Idiopathic CD4+ lymphocytopenia and systemic vasculitis.
The syndrome defined as "idiopathic CD4 lymphocytopenia' (ICL) is a rare disease of unknown aetiology, often associated with severe depression of immune defences and the occurrence of opportunistic infections. A case is reported wherein a severe immunodeficiency syndrome with persistent idiopathic CD4+ lymphopenia developed in a woman suffering from systemic microscopic polyarteritis; no signs of HIV 1/2 or HTLV I/II infection were evident. The patient died of widespread opportunistic infections. The association of ICL with vasculitis has never been reported until now. A link between the two diseases cannot be ruled out.
Bordin
G
G
II Divisione di Medicina Generale, Ospedale Maggiore, Novara, Italy.
Ballaré
M
M
Paglino
S
S
Ravanini
P
P
Dulio
D
D
Malosso
M C
MC
Boldorini
R
R
Monteverde
A
A
D000368:Aged; D001323:Autoantibodies / Q000032:analysis; D003601:Cytotoxicity Tests, Immunologic; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D009894:Opportunistic Infections / Q000150:complications* / Q000276:immunology; D010488:Polyarteritis Nodosa / Q000150:complications* / Q000276:immunology / Q000473:pathology; D018344:T-Lymphocytopenia, Idiopathic CD4-Positive / Q000150:complications* / Q000276:immunology / Q000473:pathology
D001323:Autoantibodies