Table 1.
The clinical characteristics of patients with CVID and SLE.
| Clinical Phenotypes and Organ Complications in CVID Patients (n = 33) | |
|---|---|
| No disease-related complications | 9 (27%) |
| Bronchiectasis | 4 (12%) |
| Splenomegaly | 7 (21%) |
| Autoimmunity | 20 (60%) |
| Thrombocytopenia | 10 (33%) |
| Hemolytic anemia | 6 (18%) |
| Addison–Biermer disease | 2 (6%) |
| Vitiligo | 1 (3%) |
| Chronic seronegative polyarthritis | 2 (6%) |
| Alopecia areata | 1 (3%) |
| Nonspecific inflammatory bowel disease | 1 (3%) |
| Psoriasis | 3 (6%) |
| Polyclonal lymphocytic infiltration | |
| Generalized lymphadenopathy | 19 (57%) |
| Granulomatous lesions (histopathological confirmation) | 9 (27%) |
| Immunoglobulin replacement therapy | 29 |
| Immunoglobulin naïve | 3 |
| Prednisolone | 2; dose 5 mg/day |
| Methotrexate and etanercept | 1 |
| Rituximab in anamnesis | 2 |
| Clinical data of SLE patients (n = 17) | |
| SLEDAI2K | 3.6 (min 0–max 9) |
| Treatment | 16/17 |
| Prednisolone | 10 (58%); dose: 9 mg/day (min 5–max 15 mg) |
| Antimalarials | 16 (94%) |
| Immunosuppressive medication | |
| Methotrexate | 2 (11%) |
| Rituximab in anamnesis | 1 (5%) |
CVID: Common variable immunodeficiency, SLE: systemic lupus erythematosus, SLEDAI2K: Systemic Lupus Erythematosus Disease Activity Index 2000.