Henoch-Schönlein purpura in northern Spain: clinical spectrum of the disease in 417 patients from a single center

Abstract

The severity of clinical features and the outcomes in previous series of patients reported with Henoch-Schönlein purpura (HSP) vary greatly, probably due to selection bias. To establish the actual clinical spectrum of HSP in all age groups using an unselected and wide series of patients diagnosed at a single center, we performed a retrospective review of 417 patients classified as having HSP according to the criteria proposed by Michel et al. Of 417 patients, 240 were male and 177 female, with a median age at the time of disease diagnosis of 7.5 years (interquartile range [IQR], 5.3-20.1 yr). Three-quarters of the patients were children or young people aged 20 years or younger (n = 315), and one-quarter were adults (n = 102). The most frequent precipitating events were a previous infection (38%), usually an upper respiratory tract infection, and/or drug intake (18.5%) shortly before the onset of the vasculitis. At disease onset the most common manifestations were skin lesions (55.9%), nephropathy (24%), gastrointestinal involvement (13.7%), joint symptoms (9.1%), and fever (6.2%). Cutaneous involvement occurring in all patients, mainly purpuric skin lesion, was the most common manifestation when the vasculitis was fully established, followed by gastrointestinal (64.5%), joint (63.1%), and renal involvement (41.2%). The main laboratory findings were leukocytosis (36.7%), anemia (8.9%), and increased serum IgA levels (31.7%). The most frequent therapies used were corticosteroids (35%), nonsteroidal antiinflammatory drugs (14%), and cytotoxic agents (5%). After a median follow-up of 12 months (IQR, 2-38 mo), complete recovery was observed in most cases (n = 346; 83.2%), while persistent, usually mild, nephropathy was observed in only 32 (7.7%) cases. Relapses were observed in almost a third of patients (n = 133; 31.9%).In conclusion, although HSP is a typical vasculitis affecting children and young people, it is not uncommon in adults. The prognosis is favorable in most cases, depending largely on renal involvement.

FIGURE 1

Microscopic lower-power view of skin biopsy of a patient with Henoch-Schönlein purpura showing leukocytoclastic vasculitis. (H & E stain, original magnification × 6). (Figure courtesy of Dr. González-Vela, MD, PhD, Pathology Division.)

FIGURE 2

Typical nonthrombocytopenic palpable purpura in the lower extremities of a patient presenting with Henoch-Schönlein purpura. (Figure courtesy of Dr. López Escobar, MD, Dermatology Division.)

FIGURE 3

Renal biopsy from a patient with Henoch-Schönlein purpura nephropathy with mesangial glomerulonephritis (H & E stain, original magnification × 100). (Figure courtesy of Dr. González-Vela, MD, PhD, Pathology Division.)

FIGURE 4

Main clinical features of 417 adult and child patients with Henoch-Schönlein purpura at disease onset (A) and when the disease was fully established (B). Data are expressed as percentages.

FIGURE 5

Main laboratory data of 417 patients with Henoch-Schönlein purpura by age: adults and children. Data are expressed as percentages.

FIGURE 6

Main differences in laboratory data, treatment, and outcome between patients with Henoch-Schönlein purpura with and without nephropathy. Data are expressed as percentages.