Diffuse alveolar hemorrhage associated with proteinase 3-specific anti-neutrophil cytoplasmic antibodies

M Kikawada; Y Ichinose; K Minemura; A Kunisawa; H Kusumoto; S Okabe; M Yonemaru; M Ogura; T Nakao; Y Ebihara; K Toyama

doi:10.2169/internalmedicine.36.430

Diffuse alveolar hemorrhage associated with proteinase 3-specific anti-neutrophil cytoplasmic antibodies

Intern Med. 1997 Jun;36(6):430-4. doi: 10.2169/internalmedicine.36.430.

Authors

M Kikawada¹, Y Ichinose, K Minemura, A Kunisawa, H Kusumoto, S Okabe, M Yonemaru, M Ogura, T Nakao, Y Ebihara, K Toyama

Affiliation

¹ First Department of Internal Medicine, Tokyo Medical College.

PMID: 9213192
DOI: 10.2169/internalmedicine.36.430

Abstract

A 31-year-old man was referred to our hospital for the management of progressive diffuse alveolar hemorrhage associated with renal dysfunction. Leukocytoclastic vasculitis was shown by skin biopsy and crescentic glomerulonephritis was also detected, in addition to positivity for proteinase 3-specific anti-neutrophil cytoplasmic antibodies (PR3-ANCA). The patient was diagnosed as a rare case of PR3-ANCA-positive pulmonary-renal vasculitic syndrome without granulomatous lesions. There was a good response to combination therapy with steroids and cyclophosphamide.

Publication types

Case Reports

MeSH terms

Adult
Antibodies, Antineutrophil Cytoplasmic / blood*
Autoantigens
Cyclophosphamide / therapeutic use
Glomerulonephritis / complications
Glomerulonephritis / diagnosis
Glomerulonephritis / drug therapy
Hemorrhage / diagnosis
Hemorrhage / drug therapy
Hemorrhage / immunology*
Humans
Lung Diseases / diagnosis
Lung Diseases / drug therapy
Lung Diseases / immunology*
Male
Myeloblastin
Pulmonary Alveoli
Serine Endopeptidases / immunology*
Steroids / therapeutic use
Syndrome
Vasculitis / diagnosis
Vasculitis / drug therapy
Vasculitis / immunology

Substances

Antibodies, Antineutrophil Cytoplasmic
Autoantigens
Steroids
Cyclophosphamide
Serine Endopeptidases
Myeloblastin