treatment. Visual acuity in 53 patients examined by ophthalmologists who referred the patients for an evaluation of retinopathy before laser treatment, was compared to that of 47 patients examined by ophthalmologists who also performed the photocoagulation. The number of eyes that became blind (visual acuity < or = 6/60) during the four-year period was higher (23/90 vs 9/91; p < 0.01) among referred patients, whereas the number of retinal examinations per patient during the three-year period prior to laser treatment did not differ between the two groups. Among referred patients, 13% had not been ophthalmologically examined before the treatment-requiring retinopathy was found. Corresponding figure for those examined at the laser centre was 23%. Severe macular oedema in regularly examined patients was more common among referred patients (9/30 vs 1/32; p < 0.01). The results indicate that screening for diabetic retinopathy in older-onset diabetes was not performed satisfactorily. In addition, laser treatment was delayed in older-onset diabetic patients controlled by ophthalmologists who referred patients for photocoagulation, resulting in an increased incidence of legally blind eyes. The study also stresses the importance of carrying out knowledge of when and how to diagnose early sight-threatening diabetic retinopathy to ophthalmologists referring patients for laser treatment.

Anticipation in hereditary dentatorubral-pallidoluysian atrophy.

Anticipation refers to the progressively earlier onset and increase in disease severity in successive generations. We studied four families with hereditary dentatorubral-pallidoluysian atrophy (DRPLA), a neurodegenerative disease, and anticipation was present in the mode of inheritance. In subsequent generations DRPLA shows an earlier onset and more severe as well as additional symptoms. Older onset patients suffer from cerebellar ataxia with or without dementia, whereas younger onset patients present as progressive myoclonus epilepsy syndrome, which consists of mental retardation, dementia, and cerebellar ataxia as well as epilepsy and myoclonus. Anticipation with paternal transmission was significantly greater than with maternal transmission.

Systemic Lupus Erythematosus

In the present study we have analyzed the prevalence and characteristics of the most relevant clinical and immunologic features in 1,000 patients with SLE. Several differences in the expression of the disease have been observed in relation to the patients' age at onset, sex, and autoantibody serology. The childhood-onset patients more often had malar rashes (55% vs 39%) and nephropathy (28% vs 15%) as presenting manifestations. During the evolution of the disease, these patients had an increased prevalence only of malar rash (79% vs 56%) and a lower prevalence of rheumatoid factor (6% vs 19%). The older-onset patients (age 50 or older) less often showed malar rash (21% vs 42%), arthritis (52% vs 71%), and nephropathy (3% vs 17%) as the first symptom. During the evolution of their disease, these patients had a decreased prevalence of malar rash (33% vs 60%), photosensitivity (29% vs 47%), arthritis (73% vs 85%), nephropathy (22% vs 41%), thrombosis (4% vs 15%), and anti-La antibodies (6% vs 20%), but an increased prevalence of sicca syndrome (33% vs 15%). Males more often had serositis (28% vs 16%) as a first symptom, but they presented with a lower prevalence of arthritis (74% vs 85%) during the evolution of the disease. The presence of ANA, a high titer of anti-dsDNA, rheumatoid factor, anti-ENA, and antiphospholipid antibodies also distinguished additional homogeneous SLE subsets of clinical significance.

The Wisconsin Epidemiologic Study of Diabetic Retinopathy: XIII. Relationship of Serum Cholesterol to Retinopathy and Hard Exudate

Serum total and high-density lipoprotein (HDL) cholesterol were measured in a sample of individuals examined between 1984 and 1986 for the Wisconsin Epidemiologic Study of Diabetic Retinopathy. There was a significant trend for increasing severity of diabetic retinopathy and of retinal hard exudate with increasing cholesterol in insulin-using persons. Cholesterol levels were not related to the severity of either ocular condition in older-onset patients. Highdensity lipoprotein-cholesterol was unrelated to the severity of either lesion. In multiple logistic regression analyses, cholesterol was not a significant factor in describing the severity of retinopathy in any group but was a significant factor in describing the severity of retinal hard exudate. Glycosylated hemoglobin and diastolic blood pressure were significant descriptors of the severity of retinopathy in younger-onset patients in these multivariate analyses. Diastolic blood pressure added significantly to explaining the severity of hard exudate in older-onset insulin users. These data support the current management strategies for diabetes, which include control of level of glycemia, blood pressure, and blood lipids.

Clinical features of systemic lupus erythematosus: differences related to race and age of onset.

We compared the frequency of clinical features of systemic lupus erythematosus (SLE) and determined survival in 113 patients with younger-onset lupus (age less than 55 at clinical diagnosis) and 25 patients with older-onset disease (age greater than or equal to 55 at diagnosis). The most striking difference was in the racial distribution; 59% of the younger patients were black, compared with only 20% of the older-onset patients (P less than 0.001). Major manifestation of lupus (including clinically evident renal disease, central nervous system involvement, cutaneous involvement, and hemocytopenia) occurred with similar frequency in both age groups. Antibodies to DNA were detectable equally often in both groups, but hypocomplementemia was more common in the younger patients. Five-year survival in the younger-onset group (79%) was similar to that of the older-onset group (72%); there was a tendency toward relatively improved survival in patients survival of appropriately matched control populations. Major significant differences in racial distribution included 1) a higher incidence of serositis in older whites and in blacks regardless of age, and 2) more frequent hypocomplementemia in younger patients within both racial groups.

Diabetic Retinopathy in Joslin Clinic Patients with Adult-Onset Diabetes

While only a small proportion of adult-onset diabetic patients develop vision threatening retinopathy, this group makes up a major portion of those diabetics seeking ophthalmologic care. Fifty-three percent of patients having fundus photographs at the W.P. Beetham Eye Unit of the Joslin Clinic developed diabetes at the age of 20 or over, and almost 30% at the age of 40 or over. Twenty-two percent of all patients seen with proliferative retinopathy were in this latter group. Ten of 54 patients with proliferative retinopathy in the ⩾40 onset group were not taking insulin. The older onset patients who developed proliferative retinopathy did so after shorter durations of diabetes and with more visual disability than younger onset patients. Macular edema was more common in adult-onset patients, and its presence in a patient less than 50 years old was usually associated with proliferative or preproliferative retinopathy.