ABSTRACT
Abstract A 66-year-old woman showing renal dysfunction with elevated serum alkaline phosphatase and anti-SS-A antibody was admitted. A labial salivary gland biopsy showing infiltration of mononuclear cells and positive anti-SS-A antibody with sicca symptoms led to a diagnosis of primary Sjögren's syndrome (SS). Fanconi's syndrome was diagnosed by renal tubular acidosis along with renal glucosuria or aminoaciduria and multiple bone fractures on bone scintigraphy. Typical bilateral pulmonary shadows were confirmed as organizing pneumonia (OP) determined by the analysis of bronchoalveolar lavage fluid and transbronchial lung biopsy. A rare complication of Fanconi's syndrome with OP in SS is described.
Subject(s)
Cryptogenic Organizing Pneumonia/diagnosis , Fanconi Syndrome/complications , Fractures, Bone/etiology , Multiple Trauma/etiology , Sjogren's Syndrome/complications , Acidosis, Renal Tubular/diagnosis , Acidosis, Renal Tubular/immunology , Aged , Alkaline Phosphatase/blood , Antibodies, Antinuclear/blood , Antibodies, Antinuclear/immunology , Cryptogenic Organizing Pneumonia/immunology , Fanconi Syndrome/diagnosis , Fanconi Syndrome/immunology , Female , Fractures, Bone/diagnostic imaging , Fractures, Bone/immunology , Glycosuria, Renal/diagnosis , Glycosuria, Renal/immunology , Humans , Monocytes/immunology , Multiple Trauma/diagnostic imaging , Multiple Trauma/immunology , Radionuclide Imaging , Renal Aminoacidurias/diagnosis , Renal Aminoacidurias/immunology , Salivary Glands/immunology , Sjogren's Syndrome/diagnosis , Sjogren's Syndrome/immunologyABSTRACT
We have evaluated the autoantibody pattern, the production of specific immunoglobulins against bacteria in the urinary tract and lymphocytes populations in peripheral blood of primary renal glycosuria patients. All the affected members present autoantibodies against various antigens. In nine patients immunofluorescence revealed antibody coated bacteria in urine specimens. Imbalances in lymphocytes subpopulations are also present, with a reduction of OKT8+ cells and an increase of B and natural killer cells. These results support previous observations about immunological abnormalities in these patients.
Subject(s)
Antibodies, Bacterial/analysis , Autoantibodies/analysis , Glycosuria, Renal/immunology , Lymphocytes/immunology , Adult , Antibodies, Monoclonal , Antibody-Coated Bacteria Test, Urinary , Humans , Lymphocytes/classification , Middle AgedABSTRACT
HLA typing and a range of autoantibodies were evaluated in five families affected with type A renal glycosuria. HLA typing demonstrates that this inherited disease is controlled by an autosomal dominant gene located on chromosome six in close genetic linkage with the HLA complex. All affected family members have significant titres of autoantibodies to nuclear antigens, native DNA, smooth muscle, mitochondria, liver antigens, thyroglobulin, thyroid microsomes and renal tubule brush border with variable association. This suggests that renal glycosuria is a complex HLA-linked disease with increased susceptibility to multiple autoantibody production and this urges caution with respect to its classical definition as a benign condition.
