Eija Johansson

Localized heat urticaria associated with a decrease in serum complement factor B (C3 proactivator)

A case of localized heat urticaria is reported in a 51‐year‐old woman who within a few minutes of contact with warm water developed erythema and swelling sharply localized to the heated area. After a hot bath urticarial lesions appeared over large areas of her body, accompanied by a feeling of weakness, but no other systemic symptoms. After challenge with heat by immersing her left arm in water heated to 42°C, a rapid decrease of her serum complement level of factor B was demonstrated, suggesting that activation of an alternative complement pathway plays a role in this form of urticaria. Biopsies for immunofluorescent study of complement and immunoglobulins were negative at 30 and 180 min after heat challenge. The dermal fibres and endothelial cells of dermal vessels were capable, in vitro, of complement binding before and after exposure to heat.

Polyclhonal B-cell activation and increased lymphocyte helper-suppressor ratios in discoid lupus erythematosus

We studied polyclonal B‐cell activation in twenty‐six patients with discoid lupus erythematosus (DLE). Spontaneous plaque‐forming cells of the IgA class (IgA‐SPFC) as determined by a reverse haemolytic plaque assay were significantly more common in patients with DLE than in fifty control subjects. The patients showed a positive correlation between IgA‐SPFC and OKT4/8 ratios and also had a significantly higher mean OKT4/8 ratio. The two groups did not differ with regard to cells producing IgG or IgM or cells with OKT3, OKT4, OKT8 or OKM1 markers. None of the three patients with DLE who had IgA‐SPFC values which were above the mean (+2s·d.) for the control subjects had positive tests for ANA or low serum C3 or C4, but two of the three also had increased IgG‐SPFC values. The results indicate that polyclonal B‐cell activation occurs in a small proportion of patients with DLE.

Assessment of class-specific antibodies against denatured DNA in patients with systemic lupus erythematosus

In this retrospective study 103 serum samples from 16 females with systemic lupus erythematosus (SLE), obtained during a mean follow-up time of 2 years, were investigated for the presence of anti-denatured [single-stranded (ss)] DNA antibodies of the IgG, IgM, and IgA classes. The anti-ssDNA antibodies were determined by an enzyme-linked immunosorbent assay (ELISA), and the results were expressed in three ways: as units derived from a single serum dilution and as two parameters,E andA, calculated from the dose-response curve,E being an estimate of the effective amount of antibodies andA a function of the reaction constant between the antigen and the antibody. The simultaneous occurrence of anti-ssDNA antibodies of all three immunoglobulin classes was seen most often in the patients with the shortest duration of the disease. Clinically active disease was found to correlate with high reaction constants of the IgA anti-ssDNA antibodies. There was also an association between the IgA anti-ssDNA antibody levels and the presence of nephritis. Great fluctuations in the amounts of effective antibodies of the IgG class were seen in seven patients, in six of whom changes in the disease activity also were seen. Changes in the disease activity were unaccompanied by fluctuations in the IgG anti-ssDNA levels in four patients; two of these patients were positive for antibodies against extractable nuclear antigens. We conclude that it is of value to express the results of the anti-ssDNA ELISA as a function of the dose-response curve when monitoring patients with SLE and that immunoglobulin class-specific determinations of anti-ssDNA antibodies may provide information about the disease activity in many patients with SLE.

Langerhans Cells in SLE Skin: A Role in Lymphocyte Migration and Activation in Situ

Langerhans cells in SLE skin and mucosa were studied by using monoclonal anti-T6 and anti-Ia antibodies and avidin-biotin-peroxidase complex staining. Langerhans cells were present in cell infiltrates of natural SLE skin lesions. In developing early skin lesions induced with test antigen, Langerhans cells were the predominant inflammatory cells, suggesting that migration of Langerhans cells into the inflammatory site in fact precedes lymphocyte migration. These findings agree with the hypothesis of the role of dendritic cells in lymphocyte circulation and antigen presentation under physiological and pathological conditions. Many of the infiltrating local T lymphocytes in both natural and test antigen induced SLE lesions were activated Ia+ cells. This suggests an active immune response in both natural and test antigen induced skin lesions in SLE. The low immunomodulatory T4/T8 ratio in situ in SLE skin lesions might suggest that T8+ cells exert immunosuppressive control on the local inflammation.

The deoxyribonucleic acid (DNA) skin test in systemic lupus erythematosus. 2. Histological findings.

A total of fifty‐five biopsies from fifty‐two intradermal DNA skin tests were studied. The biopsies were taken 6, 8–10,24 or 48 h after the injection of the DNA material. Necrosis of the vessel wall was taken to be the main characteristic of a specific reaction. In forty of the fifty‐two tests the results of the histological evaluation closely matched the clinical results. In five of the fifteen cases with discrepancies, the histological evaluation ruled out clinically false positive test results.

HLA antigens in patients with chronic biologically false-positive seroreactions for syphilis and systemic lupus erythematosus

SummaryHLA antigens were examined in 118 unrelated patients of whom 49 had definite severe systemic lupus erythematosus (SLE). HLA A1 was found in 45% of the 49 SLE patients and in 20% of the controls, which consisted of 900 blood donors. HLA B8 was found in 47% of the patients and in 20% of the controls (relative risk=3.5, P=0.000017), while HLA B7 was found in 22% of the patients and in 24% of the controls.Of the 118 patients 69 had chronic biologically false-positive (CBFP) seroreactions for syphilis. In 32 of these 69 patients definite or probable SLE was diagnosed during the observation time, which varied from 3 to 16 years. HLA B7 was found in 38% of these 32 patients, while HLA B8 was found in 25%. When the occurrence of these antigens was correlated to the length of the observation time it was found that 53% of the patients in whom the disease manifested first after 10 years follow-up were carriers of HLA B7 (P=0.0005) and only 12% had HLA B8 (relative risk=0.5). In patients with a faster development of the disease both of these antigens were found in about the same frequency as in severe SLE patients.In 37 of the 69 patients no definite diagnosis could be established even it 19 of them had clinical and serological manifestations compatible with SLE. HLA B7 was found in 46% and HLA B8 in 14% of these patients. Moreover it was found that 72% of the 18 patients who had only antilipoidal antibodies in their serum were carriers of HLA B7, while 21% of the 19 patients with other circulating auto-antibodies had HLA B7. D-locus antigens were examined in 35 patients. HLA D w3 was found in 54% of the patients and in 24% of the controls. Twelve of the patients with D w3 had severe SLE and nine of them had also HLA B8.

The deoxyribonucleic acid (DNA) skin test in systemic lupus erythematosus. I. Clinical evaluation.

A clinical evaluation of the intradermal DNA‐test was carried out on a series of patients with untreated or with treated definite systemic lupus erythematosus (SLE), or with suspected SLE with or without circulating antinuclear factors. A saline solution of a commercially available DNA‐preparation was used. The course of the DNA reaction was followed for 24–48 h after the injection.

The ribonucleic acid (RNA) skin test in systemic lupus erythematosus and other connective tissue diseases

A series of 65 patients with different autoimmune diseases was examined using different RNA‐solutions for intradermal skin tests. Clinically positive results were obtained most often in patients with mixed connective tissue disease but quite often also in patients with systemic lupus erythematosus and progressive systemic sclerosis or with some symptoms of an autoimmune nature. The histological examination of the biopsies from the test sites revealed that there was no correlation between the clinically positive tests and the histological criteria usually used as a sign of a positive test.