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Biomedical subjects

F E Harper

Publications and source records attributed to F E Harper.

7 recordsLinked to original sources

Prevalence of scleroderma spectrum disorders in the general population of South Carolina.

The prevalence of scleroderma spectrum disorders (including systemic sclerosis [SSc] meeting the American Rheumatism Association criteria and the less typical disorders meeting only our study criteria) was determined in a random sample of 6,998 subjects from the general population of South Carolina. The results suggest that the prevalence of these disorders may range from 67 to 265 per 100,000, which is 4.9 to 19.2 times higher than previously reported for definite SSc. The ratio of nondefinite cases to definite cases of SSc (those meeting American Rheumatism Association criteria) was 2.5. Most of the nondefinite cases were unrecognized prior to our study, which suggests the need for improved early diagnosis of scleroderma spectrum disorders. Brief histories of the 7 patients with scleroderma spectrum disorders whose cases formed the basis for our calculation of prevalence rates are included in this report.

Adult↗

Nailfold biopsy in scleroderma and related disorders. Correlation of histologic, capillaroscopic, and clinical data.

Although nailfold capillary abnormalities associated with connective tissue disease (CTD) have been studied by direct in vivo microscopy, little is known of the underlying histology and morphology of this tissue. This report summarizes light microscopic study of glycolmethacrylate embedded nailfold biopsies from 13 CTD patients (9 scleroderma, 2 CREST, 2 undifferentiated CTD), 2 subjects with Raynaud's phenomenon alone, and 9 normal volunteers of similar age and sex distribution. The most striking and consistent finding was the presence of globular, eosinophilic, PAS-positive deposits in the cuticles of 14 of 15 patients and none of the controls. This material, identified by immunofluorescent staining as serum protein exudates, was associated with pronounced parakeratosis and elevated epithelial mitotic activity. Capillary ectasia with thinning of the basement membrane was often present in CTD biopsies. Occasional signs of endothelial swelling and proliferation were encountered in both populations. Inflammatory changes were rarely seen. In quantitative comparison with control tissues, the superficial dermis from CTD patients contained significantly fewer capillaries, cutaneous nerve bundles, and interstitial fibroblasts per unit area and fewer papillary capillaries per unit of epidermal length. Measures of capillary density in sectioned tissue correlated well with the results of in vivo microscopic examination.

Adult↗

Antibodies to collagen in scleroderma.

Using the enzyme-linked immunosorbent assay (ELISA), we detected antibodies to interstitial (type I) and basement membrane (type IV) collagens in the sera of patients with scleroderma (systemic sclerosis). Antibodies against type IV collagen were found in significant levels in these patients and correlated with the presence of abnormal pulmonary diffusion capacity. Levels of antibodies to type I collagens also correlated significantly with pulmonary diffusion capacity. Absorption of sera with type I or type IV collagens before analysis in the ELISA eliminated reactivity in an antigen-specific pattern, indicating that these antibodies reacted with determinants specific for either type I or type IV collagens. The removal of immune complexes by ultracentrifugation had no effect on serum antibody levels. Autoantibodies to basement membrane and interstitial collagens may participate in the pathogenesis of scleroderma.

Adolescent↗

A prospective study of Raynaud phenomenon and early connective tissue disease. A five-year report.

To define features of patients with Raynaud phenomenon that predict the evolution of connective tissue disorders, a prospective study was initiated. Patients with history or physical evidence of Raynaud phenomenon without causal or associated disorders underwent initial multisystem evaluation. Scleroderma (systemic sclerosis) of less than five years' duration was included for comparison. Patients were classified as having Raynaud phenomenon "only," undifferentiated connective tissue syndrome or scleroderma. Nailfold capillary microscopy was performed, and patterns were scored blindly from coded photographs. Of 91 patients with Raynaud phenomenon entered (Raynaud phenomenon only, n = 49; undifferentiated connective tissue syndrome, n = 22; scleroderma, n = 20), abnormal "scleroderma pattern" capillaries were noted in seven of 49, 19 of 22 and 19 of 20, respectively (p less than 0.005). Of 39 patients with Raynaud phenomenon only followed (mean duration, 23.7 months) undifferentiated connective tissue syndrome developed in three. Of 17 patients with undifferentiated connective tissue syndrome followed (mean duration, 27.6 months), six patients had transitions (four scleroderma; one scleroderma-systemic lupus erythematosus overlap; one SLE). Nailfold capillary abnormalities best identified transitional patients in both groups (eight of nine) and were more sensitive than ANA (six of nine), presence of digital ulcers (four of nine) or decreased esophageal motility (two of nine). This prospective study documents a useful role for capillary examinations in evaluating Raynaud phenomenon. Abnormal capillaries indicate an increased risk for connective tissue disease; normal capillaries favor idiopathic Raynaud phenomenon.

Capillaries↗

Microvascular abnormalities as possible predictors of disease subsets in Raynaud phenomenon and early connective tissue disease.

In vivo capillary examination was performed on 80 patients included in a prospective study of Raynaud phenomenon (RP): 40 RP only (RPO), 20 undifferentiated connective tissue disease (UCTD) and 20 systemic scleroderma (SD) of less than 5 years' duration. On initial examination, SD-pattern capillary abnormalities were found in 7/40 patients with RP alone, 18/20 with UCTD and 19/20 with SD. On follow-up a change of diagnosis into a definite connective tissue disease occurred in 4 patients with UCTD; one patient with RP alone died with symptoms of myositis and fulminant pulmonary vasculitis. All five showed SD-pattern capillary abnormalities of the "active" type on initial examination. A significant correlation between the "slow" capillary pattern and the presence of anticentromere antibodies was also observed. Only one patient of 25 RPO with normal capillaries available for follow-up showed any change in clinical status.

Antibodies, Antinuclear↗