A case of Henoch-Schönlein purpura with cardiopulmonary involvement

Abstract

Henoch-Shönlein purpura (HSP) commonly affects the kidney, causing hematuria and Immunoglobulin A type nephropathy. Extrarenal involvements are rare but can be life threatening if missed. Cardiac involvement has been described only in a handful of case reports. Although HSP is predominantly a pediatric disease, it should not be overlooked in older patients presenting with typical triad of symptoms. We report a rare case of HSP with both cardiac of pulmonary systems involvement and his clinical progress

Hematologic/Oncologic cardiomyopathy: A review and pooled analysis of pathophysiology, diagnosis and clinical management

“…Extra renal involvement is rare but can be life threatening if diagnosis is missed. Direct evidence of HSP related CM are lacking but case reports of cardiopulmonary involvement [109][110][111][112] and cardiac involvement [113,114] suggest CM of an ischaemic origin in HSP patients. Lecutier [113] described a case report of an 11-year old boy who died one month after the onset of HSP.…”

“…The study concluded that coronary spasm was the likely cause of transient myocardial ischaemia in the two patients [111]. Tan [109] reports a case of adult onset of HSP in a 60-year old woman who developed ST elevation myocardial infarction (STEMI) and pulmonary haemorrhage in the course of the disease. Prompt normalization of ST segment change suggested transient ischemic of the myocardium possible due to coronary artery spasm precipitated by vasculitis process, which causes injury to coronary vessels endothelium and releases vasoconstrictive substances from those cells (endothelin-1) that is known to possess potent vasoconstrictive actions [115].…”

“…Differential diagnosis of cutaneous purpura and myocardial infarction is challenging but the addition of other organ involvement such as kidney, lung and joints should suspect a systemic cause such as vasculitis of small to mediumsized vessels. Although HSP has a paediatric preponderance, it can also affect adults and should not be overlooked in adults presenting with typical signs of HSP and myocardial dysfunction [109]. Nevertheless, studies specific to HSP-CM are needed to clarify its diagnostic features as current evidence includes features general to cardiac dysfunction or HF.…”

“…Unlike other vasculitis, corticosteroids (anti-inflammation treatment) have not been shown to change the course of the disease although it may reduce the length of severe abdominal pain. In the case of severe cardiac involvement, treatment usually targets symptoms of cardiac (HF medication), gastrointestinal and renal dysfunction based on current guidelines [109].…”