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Diffuse alveolar hemorrhage may be fatal complication of Henoch-Schönlein purpura

Due to its life-threatening nature, diffuse alveolar hemorrhage in Henoch-Schönlein purpura should be diagnosed and managed immediately, according to published findings.

“Henoch-Schönlein purpura is the most common vasculitis of childhood and affects the small blood vessels,” Giada Maria Di Pietro, MD, of the Institute of Pediatrics at the University of Milan, and colleagues wrote. “Pulmonary involvement is a rare complication of Henoch-Schönlein purpura and diffuse alveolar hemorrhage is the most frequent clinical presentation.”

The researchers added that little is known about the frequency, diagnosis, management and outcomes of pulmonary complications in pediatric Henoch-Schönlein purpura.

The current study is a literature review of cases of this disease reported among pediatric patients between 1979 and 2019. The search ultimately yielded 23 patients with lung involvement.

Not only was diffuse alveolar hemorrhage the most frequently observed clinical presentation for Henoch-Schönlein purpura, most of the children with pulmonary complications also experienced renal involvement. Hematuria often associated with proteinuria, nephritis or nephrotic proteinuria and acute kidney injury were the most common of these renal outcomes.

Therefore, the researchers wrote that patients experiencing co-occurrence of rapidly progressive glomerulonephritis with diffuse alveolar hemorrhage may be diagnosed as having pulmonary renal syndrome, which may be autoimmune-related. The “Gd-IgA1 pathway is probably involved” in this particular patient subcohort.

The researchers further noted that 12 of the 23 patients in their cohort had been screened for antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Eleven of these tests came up negative.

The gold standard for diagnosis of diffuse alveolar hemorrhage in Henoch-Schönlein purpura is bronchoalveolar lavage, according to the researchers. However, they noted that chest X-ray may also be used for diagnosis. Clinical presentations include cough, hemoptysis, epistaxis, dyspnea, tachypnea, chest pain and shortness of breath.

First-line therapy in this patient population should be pulse methylprednisolone. In the case of respiratory failure, immunosuppressive therapy composed of cyclophosphamide or azathioprine plus corticosteroids is necessary. It was suggested that oral corticosteroids are insufficient as a preventive measure for diffuse alveolar hemorrhage among these patients.

Other findings indicated mortality in four of the 23 patients in the cohort. Resolution of the pulmonary involvement was reported in 18 patients.

“Limited data are available for the prevention and treatment of lung involvement in Henoch-Schönlein purpura,” the researchers concluded. Although they underscored the use of methylprednisolone, researchers wrote that further data are needed to clarify debate on the ideal intervention. “No guidelines regarding the treatment of diffuse alveolar hemorrhage are so far available.” – by Rob Volansky

Disclosures: The authors report no relevant financial disclosures.