Impairment of lung diffusion capacity in Schönlein-Henoch purpura

doi:10.1016/S0022-3476(05)82533-8

The Journal of Pediatrics

Volume 121, Issue 1, July 1992, Pages 12-16

https://doi.org/10.1016/S0022-3476(05)82533-8 Get rights and content

Twenty-nine children with typical Schönlein-Henoch purpura (SHP) were tested at the initial phase of the disease for respiratory function. Of the 29 patients, 28 had a decrease of lung transfer for carbon monoxide (TLCO) as measured by a steady-state method. Lung volumes and blood gas values were normal; slight radiologic signs of interstitial lung involvement were observed in 18 of 26 patients. There was a decrease in TLCO to 56.8% of normal values for height and gender and to 58.5% when normal values were volume-adjusted to functional residual capacity. In 19 of 25 patients, TLCO measurements were performed at 3-month intervals during follow-up. In all cases, normalization of TLCO values was observed only after complete clinical recovery from SHP. All children with persisting symptoms, even limited to microscopic hematuria or slight proteinuria, had low TLCO values. In one patient low TLCO during follow-up preceded a late relapse of SHP in the form of acute nephritic disease with characteristic IgA deposits on renal biopsy. We conclude that low TLCO in SHP is probably related to alteration of the alveolar-capillary membrane by circulating immune complexes. This noninvasive technique may be useful in diagnosis, and during the follow-up of the disease as an early indicator of reactivation.

References (16)

KathuriaS et al.
Fatal pulmonary Henoch-Schönlein syndrome
Chest
(1982)
SaulsburyFT
IgA rheumatoid factor in Henoch-Schönlein purpura
J Pediatr
(1986)
ThompsonAJ et al.
Vascular IgA deposits in clinically normal skin of patients with renal disease
Pathology
(1980)
KauffmannRH et al.
Circulating IgA immune complexes in Schönlein-Henoch purpura: a longitudinal study of their relationship to disease activity
Am J Med
(1980)
AllenJM et al.
Anaphylactoid purpura in children (Schölein-Henoch syndrome)
Am J Dis Child
(1960)
NielsenHE
Epidemiology of Schönlein-Henoch purpura
Acta Paediatr Scand
(1988)
HabibR et al.
Renal involvement in Schönlein-Henoch purpura
ChaussainM et al.
Lung transfer factor for CO in normal children by a steady-state method
Bull Eur Physiopathol Respir
(1982)

There are more references available in the full text version of this article.

Cited by (67)

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2022, Disease-a-Month
Citation Excerpt :
It is likely that pulmonary involvement in IgA disease occurs when the alveolar capillaries are damaged via an autoimmune pathway leading to basement membrane damage.55 A decrease of about 57% in lung transfer for carbon monoxide (TLCO) was demonstrated in children with IgA vasculitis, and this was attributed to damage to the alveolar capillary membrane.56 Despite IgA vasculitis being more common in the pediatric population, the clinical finding of DAH occurs more frequently in the adult population.57
Pulmonary renal syndrome (PRS) is a constellation of different disorders that cause both rapidly progressive glomerulonephritis and diffuse alveolar hemorrhage. While antineutrophil cytoplasmic antibody associated vasculitis and anti-glomerular basement membrane disease are the predominant causes of PRS, numerous other mechanisms have been shown to cause this syndrome, including thrombotic microangiopathies, drug exposures, and infections, among others. This syndrome has high morbidity and mortality, and early diagnosis and treatment is imperative to improve outcomes. Treatment generally involves glucocorticoids and immunosuppressive agents, but treatment targeted to the underlying disorder can improve outcomes and mitigate side effects. Familiarity with the wide range of possible causes of PRS can aid the clinician in workup, diagnosis and early initiation of treatment. This review provides a summary of the clinical presentation, etiologies, pathophysiology, and treatment of PRS.
Leukocytoclastic Vasculitis: Henoch–Schönlein Purpura and Hypersensitivity Vasculitis
2015, Textbook of Pediatric Rheumatology
Pulmonary Hemorrhage in Henoch-Schönlein Purpura: Case Report and Systematic Review of the English Literature
2013, Seminars in Arthritis and Rheumatism
Citation Excerpt :
Recurrent unrecognized DAH may be associated with reticular infiltrates mimicking an interstitial lung disease and was observed in 13.9% of patients (11,22). Subclinical reductions of transfer factor of the lung for carbon monoxide (TLCO) with active vasculitis (60), the association of reduced TLCO with systemic relapses (60,61), and the finding of hemosiderin-laden macrophages in the absence of pulmonary symptoms and normal radiographs (21) suggest that incipient alveolar hemorrhage may be more common than is clinically evident. The prognostic implication of subclinical alveolar hemorrhage, however, remains unclear.
Diffuse alveolar hemorrhage (DAH) is a rare complication of Henoch-Schönlein purpura (HSP) and data on its prevalence, management, and outcomes are scant.
To enable evidence-based management of DAH in HSP.
A case report and a systematic review were conducted of all reported cases of DAH complicating HSP in the English literature.
DAH predominantly affects older male children and adults with HSP. The occurrence of DAH in HSP is rare and the reported prevalence ranged from 0.8% to 5%. DAH occurred variably after the diagnosis of HSP, ranging from 2 days to 18 years. Hemoptysis (75%), drop in hemoglobin (74%), and chest infiltrates (94%) were the most common clinical findings. Lung biopsy showed leukocytoclastic vasculitis with alveolar hemorrhage (69.2%) or only alveolar hemorrhage (31.8%) with variable IgA staining by immunofluorescence. DAH was frequently severe and 50% of the patients required mechanical ventilation. Cyclophosphamide and pulse methylprednisolone for DAH was associated with better outcomes, particularly in patients who were already receiving steroids at the time of DAH. Steroids and immunosuppressants were administered for a median duration of 9 and 4.5 months, respectively. Systemic recurrences (27.7%) and recurrences of DAH (8.3%) were frequent. DAH was associated with high mortality (27.6%) and morbidity (persistent urinary abnormalities, 12%; chronic renal failure, 9%; complications of therapy, 27%).
DAH is a life-threatening complication in HSP. Current protocols use pulse methylprednisolone and cyclophosphamide for 6 months.
Leukocytoclastic vasculitis
2011, Textbook of Pediatric Rheumatology
Henoch-Schönlein Purpura
2008, Pediatric Emergency Medicine
Pulmonary Manifestations of Systemic Disorders
2008, Pediatric Respiratory Medicine

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^†: Supported by grant No. 90CN48 from INSERM, Paris, France.

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Original articleImpairment of lung diffusion capacity in Schönlein-Henoch purpura†

Chest

J Pediatr

Pathology

Am J Med

Anaphylactoid purpura in children (Schölein-Henoch syndrome)

Am J Dis Child

Epidemiology of Schönlein-Henoch purpura

Acta Paediatr Scand

Renal involvement in Schönlein-Henoch purpura

Lung transfer factor for CO in normal children by a steady-state method

Bull Eur Physiopathol Respir

Original article
Impairment of lung diffusion capacity in Schönlein-Henoch purpura†