High Rate of Autoimmune Manifestations During Idiopathic CD4 Lymphocytopenia

Alexis Régent¹, Brigitte Autran², Guislaine Carcelain², Benjamin Terrier³, Alain Krivitzky⁴, Eric Oksenhendler⁵, Nathalie Costedoat-Chalumeau⁶, Pascale Hubert², Olivier Lortholary⁷, Nicolas Dupin⁸, Patrice Debré², Loic Guillevin⁹ and Luc Mouthon¹, ¹Internal Medicine, Hopital Cochin, Paris, France, ²INSERM, UMR945, AP-HP, Hôpital Pitié-Salpêtrière, Laboratoire d'Immunologie Cellulaire Et Tissulaire, Paris, France, ³National Referral Center for Rare Systemic Autoimmune Diseases, Cochin Hospital, Paris, France, ⁴Département de médecine interne, Hôpital Avicenne, AP-HP, Bobigny, France, ⁵Département d'Immunologie Clinique, Hôpital Saint-Louis, AP-HP, Paris, France, ⁶Internal Medicine, Assistance Publique-Hôpitaux de Paris, Hopital Pitié-Salpétrière, Paris, France, ⁷Service de maladies infectieuses, Hôpital Necker-Enfants malades, AP-HP, Paris, France, ⁸Service de Dermatologie, Hôpital Cochin, AP-HP, Paris, France, ⁹Department of Internal Medicine, Referral Center for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, AP–HP, Université Paris Descartes, Paris, France, Paris, France

Meeting: 2012 ACR/ARHP Annual Meeting

Keywords: Auto-immunity, B cells, infection and morbidity and mortality, T cells

Session Information

Title: Miscellaneous Rheumatic and Inflammatory Diseases: Periodic Fever Syndromes

Session Type: Abstract Submissions (ACR)

Background/Purpose: When first described by the Center for Disease Control, idiopathic CD4 lymphocytopenia (ICL) was characterized by opportunistic infections in patients with a CD4 count≤300/mm³ or ≤20% lymphocytes. It is now well established that auto-immune manifestations occur during primary or secondary immunodeficiencies.

Methods: We prospectively included 36 patients (21 females) with ICL between January 1991 and June 2011. T lymphocyte phenotyping and lymphocyte proliferation assay were realized at diagnosis. Infectious, autoimmune manifestations and malignancies during a mean follow-up of 7.6±6.7 years were recorded and correlated with data at inclusion.

Results:

Twenty four patients showed infections (11 with human papillomavirus infection), 11 autoimmune symptoms, 5 malignancies and 7 mild or no symptoms. Autoimmune and/or inflammatory manifestations include immune thrombocytopenic purpura (n=5), autoimmune hemolytic anemia (n=3), central nervous system vasculitis (n=1), Goodpasture syndrome (n=1), grade II duodenal villous atrophy (n=1), Crohn disease (n=1), antiphospholipid syndrome (n=1) and Hashimoto’s thyroiditis (n=1). At the time of diagnosis, mean CD4⁺, CD8⁺, CD19⁺ and natural killer (NK) cell counts were 126/mm3 (range 4–294), 238/mm3 (1–1293), 107/mm3 (3–547) and 115/mm3 (5–416), respectively. Most patients exhibited deficiency in CD8⁺, CD19⁺, and/or NK cells. Patients with infections had a significantly lower NK cell count (p=0.03) and those with autoimmune manifestations higher CD45RO⁺CD8⁺ count (p=0.02). T-cell proliferation induced by mitogens and antigens revealed great discrepancies. Six patients died (16.6%) during follow-up. CD4⁺ T-cell count < 150/mm3 and NK cell count <100/mm³ were predictors of death.

Conclusion:

30.5 % patients with ICL show auto-immune manifestations during clinical course. These patients had higher CD45RO⁺CD8⁺ count at diagnosis. In addition to CD4 lymphocytopenia, patients often have CD8, CD19 and NK cell defects. Mortality is related to an initial CD4⁺ count≤ 150/mm³ and NK cells≤100/mm³.

Disclosure:

A. Régent,
None;

B. Autran,
None;

G. Carcelain,
None;

B. Terrier,
None;

A. Krivitzky,
None;

E. Oksenhendler,
None;

N. Costedoat-Chalumeau,
None;

P. Hubert,
None;

O. Lortholary,
None;

N. Dupin,
None;

P. Debré,
None;

L. Guillevin,
None;

L. Mouthon,
None.

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