Henoch Schonlein Purpura with Rheumatic Carditis

In 1948 Gairdner firstly defined the linkage of Schoenlein-Henoch purpura (HSP) vasculitis with rheumatic fever. Since the first report of that association, only a few cases of HSP with acute rheumatic fever have been reported.

HSP vasculitis and acute rheumatic fever are both multisystemic diseases affecting joints and heart. Some patient had migratory arthritis and valvulitis, which favored acute rheumatic fever. Arthritis and arthralgia may be found in HSP, but arthritis in HSP patients is non- migratory. It is proposed that antigenic stimuli of group-A beta- hemolytic streptococcus (GABHS) infections may trigger HSP. However, elevated ASO titers suggested a recent streptococcus infection.

Different immunological mechanisms are responsible for the pathogenesis of HSP vasculitis and acute rheumatic fever. Cellular immune response mediated by T-lymphocytes to streptococcal M-proteins cause destruction of cardiac tissues. HSP vasculitis is an IgA mediated disorder, raised serum IgA levels, IgA class antibodies and immune complexes have been described in those patients. In some patient serum IgA level had been elevated and skin biopsy demonstrated leucocytoclastic vasculitis which was consistent with HSP vasculitis. Even though the role of GABHS infection in HSP vasculitis isn’t clear, GABHS antigens may initiate parallel immunological processes resulting in both disorders.

Cardiac involvement in HSP vasculitis patients is very rare and previously there was only one report of HSP and acute rheumatic fever co-occurrence without time interval between those disorders. Some patients were firstly diagnosed as HSP vasculitis and 3 days after, a cardiac murmur of acute valvulitis and migratory arthritis developed. The development of acute rheumatic fever at the same time with HSP vasculitis.

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