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

Calvo-Río V, Loricera J, Mata C, Martín L, Ortiz-Sanjuán F, Alvarez L, González-Vela MC, González-Lamuño D, Rueda-Gotor J, Fernández-Llaca H, González-López MA, Armesto S, Peiró E, Arias M, González-Gay MA, Blanco R - Medicine (Baltimore) (2014)

Bottom Line: 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.

View Article: PubMed Central - PubMed

Affiliation: From Divisions of Rheumatology (VC-R, JL, CM, FO-S, JR-G, EP, MAG-G, RB), Nephrology (LM, MA), Pediatrics (LA, DG-L), Pathology (MCG-V), and Dermatology (HF-L, MAG-L, SA), Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain.

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.

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Related in: MedlinePlus

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.)
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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.)

Mentions: We reviewed the clinical records of all patients with a diagnosis of cutaneous vasculitis seen in our center (Hospital Universitario Marqués de Valdecilla, Santander, Spain) between January 1975 and December 2012. Special attention was paid to patients with a diagnosis of vasculitis seen at the rheumatology, dermatology, nephrology, pediatrics, and internal medicine divisions. In addition, we tracked information on vasculitis from the pathology division. We assessed 417 patients seen at a single center who were classified as having HSP according to the criteria proposed by Michel et al33 (Table 1). Patients who did not fulfill these criteria were not included in the study. These criteria were based on the American College of Rheumatology (ACR) database and methodology.33,34 HSP was pathologically confirmed in 110 cases by a skin biopsy showing the characteristic histologic findings consistent with leukocytoclastic vasculitis (Figure 1), including neutrophilic infiltration; leukocytoclasia; fibrinoid necrosis into the vessel wall of arterioles, capillaries, and postcapillary venules; and red cell extravasation.16,28 The remaining 307 patients without skin biopsy had typical nonthrombocytopenic symmetric palpable purpura (Figure 2). In addition, all of them fulfilled the criteria proposed by Michel et al.33


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

Calvo-Río V, Loricera J, Mata C, Martín L, Ortiz-Sanjuán F, Alvarez L, González-Vela MC, González-Lamuño D, Rueda-Gotor J, Fernández-Llaca H, González-López MA, Armesto S, Peiró E, Arias M, González-Gay MA, Blanco R - Medicine (Baltimore) (2014)

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.)
© Copyright Policy
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4616305&req=5

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.)
Mentions: We reviewed the clinical records of all patients with a diagnosis of cutaneous vasculitis seen in our center (Hospital Universitario Marqués de Valdecilla, Santander, Spain) between January 1975 and December 2012. Special attention was paid to patients with a diagnosis of vasculitis seen at the rheumatology, dermatology, nephrology, pediatrics, and internal medicine divisions. In addition, we tracked information on vasculitis from the pathology division. We assessed 417 patients seen at a single center who were classified as having HSP according to the criteria proposed by Michel et al33 (Table 1). Patients who did not fulfill these criteria were not included in the study. These criteria were based on the American College of Rheumatology (ACR) database and methodology.33,34 HSP was pathologically confirmed in 110 cases by a skin biopsy showing the characteristic histologic findings consistent with leukocytoclastic vasculitis (Figure 1), including neutrophilic infiltration; leukocytoclasia; fibrinoid necrosis into the vessel wall of arterioles, capillaries, and postcapillary venules; and red cell extravasation.16,28 The remaining 307 patients without skin biopsy had typical nonthrombocytopenic symmetric palpable purpura (Figure 2). In addition, all of them fulfilled the criteria proposed by Michel et al.33

Bottom Line: 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.

View Article: PubMed Central - PubMed

Affiliation: From Divisions of Rheumatology (VC-R, JL, CM, FO-S, JR-G, EP, MAG-G, RB), Nephrology (LM, MA), Pediatrics (LA, DG-L), Pathology (MCG-V), and Dermatology (HF-L, MAG-L, SA), Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain.

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.

Show MeSH
Related in: MedlinePlus