A clinicopathological study of the adult Henoch-Schonlein Purpura

Kwon, Eun Hoe; Kim, Sung Jin; Na, Min Ah; Jung, Yoo Suck; Lee, Dong Won; Lee, Soo Bong; Kwak, Ihm Soo

Abstract

BACKGROUND: Henoch-Shnlein purpura is a systemic disease and has characteristic features of purpuric skin rash, abdominal pain, arthralgia and abnormal urinary findings and characterized by immunoglobulin A deposits in the involved organ. Renal involvement is the most important prognostic factor in Henoch-Shnlein purpura.
METHODS
I retrospectively analyzed the clinical data of 48 patients with Henoch-Shoenlein purpura and 10 biopsy-proven Henoch-Schoenlein nephritis examined at Pusan National University Hospital.
RESULTS
23 cases were males and 25 cases females. Male to female ratio was 1:1.08. The peak of seasonal incidence was seen in spring (41.7%) and winter (27.1%) At presentation, skin purpura was present in 100% of patients, arthralgia was reported in 52.1% and gastrointestinal involvement in 70.8%. 75% of the patients showed renal involvement and pulmonary hemorrhage observed in 2 cases. Common types of skin rash were petechiae, purpura and erythematous maculopapular lesion on the lower extremities. The most common gastrointestinal symptom was abdominal pain (82.3%). Frequently involved joints were knee and ankle (48.0%). Renal involvement were microscopic hematuria (50.0%), proteinuria (50.0%), gross hematuria (29.2%). 25% of patients reached renal insufficiency (GFR<70 mL/min) and 4 cases (8.3%) reached acute renal failure (GFR<30 mL/min and anuria) and 3 patients of them experienced hemodialysis. All renal biopsies showed predominant IgA mesangial deposition. There were 2 cases of grade II, 6 cases of grade III and 2 cases of grade IV by classification of Meadow. Steroids were given in 16 patients (33.3%). Methylprednisolone pulse treatements were given in 4 patients for severe nephritis. 10 patients received angiotensin converting enzyme inhibitor. Clinical remission of extrarenal symptoms was achieved in 83.3% but relapses of purpura were observed in 27.1%. Clinical remissions of nephritis defined as the absence of proteinuria, hematuria and normal renal function was achieved in only 8.3%, Abnormal urinary finding continued in 37.8%. 8.3% of patients expired.
CONCLUSION
Although it has been well known that Henoch-Shnlein purpura has benign courses, we experienced 4 case of deaths and The cause of death was acute renal faliure, pulmonary hemorrhage, adult respiratory distress syndrome, massive gastrointestinal bleeding, peritonitis due to bowel perforation.