Pulmonary sarcoidosis associated with acquired humoral and cellular immunodeficiency.
A previously healthy 27-year-old man with class II pulmonary sarcoidosis developed severe humoral immunodeficiency within the course of the disease with an IgG of less than 250 mg/ml and undetectable levels of IgA and IgM. Repeated skin tests were negative for seven common recall antigens. Cellular blood test demonstrated normal numbers of B cells and slight T-cell lymphopenia with a normal T-helper/suppressor subset distribution (ratio 1.6). In contrast, parallel examination of the bronchial alveolar lavage fluid (BAL) demonstrated highly elevated numbers of T cells with a subset ratio of 3.1 and significant numbers of activated T cells as revealed by the expression of Ia and Tac antigens. Functional in vitro assays showed a greatly decreased mitogenic response of blood T cells and diminished production of immunoglobulins. These data indicate that, despite a severely depressed systemic humoral and cellular immune system, T-cell activation can take place at the inflammatory site, potentially causing the lesions characteristic of sarcoidosis.
Burmester
G R
GR
Gramatzki
M
M
von Gernler
J
J
Bartels
O
O
Kalden
J R
JR
D000328:Adult; D000917:Antibody Formation; D003876:Dermatitis, Atopic / Q000276:immunology; D006801:Humans; D017098:IgA Deficiency; D007111:Immunity, Cellular; D007074:Immunoglobulin G / Q000032:analysis; D007075:Immunoglobulin M / Q000172:deficiency; D007153:Immunologic Deficiency Syndromes / Q000150:complications / Q000276:immunology*; D008171:Lung Diseases / Q000150:complications*; D008297:Male; D008938:Mitosis; D010641:Phenotype; D012507:Sarcoidosis / Q000150:complications*; D012882:Skin Tests; D006377:T-Lymphocytes, Helper-Inducer / Q000276:immunology; D050378:T-Lymphocytes, Regulatory / Q000276:immunology
D007074:Immunoglobulin G; D007075:Immunoglobulin M