Clinical features and predictors of osteoarticular manifestations in common variable immunodeficiency

• Published in: Clinical rheumatology Vol. 42; no. 11; pp. 3123 - 3129
• Main Authors: Ben Khaled, Monia, Merdassi, Amani, Rekaya, Samia, Fraj, Ilhem Ben, Lamouchi, Takwa, Zaiter, Ikram, Kouki, Ridha, Bejaoui, Mohamed, Mellouli, Fethi, Ouederni, Monia
• Format: Journal Article
• Language: English
• Published: Cham Springer International Publishing 01.11.2023; Springer Nature B.V
• Subjects: Body weight; Bone mineral density; Brief Report; CD4 antigen; Common variable immunodeficiency; Diarrhea; Etiology; Hematology; Immune system; Inflammation; Iron deficiency; Medicine; Medicine & Public Health; Multivariate analysis; Nutrient deficiency; Osteoporosis; Patients; Pediatrics; Rheumatology; Stem cell transplantation; Autoimmunity; Osteoporosis; Arthritis; Common variable immunodeficiency; Complications; Arthralgia
• ISSN: 0770-3198; 1434-9949
• DOI: 10.1007/s10067-023-06722-5

Introduction This study aimed to assess osteoarticular manifestations in patients with common variable immunodeficiency (CIVD) and to identify the predictive factors. Methods This was a retrospective and prognostic study conducted in the pediatrics: immuno-hematology and stem cell transplantation department, including patients who fit the definition of CVID. A Cox model analysis was used to identify predictive factors. Results A total of 36 patients were enrolled. Osteoarticular involvement was noted in 15 patients (42%) with a cumulative incidence of 90% after a median follow-up of 25 years. Non-infectious manifestations were reported in 14 patients (39%). The cumulative risk of inflammatory or autoimmune osteoarticular etiology was 74%. Well-characterized rheumatic diseases were retained in six patients and unlabeled autoimmune or inflammatory mechanism in five cases. Bone mineral density revealed osteoporosis in six cases leading to a cumulative risk of degenerative complications of 72%. The cumulative incidence of infectious complications was 17%. In multivariate analysis, predictors of osteoarticular complications were low body weight (HR = 8.67, CI: 1.496–50.278, p  = 0.01) and hepatomegaly at diagnosis (HR = 6.2, CI: 1.537–25.075, p  = 0.01). Reduced CD4 cells rate < 600 cells/mm 3 and hepatomegaly were predictors of autoimmune or inflammatory complications, while chronic diarrhea and iron deficiency were associated with degenerative manifestations. Conclusions Osteoarticular manifestations have emerged as a real health problem for CVID patients. Risk increases with low body weight, hepatomegaly, chronic diarrhea, iron deficiency, and CD4 cells rate under 600 cell/mm 3 . Elucidating the mechanisms of these complications in CVID is important for developing preventive strategies. Key Points • This retrospective and prognostic study described the clinical characteristics of osteoarticular manifestations in 36 patients with CVID to ensure better recognition and understanding of this association by clinicians. • Identification of predictive factors of osteoarticular complications according to its etiology is crucial to establish appropriate, optimal and early management of patients at risk.