Folke D. Lindström

Peripheral Blood Lymphocyte Cell Surface Markers during the Course of Systemic Lupus Erythematosus

Peripheral blood lymphocytes from 23 patients with active systemic lupus erythematosus (SLE) were serially studied. Changes in bone marrow-derived lymphocytes (B cells), as measured by surface Ig receptors and C3 receptors, and in thymus-derived cells (T cells) measured by rabbit T-cell-specific antiserum and E-binding techniques, were correlated with fluctuations in clinical disease activity and treatment. In normal controls B- and T-cell percentages remained relatively stable, although the situation in SLE was much more labile. A relative and absolute decrease in T lymphocytes and cells bearing a receptor for C3 was found in active lupus. Absolute numbers of cells bearing surface Ig were decreased to a lesser extent, whereas the proportion of these cells was increased. It is postulated that the increase in autoantibody formation and diminished delayed hypersensitivity seen in systemic lupus may be due to a loss of T-lymphocyte function.

Multiple myeloma or benign monoclonal gammopathy? A study of differential diagnostic criteria in 44 cases.

Abstract Plasma immunoglobulin levels, Bence-Jones proteinuria, and bone marrow cytology were examined in 44 patients with benign monoclonal gammopathy (BMG), in order to study differential diagnostic criteria between this state and multiple myeloma. High immunoglobulin concentrations, in the range usually attributed to multiple myeloma, and subnormal concentrations of uninvolved polyclonal (“background”) immunoglobulin were frequently seen in BMG. Of the BMG patients, 40% had Bence-Jones proteinuria defined as a urinary M component, and myeloma-like accumulations of plasma cells were seen in the bone marrow in several instances. We conclude that criteria used to distinguish between myeloma and BMG are not specific and that differences between the two conditions are quantitative rather than qualitative.

Urolithiasis and distal renal tubular acidosis preceding primary Sjögren's syndrome: a retrospective study 5–53 years after the presentation of urolithiasis

Objectives. Distal renal tubular acidosis (dRTA) can be associated with autoimmune diseases such as primary Sjögrens syndrome (SS). Our objective was to study SS‐associated symptoms, autoantibodies and renal histopathology in patients with urolithiasis and dRTA.

Biochemical markers of renal disease in primary Sjögren's syndrome.

Primary Sjögrens syndrome (SS) is characterized by an inflammatory process in the salivary and lacrimal glands, but the kidneys may also be involved. Renal tubular functions were studied in 27 patients with SS, all females, age 37-78. Both SS-patients with and without known distal renal tubular acidosis (dRTA) were included, dRTA was found in 18/27 (67%), impaired urine concentrating ability in 13/27 (48%). Hypocitraturia was identified in 20/27 (74%) and reduced tubular reabsorption of phosphate (TRP%) in 18/27 (67%). Tubular proteinuria (alpha 1-mikroglobulin) was present in 11/24 (46%), and tubular enzymuria (NAG) in 7/24 (29%). Hypocitraturia and/or dRTA were found in all patients with any kind of abnormal renal tubular function test. All except one of the patients with dRTA not treated with sodium bicarbonate had hypocitraturia. We conclude that distal tubular dysfunction was common in our SS-patients, but a concommitant proximal dysfunction was also seen. Determination of urinary citrate represents a valuable test for detection of renal disease in SS.

Serum anti-immunoglobulins in multiple myeloma and benign monoclonal gammopathy

Sera from 111 patients with IgG myeloma (MM) and 76 patients with IgG benign monoclonal gammopathy (BMG) were examined for the presence of anti-IgG antibodies. Forty-nine and five-tenths percent of MM patients were positive as compared to 26.3% of patients with BMG and 28% of age-matched controls (P < 0.005). Anti-IgG antibodies were most easily detected using Rh-positive cells coated with various human incomplete antibodies. Anti-IgG antibodies showed specificity for determinants present on whole IgG and, in some instances, Fc. No clear evidence for anti-idiotypic reactivity with autologous M components could be obtained. In most sera, anti-IgG factors appeared to be present in low-molecular-weight fractions (3.5–5.0S). The apparent increase in anti-Ig factors in MM as opposed to BMG may reflect a basic difference in control mechanisms.

Glomerular Filtration Rate in Primary Sjögren's Syndrome with Renal Disease

Renal disease in Primary Sjögrens syndrome (SS) is often overlooked, because of a paucity of symptoms. Distal renal tubular acidosis (dRTA) and tubulointerstitial nephritis (TIN) might be present. Only a few cases of SS with decreased glomerular filtration rate (GFR) have been reported. We have studied GFR in 27 female SS-patients, mean age 62 years (37-78). GFR was measured as the single injection 51Cr-EDTA plasma clearance. Eighteen women had normal GFR (group 1), and nine (33%) had values below the lower normal limit (group 2). In group 2, dRTA was present in 8/9 urolithiasis in 6/9, previous upper urinary tract infection (UTI) in 2/9 and TIN in 5/6 patients who were kidney biopsied. Among patients with dRTA 8/18 (44%) had decreased GFR. We conclude that decreased GFR is not unusual in SS-patients with dRTA, and decreased GFR is mostly associated with TIN. Urolithiasis and UTI may contribute to decreased GFR in some individuals.

α1 Antitrypsin deficiency in a patient with systemic vasculitis and primary Sjögren's syndrome

The internal homoeostasis in man is critically dependent on regulation of proteolytic enzymes in tissues and fluids by endogenous inhibitors. α1 Antitrypsin is the most abundant protease inhibitor (Pi) in plasma, and controls tissue degradation by proteases such as trypsin, neutrophil elastase, and proteinase 3.1 Homozygous α1 antitrypsin deficiency is known to predispose to emphysema and chronic liver disease.2,3 Recently, a strong correlation has been found between systemic small vessel necrotising vasculitis and both heterozygous and homozygous α1 antitrypsin deficiency.4 Also, such deficiency has been found to confer a more disseminated disease and worse prognosis to patients with antineutrophil cytoplasmic antibody (ANCA) positive vasculitis.5 However, there is disagreement about the clinical implication of an intermediate α1 antitrypsin deficiency: is it an accidental finding or does it imply susceptibility to autoimmune disease? Thus, reports have …

Evaluation of lip salivary gland biopsy in 21 patients with primary Sjögren's syndrome

Lip salivary gland biopsy was performed in 21 (20 female) patients, age 26 to 74 years, with primary Sjögrens syndrome. The procedure helped identify a patient group with many autoimmune phenomena, including hypergammaglobulinemia, autoantibodies, autoimmune thyroiditis, vasculitis, and immunocytoma. A marked association with the HLA-DR3 antigen was noted. It is concluded that lip salivary gland biopsy is a simple and safe procedure that makes possible the identification among non-RA patients with sicca symptoms those who have autoimmune disturbance. This patient group deserves close medical supervision in order to detect autoimmune disease that requires treatment, such as autoimmune thyroiditis and lymphoma.