Circulating autoantibodies as serological markers in the differential diagnosis of pulmonary renal syndrome

RAMESH SAXENA, PER BYGREN, BERTIL ARVASTSON, JÖRGEN WIESLANDER

Research output: Contribution to journalArticle

52 Citations (Scopus)

Abstract

Abstract. Objectives. Pulmonary renal syndrome (lung haemorrhage and glomerulonephritis) is a fulminant condition that warrants a rapid diagnosis and treatment to prevent mortality and preserve renal functions. However, the patients frequently present with non‐specific pulmonary symptoms in the early phase of the syndrome and the diagnosis is often missed. Recently, several autoantibodies have been described in association with various forms of glomerulonephritis. We evaluated the association as well as the diagnostic and the prognostic significance of these antibodies in pulmonary renal syndrome. Design. Retrospective clinical study. Setting. University Hospital. Subjects. Forty consecutive patients with biopsy verified glomerulonephritis and overt haemoptysis or pulmonary infiltrates compatible with lung haemorrhage. Interventions. Analysis of proteinase 3 antineutrophil cytoplasm antibodies (PR3‐ANCA), myeloperoxidase (MPO)‐ANCA, antiglomerular basement membrane (GBM) and anti‐entactin antibodies. Results. Thirty‐six (90%) patients possessed one or more autoantibodies. Twenty‐seven (70%) patients had ANCA (PR3‐ANCA, MPO‐ANCA or both). The remaining positive patients (n = 9) had anti‐GBM antibodies. Only two patients had anti‐entactin antibodies, suggesting a poor association of these antibodies with PRS. The majority of patients with anti‐GBM antibodies had a very poor clinical outcome (five irreversible renal failure; three deaths). On the other hand, despite no significant difference in clinical features or renal morphology from patients with anti‐GBM antibodies, 19 patients (70%) with ANCA recovered completely following treatment. Conclusions. Our study demonstrated that the presence of autoantibodies is a predominant feature of PRS and that the type of immunologic injury is of paramount importance in determining the course of illness in this syndrome. Analysis of the aforementioned antibodies can help in an early differential diagnosis and thus, in better management of PRS. 1995 Blackwell Publishing Ltd

Original languageEnglish (US)
Pages (from-to)143-152
Number of pages10
JournalJournal of Internal Medicine
Volume238
Issue number2
DOIs
StatePublished - 1995

Fingerprint

Autoantibodies
Differential Diagnosis
Antibodies
Antineutrophil Cytoplasmic Antibodies
Glomerulonephritis
Myeloblastin
Lung
Cytoplasm
Rapidly progressive glomerulonephritis with pulmonary hemorrhage
Hemorrhage
Kidney
Hemoptysis
Basement Membrane
Peroxidase
Renal Insufficiency
Early Diagnosis
Retrospective Studies
Biopsy
Mortality
Wounds and Injuries

Keywords

  • ANCA
  • entactin
  • glomerulonephritis
  • Goodpasture syndrome
  • haemoptysis

ASJC Scopus subject areas

  • Internal Medicine

Cite this

Circulating autoantibodies as serological markers in the differential diagnosis of pulmonary renal syndrome. / SAXENA, RAMESH; BYGREN, PER; ARVASTSON, BERTIL; WIESLANDER, JÖRGEN.

In: Journal of Internal Medicine, Vol. 238, No. 2, 1995, p. 143-152.

Research output: Contribution to journalArticle

SAXENA, RAMESH ; BYGREN, PER ; ARVASTSON, BERTIL ; WIESLANDER, JÖRGEN. / Circulating autoantibodies as serological markers in the differential diagnosis of pulmonary renal syndrome. In: Journal of Internal Medicine. 1995 ; Vol. 238, No. 2. pp. 143-152.
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AB - Abstract. Objectives. Pulmonary renal syndrome (lung haemorrhage and glomerulonephritis) is a fulminant condition that warrants a rapid diagnosis and treatment to prevent mortality and preserve renal functions. However, the patients frequently present with non‐specific pulmonary symptoms in the early phase of the syndrome and the diagnosis is often missed. Recently, several autoantibodies have been described in association with various forms of glomerulonephritis. We evaluated the association as well as the diagnostic and the prognostic significance of these antibodies in pulmonary renal syndrome. Design. Retrospective clinical study. Setting. University Hospital. Subjects. Forty consecutive patients with biopsy verified glomerulonephritis and overt haemoptysis or pulmonary infiltrates compatible with lung haemorrhage. Interventions. Analysis of proteinase 3 antineutrophil cytoplasm antibodies (PR3‐ANCA), myeloperoxidase (MPO)‐ANCA, antiglomerular basement membrane (GBM) and anti‐entactin antibodies. Results. Thirty‐six (90%) patients possessed one or more autoantibodies. Twenty‐seven (70%) patients had ANCA (PR3‐ANCA, MPO‐ANCA or both). The remaining positive patients (n = 9) had anti‐GBM antibodies. Only two patients had anti‐entactin antibodies, suggesting a poor association of these antibodies with PRS. The majority of patients with anti‐GBM antibodies had a very poor clinical outcome (five irreversible renal failure; three deaths). On the other hand, despite no significant difference in clinical features or renal morphology from patients with anti‐GBM antibodies, 19 patients (70%) with ANCA recovered completely following treatment. Conclusions. Our study demonstrated that the presence of autoantibodies is a predominant feature of PRS and that the type of immunologic injury is of paramount importance in determining the course of illness in this syndrome. Analysis of the aforementioned antibodies can help in an early differential diagnosis and thus, in better management of PRS. 1995 Blackwell Publishing Ltd

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