Immunofluorescence studies in progressive systemic sclerosis (scleroderma) and mixed connective tissue disease

Abstract

SUMMARY Immunofluorescence (IF) investigations of the skin were performed in thirty patients with progressive systemic sclerosis (scleroderma) and eight patients with mixed connective tissue disease (MCTD). The results show that speckled epidermal nuclear immunoglobulin deposition occurs not only in MCTD but also in true scleroderma. Granular IgM deposition at the dermo‐epidermal junction of light‐exposed skin was detected in both groups of patients, but six of eight MCTD patients also showed a granular IgM band in non‐exposed skin. Antinuclear antibodies (ANA) were demonstrated in the sera of 96% and 100% of patients with scleroderma and MCTD respectively. The pattern of nuclear IF staining in scleroderma included dense fine speckles, large coarse speckles, threads, nucleolar and centromere staining. In MCTD, by contrast, the ANA staining pattern consisted of threads. The significance of ANA titres and immunological specificities for the in vivo reaction of serum ANA with epidermal nuclear antigens is discussed.

Immunofluorescence in dermatology

“…172,194 Systemic sclerosis DIF in systemic sclerosis is either negative or nonspecific. 148,189,[194][195][196][197] Patients with positive DIF findings are likely to have overlapping features with SLE and dermatomyositis. 196 One study reported granular deposition of IgM along the DEJ in sun-exposed skin in 60% of the patients.…”

“…196 One study reported granular deposition of IgM along the DEJ in sun-exposed skin in 60% of the patients. 194 Approximately 15% had perivascular deposits. These are usually seen in patients who have associated vasculitis.…”

“…Epidermal nuclear fluorescence similar to MCTD and SLE (see Table VI) may occasionally be seen. 172,190,194,198 DIF is of little or no value in the diagnosis of systemic sclerosis.…”

Human autoantibodies: probes for nucleolus structure and function

“…Antibodies reactive with nucleolar components are part of the spectrum of autoantibodies present in autoimmune sera, particularly from patients with scleroderma, as has been reported by many investigators (Ritchie 1970;Pinnas et al 1973 ;Tan et al 1980;Bernstein et al 1983;Reimer et al 1983). Previously, antinucleolar autoantibodies were used in immunofluorescence microscopy by several investigators to gain insight into the structural organization of the nucleolus (Ritchie 1969 ;Ringertz et al 1973 ;Hernandez-Verdun et al 1979).…”

In vitro complement activation by antinuclear antibodies in the epidermis from patients with systemic sclerosis

“…In a recent study using DIF, we reported on the phenomenon of ANA in skin biopsies from scleroderma patients without the n-RNP specificity of serum ANA (Reimer et al, 1983). Among these ANA were those giving either a large coarse, a dense fine, a thready or a nucleolar staining pattern by indirect immunofiuorescence on KB-cells as the substrate.…”