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Progressive systemic (sclerosis) is one of the most enigmatic of the rheumatic diseases. It is a connective tissue disorder of unknown etiology characterized by fibrosis in skin and internal organs. Although similar lesions are found with increased prevalence in workers exposed to coal, gold, silica, and polyvinyl chloride, most patients have had no known predisposing factors. Select reports of a familial occurrence of PSS have been observed but a definitive genetic basis is lacking and no clear associations with the major histocompatability complex have been demonstrated. Moreover, although a variety of immunologic abnormalities in patients with PSS have been reported, they are generally diffuse and non-diagnostic. Such abnormalities include defects in cell mediated immunity, increases in sera immunoglobulins, antinuclear antibodies, and cryoglobulins. In contrast to these non-specific findings, there appears to be significant evidence of a relationship between cell mediated immunity to collagen and appearance of scleroderma. For example, peripheral blood lymphocytes in patients with scleroderma undergo lymphocyte transformation when cultured with specific collagen preparations. The pathology of skin and internal organs in PSS generally reflects both collagen deposition and small vessel occlusion. All organ systems may be involved but mortality significantly increases with involvement of heart, kidney, or lung. Unfortunately, at present a reliable experimental model of PSS has not been found although similar immunopathology can be induced in homologous disease of rats and in chronic graft vs host disease of humans.

Rhesus monkey infants fed a marginally zinc-deficient diet (4 ppm) from conception through 12 mo of postnatal life were monitored for changes in hematological, biochemical, and immunological parameters. These zinc-deprived (ZD) infants were compared to control infants whose mothers were fed a zinc-replete (100 ppm) diet either ad libitum (AL) or pair-fed (PF) throughout gestation and lactation. Blast transformation of peripheral blood lymphocytes to phytohemagglutinin (PHA-P), concanavalin A (Con A), and pokeweed mitogen (PWM), was dramatically depressed in the zinc-deficient (ZD) group. Similarly, ZD infants had reduced polymorphonuclear leukocyte function as measured by chemotaxis to endotoxin-activated plasma and phagocytosis of Candida albicans. Levels of serum IgM were significantly altered in zinc-deficient infants compared to controls. Serum concentrations of IgG and IgA were similar in zinc-deficient and control infants. ZD infants also manifested a hypochromic microcytic anemia at one month of age, reduced activity of the zinc metalloenzyme alkaline phosphatase, and lower activity of SGPT.

Serologic and correlational testing was performed in a series of 65 patients diagnosed as having juvenile arthritis (JA) and in 21 age matched controls to detect the presence of antinuclear antibodies (ANA), antibodies to ssDNA, IgM and IgG rheumatoid factor (RF), immune complexes (IC) and antibodies to bovine type I and human type II collagen. ANA were found in 51% of the JA patients; the highest incidence (75%) was noted in the pauciarticular onset disease group. Low levels of anti-ssDNA antibodies were detected in 22% of the patients, all of whom had active disease. IgM RF was detected in 35% of the JA patients but only 6% of patients had IgG RF. Similarly, about one fourth of the JA patients had IC detected by the Clq assay. Antibodies to bovine type I and human type II collagen were noted in about 12% of the JA patients.