Pulmonary Hemorrhage Associated with Henoch-Schönlein Purpura in Pediatric Patients: Case Report and Review of the Literature

doi:10.1016/j.semarthrit.2011.03.007

Seminars in Arthritis and Rheumatism

Volume 41, Issue 2, October 2011, Pages 305-312

https://doi.org/10.1016/j.semarthrit.2011.03.007 Get rights and content

Objectives

To characterize the clinical characteristics of pediatric patients with pulmonary hemorrhage and Henoch-Schönlein purpura (HSP).

Methods

Presentation of a pediatric patient with pulmonary hemorrhage associated with HSP and review of relevant cases based on a PubMed search (1966 to April 2010).

Results

We identified 17 previously reported pediatric cases with HSP and pulmonary hemorrhage. The most frequent clinical manifestations were rash (17 patients, 100%), proteinuria (14 patients, 82%), and abdominal pain (13 patients, 76%). Six patients (35%) had complete resolution of symptoms; 7 patients (41%) had partial recovery, and 4 patients (23%) died. Nine patients (53%) had acute respiratory failure following intubation and 3 of these patients (33%) died. Five patients were treated with methylprednisolone pulse therapy and 1 was also given low-dose cyclophosphamide treatment, but 2 of these 5 patients (40%) died. Three patients were given cyclophosphamide pulse therapy plus steroid (nonpulse or pulse) therapy, and all survived. Among the 6 nonintubated patients, all were given steroid treatment with or without an immunosuppressant drug, and all survived. In our reported case, plasma exchange appeared to help resolve the pulmonary hemorrhage and crescentic glomerulonephritis that were associated with HSP.

Conclusions

For pediatric HSP patients with pulmonary hemorrhage but no respiratory failure, methylprednisolone pulse or nonpulse therapy could be the first-line therapy. In the presence of respiratory failure, cyclophosphamide pulse therapy is suggested. Plasma exchange may be considered for treatment of pulmonary renal syndrome or refractory pulmonary hemorrhage.

Section snippets

Methods

We present a previously unreported case of pediatric HSP with pulmonary hemorrhage. A PubMed (www.ncbi.nlm.nih.gov/pubmed) search of the literature (1966 to April 2010) was performed to identify all previously reported cases of pulmonary hemorrhage associated with HSP by using the terms Henoch-Schönlein purpura, pediatric, pulmonary hemorrhage, and pulmonary renal syndrome. We found 16 cases and summarized the reported symptoms, clinical findings, treatments, and outcomes.

Case Report

In March of 2009, an 11-year-old girl, who had HSP diagnosed by intermittent abdominal pain, skin rash, and small intestine biopsy when she was 4 years old, was referred to our emergency department due to intermittent abdominal pain and multiple purpura over her elbows and lower legs for the previous 3 days. She also had a mild cough without sputum, vomiting, anorexia, and general malaise. A physical examination indicated multiple purpura over her elbows and distal legs and bilateral coarse

General Characteristics, Clinical Manifestation, and Laboratory Findings

Based on our PubMed review, there were 16 previously reported pediatric cases of HSP associated with pulmonary hemorrhage since 1966. Table 1 shows the general characteristics, clinical features, and laboratory findings of these 16 patients and our patient (patient 17). Nine of these cases (53%) were male and the mean age at diagnosis of HSP with pulmonary hemorrhage was 10.5 years (range, 1.75-17 years). Pulmonary hemorrhage was the first manifestation of HSP in 15 patients (88%). All patients

Discussion

HSP is a small-vessel vasculitis, usually seen in children, that is characterized by purpura, arthralgia, gastrointestinal symptoms, and glomerulonephritis. The diagnosis of HSP in our patient is based on clinical findings and pathology including typical purpura, abdominal pain, and glomerulonephritis at this admission. A renal biopsy indicated IgA deposition, which was compatible with HSP. The results of autoimmune survey in our patient were all negative except p-ANCA, which is frequently

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Cited by (25)

Adult IgA vasculitis (Henoch-Schönlein purpura)
2019, Nephrologie et Therapeutique
La vascularite à IgA, anciennement purpura rhumatoïde est une vascularite systémique des petits vaisseaux à dépôts d’immunoglobulines A (IgA). Elle est beaucoup plus fréquente chez l’enfant que chez l’adulte (150 à 200 pour 1). La prévalence de la vascularite à IgA chez l’adulte n’est pas connue et son incidence est estimée à 1 par million. La maladie de l’adulte semble en effet différer de celle de l’enfant par l’incidence et la gravité des manifestations cliniques. La vascularite à IgA est caractérisée par l’association d’un purpura vasculaire cutané à des signes articulaires et gastro-intestinaux. Une atteinte rénale s’associe parfois à ces signes. Il s’agit alors d’une glomérulonéphrite à dépôts mésangiaux d’IgA. Plus rarement des localisations neurologiques, pulmonaires, cardiaques ou urogénitales peuvent s’observer. Sa physiopathologie demeure inconnue mais les IgA joueraient un rôle central. Lorsque la symptomatologie est peu sévère seul un traitement symptomatique est conseillé. À l’opposé, dans les formes digestives ou rénales sévères, des traitements plus agressifs, associant le plus souvent, des corticostéroïdes à un immunosuppresseur ont été proposés. Leur efficacité est en cours d’évaluation. Le pronostic à court terme de la maladie dépend de la sévérité de l’atteinte digestive et à long terme, de la sévérité de l’atteinte rénale. Les études ayant un suivi suffisamment prolongé montrent qu’un tiers des malades adultes évoluent vers l’insuffisance rénale terminale, comme pour la néphropathie à IgA. De nombreux auteurs suggèrent d’ailleurs que la néphropathie à IgA et la vascularite à IgA seraient deux entités de la même maladie.
IgA vasculitis is a systemic vasculitis affecting small vessels. IgA vasculitis usually affect children whereas it is rare in adults (150 to 200 for 1) in which the disease is often more serious with more frequent and severe nephritis. Prevalence of adult IgA vasculitis is unknown and its annual incidence is 1 in 1 million. The dominant clinical features include cutaneous purpura, arthritis and gastrointestinal symptoms. Sometimes nephritis can add, typically as glomerulonephritis with IgA mesangial deposits. Pulmonary, cardiac, genital and neurological symptoms have also been observed. Although the cause is unknown, it is clear that IgA plays a pivotal role in the immunopathogenesis of IgA vasculitis. Only symptomatic treatment is advised in case of self-limited disease. Treatment of severe IgA vasculitis, nephritis or gastrointestinal manifestations, is not established but some studies, which need to be confirmed, reported the benefit of corticosteroids combined with immunosuppressive drugs. Short-term outcome depends of the severity of the gastro-intestinal manifestations. The long-term prognosis is heavily dependent on the presence and severity of nephritis. Studies with prolonged follow-up show up to one third of adult patients reaching end stage renal failure, as for IgA nephropathy. Some authors even suggest that IgA nephropathy and IgA vasculitis would be the same disease.
Diagnostic Imaging: Pediatrics
2017, Diagnostic Imaging: Pediatrics
Henoch-Schönlein purpura/immunoglobulin-A vasculitis
2015, Rheumatology: Sixth Edition
Henoch-Schönlein purpura in the adult
2014, Revue de Medecine Interne
Le purpura rhumatoïde ou purpura de Schönlein-Henoch est une vascularite systémique des petits vaisseaux en rapport avec des dépôts de complexes immuns contenant des immunoglobulines A (IgA). Il est caractérisé par l’association de signes cutanés, articulaires et gastro-intestinaux pouvant survenir par poussées successives. Une atteinte rénale est parfois associée à ces signes et sa fréquence est extrêmement variable selon les séries. Plus rarement, d’autres organes tels que le poumon, le cœur ou le système nerveux peuvent être concernés. L’histologie cutanée (vascularite leucocytoclasique) et rénale (glomérulonéphrite proliférative endocapillaire), associée à la présence de dépôts d’IgA dans ces tissus, permettent de confirmer le diagnostic clinique. Le pronostic de la maladie à court terme dépend de la sévérité de l’atteinte digestive mais à long terme, elle est tributaire de l’atteinte rénale. Les récentes publications de séries pédiatrique et adulte montrent ainsi l’existence d’une insuffisance rénale chronique, évolutive parfois plus de dix ans après la première poussée. Le traitement du purpura rhumatoïde est le plus souvent symptomatique. Le bénéfice de traitements plus spécifiques (corticostéroïdes ou immunosuppresseurs) au cours des formes viscérales sévères (le plus souvent abdominale ou rénale) n’est pas encore établi.
Henoch-Schönlein purpura is a systemic vasculitis of the small vessels characterized by perivascular leucocyte infiltrates. It is an immunoglobulin A-related immune complex-mediated disease involving the skin, the joints and the gastrointestinal system. Renal disease may sometimes be associated to these clinical manifestations. Prevalence of the nephritis is highly variable, depending on the series. More rarely, other organs such as the lungs, the heart or the nervous system may be involved. The clinical diagnosis is confirmed by histopathology of the skin (leukocytoclastic vasculitis) and kidney (endo-capillary proliferative glomerulonephritis), showing IgA deposits in these tissues. Short-term prognosis depends on the severity of digestive involvement, but long-term prognosis depends on the renal disease. Recent publications of pediatric and adult series show that the chronic renal failure may progress, sometimes more than ten years after the initial flare. Treatment is usually supportive. The benefit of more specific treatments (corticosteroids or immunosuppressive drugs) in severe visceral forms (usually abdominal or kidney) has not yet been established.
Pulmonary Affectation of Vasculitis
2012, Archivos de Bronconeumologia
La afectación del tracto respiratorio es frecuente en algunos tipos de vasculitis, fundamentalmente en las asociadas a anticuerpos anticitoplasma de neutrófilo. La presentación clínica, radiológica e histopatológica también es heterogénea y condiciona la evolución. Es necesaria, por tanto, una orientación clínica y diagnóstica precoz ya que, gracias a los nuevos tratamientos, y a pesar de ser enfermedades potencialmente graves, su pronóstico ha mejorado de manera considerable en los últimos años.
En el presente trabajo se realiza una actualización del diagnóstico y las nuevas opciones terapéuticas de las vasculitis pulmonares.
Respiratory tract affectation is frequent in some types of vasculitis, fundamentally in those associated with anti-neutrophil cytoplasmic antibodies. The clinical, radiological and histopathological presentation is also heterogeneous and conditions the evolution. It is therefore necessary to establish an early diagnosis based on the symptoms because, thanks to new treatments, and despite them being potentially serious diseases, their prognosis has improved considerably in recent years.
The present paper updates the diagnosis and the new therapeutic options for pulmonary vasculitis.
Correlates of systemic disease in adult Henoch-Schönlein purpura: A retrospective study of direct immunofluorescence and skin lesion distribution in 87 patients at Mayo Clinic
2012, Journal of the American Academy of Dermatology
Detection of IgM in lesional skin of adult patients with Henoch-Schönlein purpura via direct immunofluorescence (DIF) has been associated with the presence of renal disease.
We sought to examine whether DIF findings of skin biopsy specimens and distribution of skin lesions were associated with the presence of systemic disease, including renal, gastrointestinal tract, and joint involvement.
We performed a retrospective review of adult patients with Henoch-Schönlein purpura seen at Mayo Clinic between 1992 and 2011.
Of the 87 patients (mean age, 46.1 years), 51 (59%) were male. A total of 39 patients (45%) had renal disease; 32 (37%), gastrointestinal tract involvement; 39 (45%), joint involvement; and 65 (75%), some systemic involvement. In all, 61 patients (70%) had cutaneous lesions above the waist. The DIF findings showed the presence of IgA in all 87 patients (100%). In addition, findings were positive for IgM in 32 patients (37%); IgG in 3 patients (3%); C3 in 75 patients (87%); and fibrinogen in 78 patients (92%). IgM was not found to be significantly associated with renal disease (P = .10); however, absence of fibrinogen was correlated with presence of renal involvement (P = .04). No other correlations were detected between DIF findings and systemic disease. Lesions above the waist were not significantly associated with renal (P = .12) or any (P = .76) systemic involvement.
This study is retrospective.
Neither IgM in lesional skin nor distribution of skin lesions above the waist was a reliable indicator of renal or systemic disease in adults with Henoch-Schönlein purpura.

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: The authors have no conflicts of interest to disclose.

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MiscellaneousPulmonary Hemorrhage Associated with Henoch-Schönlein Purpura in Pediatric Patients: Case Report and Review of the Literature

Objectives

Methods

Results

Conclusions

Section snippets

Methods

Case Report

General Characteristics, Clinical Manifestation, and Laboratory Findings

Discussion

Am J Med

Lancet

Anaphylactoid purpura nephritis in childhood: natural history and immunopathology

Adv Nephrol Necker Hosp

Harrison's principles of internal medicine

Henoch-Schönlein purpura nephritis and IgA nephropathy

Henoch-Schönlein purpura: clinical presentation

Contrib Nephrol

Fatal pulmonary hemorrhage in Henoch-Schönlein purpura

Cutis

Henoch Schönlein purpura presenting with pulmonary haemorrhage

Med

Pulmonary disease with hemorrhage in Henoch-Schönlein purpura

Pediatrics

Pulmonary vasculitis with hemorrhage in anaphylactoid purpura

Pediatr Pulmonol

Pulmonary hemorrhage in an adolescent with Henoch-Schönlein purpura

West J Med

Pulmonary hemorrhage: an often fatal complication of Henoch-Schoenlein purpura

Pediatr Dermatol

Henoch-Schonlein purpura and pulmonary hemorrhage: a report and literature review

Pediatr Nephrol

Miscellaneous
Pulmonary Hemorrhage Associated with Henoch-Schönlein Purpura in Pediatric Patients: Case Report and Review of the Literature