Tehran University Medical Journal

Primary Immune Deficiencies are a group of heterogeneous disorders that involve the innate or acquired immune system, or a combination of them. The underlying disorder may be related to decreased levels or function, or a complete lack of one or more components of the immune system in general. These diseases can occur with a prevalence of about 1 in 10000 live births. According to the fourth update on the Iranian national registry of Primary Immune Deficiency in October 2018, the total number of registered PIDs in Iran are 3056 patients. However, it is supposed to be more prevalent and it seems increasing awareness shall reveal many new cases, especially in societies with prevalent consanguineous marriages like Iran. These disorders predispose patients to recurrent infections, autoimmunity and malignancy and can cause a huge burden on health care systems. This group of diseases has a wide range of symptoms, which quick recognition and timely treatment of them, can greatly reduce the complications of the disease. These symptoms may include recurrent or severe infections, failure to thrive, autoimmune disorders, as well as articular-skeletal manifestations. A variety of skeletal manifestations are seen in patients with primary immunodeficiency, among which septic arthritis caused by pyogenic bacteria or mycoplasma arthritis is the most common joint-bone manifestation. Joint and skeletal involvement is less commonly seen as a sign of primary immune defects. This issue is importance in reducing the cost of diseases and improving the patients’ quality of life. Our review attempted to introduce the most common manifestations of bone and joint in patients with primary immunodeficiency and available treatments for these manifestations. Because of the wide range of symptoms in these patients, it is recommended to observe the rare and suspicious manifestations in the patients with any atypical bone and joint presentations such as: recurrent septic arthritis, infection with unusual germs, immunodeficiency in their relatives, and any history of well-known red flags of PIDs. The Rheumatologist should consider these manifestations and think about the possibility of deficiency disorder.

Background: This cross-sectional study aims to evaluate the association between patellofemoral joint alignment measurements and patellofemoral cartilage defects using MRI images.
Methods: A retrospective and cross-sectional study was conducted on 530 knee MRI images from Golestan and Imam Khomeini hospitals in Ahvaz, Khuzestan, Iran. The images were obtained from individuals experiencing non-traumatic knee pain and were selected based on their age, with subjects over 30 years of age being included in the study. This survey was conducted over the period from March 2023 to March 2024. The parameters of femoral groove angle (SA), femoral groove depth (SD), lateral patellofemoral angle (LPFA), tibial-trochlear tubercle groove distance (TT-TG), lateral patellar displacement (LPD), patellar inclination angle, and patellar height were evaluated. In addition, the Caton-Deschamps index and the degree of focal cartilage defect (from 0 to III) in the knee joint were evaluated.

Results: Regarding body mass index, 15.7% of the patients studied were underweight, 42.3% were of normal weight, 31.1% were overweight, 10.4% were obese, and 0.6% were extremely obese. There was a significant relationship between body mass index and patellar-lateral articular cartilage defects in the lateral tibia and lateral femur. There was a significant relationship between age and patellar-lateral articular cartilage defects in the lateral femur. There was a significant relationship between lateral patellar articular cartilage defects and lateral patellar dislocation, lateral patellofemoral angle, tibial prominence-skeletal groove distance, and patellar inclination angle. In addition, there was a significant relationship between medial patellar articular cartilage defects and knee groove angle. Conclusion: Patellofemoral misalignment is multivariately associated with knee articular cartilage defect. The results of this study will be of use to medical professionals, and the early diagnosis of knee osteoarthritis can prevent the development of the condition. It is recommended that similar studies and surveys be conducted in other provinces of Iran so that the statistical population is highly vulnerable.