Kidney Damage in Hemorrhagic Vasculitis Occurring in Childhood and Adulthood

Abstract

Introduction. Nephropathy is diagnosed in 30–60 % of patients with hemorrhagic vasculitis (HV) (Schönlein Henoch puprupa) and occurred in each fourth of them in the onset of the disease and with the same incidence at first recurrence of the patho­logical process. In recent years, the relative and absolute number of patients with this form of glomerular disease significantly increased. According to the results of the kidney biopsy in children, Henoch glomerulonephritis (HGN) is the most common variant of the secondary immunoglobulin (Ig) A nephritis. The nature of the clinical course and morphological manifestations of the HGN in patients with HV, which began in childhood and adulthood, remains unexplored. This was the purpose and objectives of this study. Materials and methods. The study included 174 patients with HV (83 % of men and 47 % of women). In 92 cases, vasculitis debuted in children (on average in 12 years), and in 82 — in the adults (on average in 25 years). I, II and III degree of activity of pathological process are set at a ratio of 1 : 2 : 2. Seropositivity for high levels of IgA occurred in 40 % of cases, by the presence of rheumatoid factor — in 27 %. At the time of the survey, cutaneous syndrome was diagnosed in 68 % of patients in the form of urticarial, hemorrhagic, papule-nodular, papule-necro­tic, pustular-ulcerative, necrotic-ulcerative, nodose-ulcerative and polymorphic forms, and articular syndrome — in 48 %. In 24 cases, kidney biopsy was performed. Results. Renal disease was revealed in 71 % of patients with HV, while on the background of nephropathy the integral index of the severity of extrarenal patho­logy was significantly higher. According to the characteristics of the articular syndrome, patients with nephropathy and without it differed little among themselves. The severity of muscle syndrome has the impact on the development of the HV. In turn, renal pathology significantly influenced the development of changes in interphalangeal joints of the feet and ligamentosis of knee joints. There is a direct connection between the renal pathology and lesion of maxillary joints and formation of tendovaginitis. Nephro­tic syndrome is diagnosed in 4 % of patients with HV, and chronic renal disease — in 28 % (the ratio of I, II, III and IV its stages was 10 : 5 : 1 : 1). The rate of progression of renal pathology has influenced by patients’ age at the onset of HV, the degree of di­sease activity, lesions of skin and gastrointestinal tract, the level of mean arterial pressure has been influenced by the involvement in the process of the pancreas, the peripheral vascular resistance — the liver, spleen and heart. Nephrotic syndrome and chronic kidney disease stage IV took place only in cases of onset of the disease in adulthood, hypertension was registered 1.6 times more likely, and decreased glomerular filtration rate — 2.4 times. IV and V morphological classes of HGN were found only in patients with the beginning of HV in adulthood, and VI — exceptionally in children. Morphology of the HGN in both groups resembled mesangioproliferative primary glomerulonephritis with tubulointerstitial component. In patients with disease onset in adulthood, lymphohistiocytic infiltration of blood vessels observed 2.3 times more frequently. Age at the onset glomerulonephritis affects the deposition in glomeruli IgM, and in vessels — IgA, IgG and IgM. And there are direct correlations between the age of the patients and glomerular deposition of IgA and vascular IgG. Conclusions. Regardless of the age of the patients, nephropathy occurs with the same frequency at the onset of HV, but in cases of onset of the di­sease in adulthood, the course of HGN is less favorable, followed by nephrotic syndrome, higher incidence of lesions of renal glo­meruli and stroma in the process of studying of kidney biopsies data, as well as different rates of progression of renal failure, levels of the lymphohistiocytic infiltration of the vascular wall and deposition of immune complexes in the glomeruli and vessels.

Keywords

• hemorrhagic vasculitis
• kidney
• children
• adults