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PubMed2003

[Henoch-Schönlein purpura - one of the most common types of systemic vasculitis in childhood].

UNLABELLED: The aim of our study was to estimate etiology, most common clinical findings, the course and peculiarities of treatment in children to whom Henoch-Schönlein purpura was diagnosed during 1996-2002 at Clinic of Children's Diseases of Kaunas University of Medicine Hospital. METHODS: The Henoch-Schönlein purpura diagnosis was based on the association of non-trombocytopenic purpura, arthritis and abdominal pain in 45 children, mostly at preschool age. Routine laboratory blood tests usually were normal. Serum level of imunoglobulin A (Ig A), complement 3, 4 (C(3), C(4)), antineutrophil cytoplasm antibodies were measured for some patients. Urinary analyses were performed for all patients in order to assess nephritis. RESULTS: In 1/3 (33.3%) of children Henoch-Schönlein purpura was preceded by an upper respiratory tract infection. The most common clinical signs were: non-trombocytopenic purpura -100%, subcutaneous edema - 53%, arthritis of large joints - 64%, gastrointestinal symptoms (pain, diarrhea with bleeding) - 37.5%. The incidence of renal involvement was 13.3%, but usually not at the onset of the disease. Relapses were often, and they had tendency to repeat in 1-2 week periods. Late relapses were absent. Treatment of Henoch-Schönlein purpura was symptomic. Nonsteroidal anti-inflammatory drugs which are helpful in joint pain, were prescribed for 33.3% patients. Steroids which reduce abdominal pain, melena, massive hemorrhage, nephritic symptoms, were given for 28.8% patients. CONCLUSIONS: Non-trombocytopenic purpura, arthritis and colic abdominal pain, classic triad of Henoch-Schönlein purpura that occurs in early stage of the disease, were most common. Renal involvement had tendency occur later. Nonsteroidal anti-inflammatory drugs were prescribed for patients with arthritis; prednisone reduced gastrointestinal, nephritic symptoms. Relapses were often.

Adolescent↗
PubMedDate not supplied

The role of myeloperoxidase in the pathogenesis of systemic vasculitis.

Wegener's granulomatosis, microscopic polyangiitis, Churg-Strauss syndrome and idiopathic pauci-immune necrotizing crescentic glomerulonephritis are strongly associated with the presence of anti-neutrophil cytoplasmic antibodies (ANCA). These ANCA-associated vasculitides can serologically be separated into myeloperoxidase (MPO)-ANCA and proteinase 3 (PR3)-ANCA positive patients. The unique properties of the antigen targeted by the anti-MPO antibodies could help to explain the specific characteristics of MPO-ANCA associated disease. Recently, an animal model has been developed that proves that anti-mouse MPO immunoglobulins alone are capable of causing disease similar to that in humans. Also, the in vitro pathologic effects of binding of MPO-ANCA to MPO are better understood. MPO-ANCA can activate (primed) neutrophils directly causing extensive reactive oxygen species formation and degranulation of neutrophil constituents, including MPO, resulting in a destructive inflammatory response towards the vessel wall. MPO-ANCA can prevent the clearing and inactivation of MPO by ceruloplasmin as well, resulting in increased myeloperoxidase activity. Myeloperoxidase produces not only the strong oxidant bleach (hypochlorous acid) out of hydrogen peroxide and chloride ions but also oxidizes LDL into a macrophage high-uptake form, inactivates protease inhibitors, and consumes nitric oxide. These may contribute to endothelial dysfunction and add to the chronic renal lesions observed in patients with MPO-ANCA. MPO levels are influenced by genetic factors including two, MPO463 and MPO129, single nucleotide polymorphisms. The MPO 463 polymorphism has been associated with an increased risk of development of MPO-ANCA associated disease.

Animals↗
PubMed2005

[New therapeutic strategies in ANCA-associated systemic vasculitis].

High-dose corticosteroids in combination with cytotoxic drugs are universally accepted as the initial treatment in ANCA-associated vasculitis. Cyclophosphamide is the most effective cytotoxic drug and is used in more severe cases. Because of short- and long-term side-effects, novel therapies have been tested. New prospects for the treatment of vasculitis include sequential chemotherapy (e.g. cyclophosphamide followed by azathioprine or cyclophosphamide followed by methotrexate) and/or novel immunosuppressive agents (e.g. mycophenolate mofetil, anti-TNF-alpha).

Antibodies, Antineutrophil Cytoplasmic↗
PubMed1991

IgG subclass distribution and relative functional affinity of anti-myeloperoxidase antibodies in systemic vasculitis at presentation and during follow-up.

Circulating IgG autoantibodies to myeloperoxidase (MPO) are associated with renal vasculitis and have been implicated in its pathogenesis. However, raised levels of these autoantibodies may persist during clinical remission. We tested whether this paradox could be explained by immunoglobulin subclass switching during disease evolution, since different subclasses have different immunological and biochemical properties. Sera with anti-myeloperoxidase (anti-MPO) activity from 33 patients with active disease and 20 anti-MPO positive follow-up sera were studied by an ELISA using a panel of anti-human IgG subclass monoclonal reagents previously calibrated on human myeloma proteins. Anti-MPO subclass distribution in initial sera was: IgG1, 31 (94%); IgG2, 10 (30%); IgG3, 24 (73%); and IgG4, 22 (67%). IgG3 anti-MPO decreased during follow-up (P less than 0.02), with no change in IgG1 and IgG4. Relative functional affinity of anti-MPO antibodies in purified IgG subclasses was studied by the diethylamine method. IgG3 fractions consistently had a greater affinity for MPO than the other subclasses. Sequential studies in four patients demonstrated an affinity maturation for IgG1 and IgG4 anti-MPO as IgG3 anti-MPO disappeared. We conclude that dynamic changes of subclass distribution and affinity may explain discrepancies between anti-MPO antibody titre and disease expression.

Antibody Affinity↗