Objective. To determine the frequency, clinical associations, and any major histocompatibility complex correlations of antifibrillarin antibodies in patients with systemic sclerosis (SSc).Methods. Antifibrillarin antibodies were determined by indirect immunofluorescence, immunoblotting, and immunoprecipitation, and HLA class I1 alleles by DNA oligotyping, in a large cohort of SSc patients.Results. Antifibrillarin was found in 8% of 335 SSc sera and was significantly more common in blacks (16%) than whites (S%), in males (33%) than females (14%), and in patients with cardiac, renal, or gut involvement. The HLA class I1 haplotype DRBZ *Z302, DQBZ*0604 was found significantly more frequently in SSc patients with antifibrillarin compared with racematched normal controls and 260 SSc patients without antifibrillarin. In addition, 1 or more of the HLA-DQB1

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 dis- orders. 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.

Interstitial pulmonary fibrosis is a common manifestation of systemic sclerosis (SSc) and is a pathologic feature shared by a variety of other diseases. In these other disease processes, the glycoprotein fibronectin (FN) has been shown to be released by the alveolar macrophage, and is thus implicated in the development of fibrosis. We therefore studied the release of FN by alveolar macrophages obtained by bronchoalveolar lavage of 17 patients with SSc and 14 controls. We found that SSc alveolar macrophages released significantly more FN than did those of controls. Furthermore, the level of FN correlated positively with the level of inflammation determined by cellular analysis of lavage fluid and negatively with carbon monoxide diffusing capacity. FN may therefore play a role in the development of lung fibrosis in SSc and may be a marker of alveolitis.Systemic sclerosis (SSc; scleroderma) is a disease characterized by an abnormal increase in fibrous tissue in many organs. Pulmonary fibrosis is a common Submitted for publication September 21, 1988; accepted in revised form December 22, 1988. feature of scleroderma, reported in 70-100% of cases in autopsy studies (1,2), and its relative importance in the clinical course of patients with this illness is increasing. Since the advent of improved treatment for renovascular hypertension and heart disease, which involve the 2 organ systems previously associated with the highest mortality in SSc (3), pulmonary fibrosis is emerging as the major cause of morbidity and mortality among SSc patients.Interstitial pulmonary fibrosis is a pathologic feature seen in both SSc and idiopathic pulmonary fibrosis (IPF). Bronchoalveolar lavage (BAL) has become an accepted tool for investigating changes in the lower respiratory tract in patients with these, as well as other pulmonary diseases (4). Numerous investigators have used BAL to characterize the cellular composition of the alveolar lining in SSc, and most have found changes similar to those in IPF (5-13). Alveolar macrophages of patients with IPF have been found to release factors that may directly influence the development of fibrosis, through recruitment and proliferation of fibroblasts (14-16). One such factor is the glycoprotein fibronectin (FN). We therefore undertook an investigation of pulmonary involvement in SSc, using BAL in nonsmoking patients, to evaluate the cellular content of the lavage fluid and to measure FN release by the alveolar macrophage. Correlations between the level of FN in alveolar macrophage culture supernatants and BAL cellular composition were studied, as were correlations with clinical measures of lung function. PATIENTS AND METHODSPatients and controls. The 17 SSc patients (10 women and 7 men) had a mean age of 45.3 years (range 24.9-62.5).