A clinicopathological study of the adult Henoch-Schonlein Purpura.
- Author:
Eun Hoe KWON
1
;
Sung Jin KIM
;
Min Ah NA
;
Yoo Suck JUNG
;
Dong Won LEE
;
Soo Bong LEE
;
Ihm Soo KWAK
Author Information
1. Department of Intermal Medicine, Pusan National University College of Medicine, Busan, Korea. paradox67145@hanmail.net
- Publication Type:Original Article
- Keywords:
Henoch-Shnlein purpura;
Renal involvement
- MeSH:
Abdominal Pain;
Acute Kidney Injury;
Adult*;
Ankle;
Arthralgia;
Biopsy;
Busan;
Cause of Death;
Classification;
Exanthema;
Female;
Hematuria;
Hemorrhage;
Humans;
Immunoglobulin A;
Incidence;
Joints;
Knee;
Lower Extremity;
Male;
Methylprednisolone;
Nephritis;
Peptidyl-Dipeptidase A;
Peritonitis;
Proteinuria;
Purpura;
Purpura, Schoenlein-Henoch*;
Recurrence;
Renal Dialysis;
Renal Insufficiency;
Respiratory Distress Syndrome, Adult;
Retrospective Studies;
Seasons;
Skin;
Steroids
- From:Korean Journal of Medicine
2003;65(3):323-334
- CountryRepublic of Korea
- Language:Korean
-
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.
