Subclinical pulmonary haemorrhage causing a restrictive lung defect in three siblings with a unique urticarial vasculitis syndrome

B. M S Al Riyami, J. K. Al Kaabi, E. M. Elagib, H. S. El Khatim, N. J Y Woodhouse

Research output: Contribution to journalArticle

14 Citations (Scopus)

Abstract

Three siblings with urticarial vasculitis syndrome (UVS) are described. All had restrictive lung function abnormalities caused by subclinical pulmonary haemorrhage. The latter was suspected after finding haemosiderin-laden macrophages and a friable bronchial mucosa during elective bronchopulmonary alveolar lavage (BAL). The chest radiographs were normal at presentation but after steroid withdrawal symptoms worsened, haemoglobin levels fell, and Case 1 developed acute pulmonary haemorrhage. This was documented by lung biopsy, which also revealed evidence of old haemorrhage and fibrosis. We concluded that these patients had a unique familial variant of UVS with a previously unreported restrictive lung disease due to subclinical pulmonary haemorrhage.

Original languageEnglish
Pages (from-to)309-313
Number of pages5
JournalClinical Rheumatology
Volume22
Issue number4-5
DOIs
Publication statusPublished - Oct 2003

Fingerprint

Vasculitis
Siblings
Hemorrhage
Lung
Hemosiderin
Substance Withdrawal Syndrome
Bronchoalveolar Lavage
Lung Diseases
Mucous Membrane
Hemoglobins
Fibrosis
Thorax
Macrophages
Steroids
Biopsy

Keywords

  • Subclinical pulmonary haemorrhage
  • Urticarial vasculitis

ASJC Scopus subject areas

  • Rheumatology
  • Immunology

Cite this

Subclinical pulmonary haemorrhage causing a restrictive lung defect in three siblings with a unique urticarial vasculitis syndrome. / Al Riyami, B. M S; Al Kaabi, J. K.; Elagib, E. M.; El Khatim, H. S.; Woodhouse, N. J Y.

In: Clinical Rheumatology, Vol. 22, No. 4-5, 10.2003, p. 309-313.

Research output: Contribution to journalArticle

Al Riyami, B. M S ; Al Kaabi, J. K. ; Elagib, E. M. ; El Khatim, H. S. ; Woodhouse, N. J Y. / Subclinical pulmonary haemorrhage causing a restrictive lung defect in three siblings with a unique urticarial vasculitis syndrome. In: Clinical Rheumatology. 2003 ; Vol. 22, No. 4-5. pp. 309-313.
@article{38594437b4ad44e7bd2938466b2c0730,
title = "Subclinical pulmonary haemorrhage causing a restrictive lung defect in three siblings with a unique urticarial vasculitis syndrome",
abstract = "Three siblings with urticarial vasculitis syndrome (UVS) are described. All had restrictive lung function abnormalities caused by subclinical pulmonary haemorrhage. The latter was suspected after finding haemosiderin-laden macrophages and a friable bronchial mucosa during elective bronchopulmonary alveolar lavage (BAL). The chest radiographs were normal at presentation but after steroid withdrawal symptoms worsened, haemoglobin levels fell, and Case 1 developed acute pulmonary haemorrhage. This was documented by lung biopsy, which also revealed evidence of old haemorrhage and fibrosis. We concluded that these patients had a unique familial variant of UVS with a previously unreported restrictive lung disease due to subclinical pulmonary haemorrhage.",
keywords = "Subclinical pulmonary haemorrhage, Urticarial vasculitis",
author = "{Al Riyami}, {B. M S} and {Al Kaabi}, {J. K.} and Elagib, {E. M.} and {El Khatim}, {H. S.} and Woodhouse, {N. J Y}",
year = "2003",
month = "10",
doi = "10.1007/s10067-003-0738-x",
language = "English",
volume = "22",
pages = "309--313",
journal = "Clinical Rheumatology",
issn = "0770-3198",
publisher = "Springer London",
number = "4-5",

}

TY - JOUR

T1 - Subclinical pulmonary haemorrhage causing a restrictive lung defect in three siblings with a unique urticarial vasculitis syndrome

AU - Al Riyami, B. M S

AU - Al Kaabi, J. K.

AU - Elagib, E. M.

AU - El Khatim, H. S.

AU - Woodhouse, N. J Y

PY - 2003/10

Y1 - 2003/10

N2 - Three siblings with urticarial vasculitis syndrome (UVS) are described. All had restrictive lung function abnormalities caused by subclinical pulmonary haemorrhage. The latter was suspected after finding haemosiderin-laden macrophages and a friable bronchial mucosa during elective bronchopulmonary alveolar lavage (BAL). The chest radiographs were normal at presentation but after steroid withdrawal symptoms worsened, haemoglobin levels fell, and Case 1 developed acute pulmonary haemorrhage. This was documented by lung biopsy, which also revealed evidence of old haemorrhage and fibrosis. We concluded that these patients had a unique familial variant of UVS with a previously unreported restrictive lung disease due to subclinical pulmonary haemorrhage.

AB - Three siblings with urticarial vasculitis syndrome (UVS) are described. All had restrictive lung function abnormalities caused by subclinical pulmonary haemorrhage. The latter was suspected after finding haemosiderin-laden macrophages and a friable bronchial mucosa during elective bronchopulmonary alveolar lavage (BAL). The chest radiographs were normal at presentation but after steroid withdrawal symptoms worsened, haemoglobin levels fell, and Case 1 developed acute pulmonary haemorrhage. This was documented by lung biopsy, which also revealed evidence of old haemorrhage and fibrosis. We concluded that these patients had a unique familial variant of UVS with a previously unreported restrictive lung disease due to subclinical pulmonary haemorrhage.

KW - Subclinical pulmonary haemorrhage

KW - Urticarial vasculitis

UR - http://www.scopus.com/inward/record.url?scp=0345328730&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0345328730&partnerID=8YFLogxK

U2 - 10.1007/s10067-003-0738-x

DO - 10.1007/s10067-003-0738-x

M3 - Article

C2 - 14579162

AN - SCOPUS:0345328730

VL - 22

SP - 309

EP - 313

JO - Clinical Rheumatology

JF - Clinical Rheumatology

SN - 0770-3198

IS - 4-5

ER -