Sjögren's syndrome and polymyositis or dermatomyositis.

Of four patients with Sjögren's syndrome, three had polymyositis and one had dermatomyositis. In all, deposition of IgG, IgA, IgM, and C3 was observed in muscle by immunofluorescent techniques. Serologic studies revealed elevated levels of serum IgG and IgM, rheumatoid factor, and antinuclear antibody with specificity for SS-A and SS-B antigens. In muscle there was a mononuclear cell infiltrate with plasma cell predominance around small vessels and capillaries. Ultrastructural changes in the vessels included reduplication of the basement membrane, endothelial thickening, and numerous tubuloreticular and dense inclusions. In two patients, electrondense deposits were noted in the microvasculature. This combination of immunoglobulin deposition in muscle, prominent microvascular changes, and characteristic serology suggests that the myositis in Sjögren's syndrome may result from small-vessel injury by autoantibodies or circulating immune complexes.

Cardiac complications of Wegener granulomatosis: a case report of complete heart block and review of the literature.

Wegener granulomatosis is a necrotizing vasculitis whose target organs are classically the upper and lower respiratory tracts and the kidneys. There has been other end-organ involvement documentation, emphasizing the disseminated nature of this disease, but the literature concerning cardiac involvement is limited. The few case reports and general reviews show that the two most common histologic cardiac manifestations are pericarditis and coronary arteritis, each occurring in 50% of the reported cases. The most frequent clinical manifestation is cardiac arrhythmias that are manifested as supraventricular tachyarrhythmias. We report an unusual cardiac manifestation, a case of complete heart block, occurring during the active stage of Wegener granulomatosis. The problem this case presented and the management are reported. The literature dealing with the cardiac involvement in Wegener granulomatosis is reviewed, and the specific histopathologic findings and the pathophysiologic mechanisms of this involvement are discussed.

Pulmonary immune complex deposition in Wegener's granulomatosis.

Two male patients with pulmonary manifestations of Wegener's granulomatosis are presented. One had an elevated rheumatoid factor, and both had elevated levels of immunoglobulin E. Both demonstrated characteristic necrotizing granulomatous lesions on light microscopy of lung tissue. Immunohistologic analysis of lung tissue demonstrated a granular deposition of immunoglobulin G and complement. Raji cell assay of sera demonstrated elevated levels of circulating immune complexes in the sera of the one patient tested prior to any therapy. These findings support the hypothesis that immune complex deposition contributes to the pathogenesis of Wegener's granulomatosis.

Serologic studies in patients with keratoconjunctivitis sicca.

Thirty-two patients with keratoconjunctivitis sicca (KCS) were screened for the presence of antinuclear antibodies, rheumatoid factor, and autoantibodies associated with Sjögren's syndrome (designated SS-A and SS-B). None of these patients had or were found to have clinical evidence of connective-tissue disease. The conditions of 19 (59%) patients were antinuclear-antibody-positive and 18 (56%) were rheumatoid-factor-positive. We found SS-A and/or SS-B autoantibodies in ten (31%) patients. There seems to be a high incidence of serologic abnormalities in patients with KCS, even when those patients with connective-tissue disease are excluded. Serologic testing seems to be a useful adjunct in the early diagnosis of primary Sjögren's syndrome. The presence of SS-A and SS-B autoantibodies correlated well with the clinical diagnosis of Sjögren's syndrome and seemed to identify the conditions of patients who may have a higher incidence of systemic complications with KCS.

The incidence of Sjögren's sicca complex in a population of patients with keratoconjunctivitis sicca.

A prospective study was performed on patients who came to a university ophthalmology outpatient clinic with the complaint of dry eyes and who were documented to have keratoconjunctivitis sicca (KCS). Of 45 patients studied 1 had primary amyloidosis, and another 10 had KCS with a previously diagnosed connective tissue disease. Of the remaining 34 patients, 19 had KCS alone without xerostomia, and the remaining 15 had KCS in association with subjective or objective xerostomia. Eight of these 15 patients were subsequently shown to have clinical Sjögren's syndrome--sicca complex, and for the majority, serologic and biopsy findings supported this diagnosis.