Analysis of five cases of monogenic lupus related to primary immunodeficiency diseases.
Inflamm Res
; 70(10-12): 1211-1216, 2021 Dec.
Article
en En
| MEDLINE
| ID: mdl-34559261
ABSTRACT
OBJECTIVE AND DESIGN:
We studied five cases of PID-related monogenic lupus to explore the characteristics. MATERIAL ORSUBJECTS:
Among 42 cases of PID patients between 2017-2020, 5 patients were diagnosed as PID-related monogenic lupus, including 2 males and 3 females, with age range from 2 years 3 months to 13 years old. TREATMENTS DMARDs, biological agents and stem cell transplantation were used to treat different patients.METHODS:
We collected the clinical observation indicators, auxiliary examination and treatment of the five patients.RESULTS:
Patient 1 was diagnosed with monogenic lupus secondary to severe combined immunodeficiency and received prednisone and methotrexate treatment. Patient 2 was diagnosed with monogenic lupus secondary to activated phosphoinositide 3-kinase δ syndrome. Allogeneic stem cell transplantation was conducted. Patient 3 was diagnosed with monogenic lupus secondary to RAS-associated lymphoproliferative disease. The child was treated with prednisone and rituximab. Patient 4 was diagnosed with monogenic lupus secondary to PSTPIP1-associated myeloid-related proteinaemia inflammatory syndrome. The child was given methylprednisolone, methotrexate, and infliximab. Patient 5 was diagnosed with monogenic lupus secondary to A20 haploinsufficiency. The child was treated with methylprednisolone and infliximab.CONCLUSIONS:
Multiple PIDs can lead to monogenic lupus. Different PID-related monogenic lupus has different suitable targeted drugs.Palabras clave
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Enfermedades Autoinmunes
/
Enfermedades de Inmunodeficiencia Primaria
Tipo de estudio:
Diagnostic_studies
Límite:
Adolescent
/
Child
/
Child, preschool
/
Female
/
Humans
/
Male
Idioma:
En
Revista:
Inflamm Res
Asunto de la revista:
ALERGIA E IMUNOLOGIA
/
PATOLOGIA
Año:
2021
Tipo del documento:
Article
País de afiliación:
China