Volume 6, Issue 4

Calcium pyrophosphate deposition (CPPD) disease is a common, age-related crystalline arthropathy with diverse clinical presentations. While the knees and wrists are most frequently affected, shoulder involvement is increasingly recognized, occurring in up to 13% of cases, though often underdiagnosed. This mini-review provides a comprehensive overview of the epidemiology, pathogenesis, clinical manifestations, diagnosis, and treatment of shoulder CPPD, contextualized by an illustrative case of a 78-year-old woman with atypical calcifications in the axillary recess and supraspinatus muscle. A key focus is the diagnostic challenge when synovial fluid analysis, the gold standard for crystal confirmation, is technically unfeasible, a common scenario in clinical practice. We systematically discuss modern imaging techniques (ultrasound, dual-energy computed tomography [CT], conventional radiography) and demonstrate the practical application of the 2023 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) classification criteria for establishing probable CPPD when crystal analysis is unavailable. The review addresses critical differential diagnosis considerations, particularly distinguishing CPPD from basic calcium phosphate (BCP) deposition disease, and summarizes evidence-based therapeutic strategies for acute pseudogout flares and chronic inflammatory arthritis, including emerging biologic therapies targeting the interleukin-1 (IL-1) pathway. This comprehensive resource aids clinicians managing shoulder calcifications in the absence of definitive crystal confirmation.

Objectives This study aimed to summarize the features of IgG4-related sclerosing mesenteritis (IgG4-SM) based on both our large prospective IgG4-related disease (IgG4-RD) cohort and literature-reported IgG4-SM cases. Methods We applied a mixed-methods approach, integrating data from 29 patients with IgG4-SM and 87 matched IgG4-RD patients without mesenteritis, all enrolled in a prospective cohort of IgG4-RD from Peking Union Medical College Hospital (PUMCH) since 2011. Additionally, we included 32 IgG4-SM cases reported in public databases since 2011. Demographic, clinical, laboratory, imaging and treatment data were systematically compared. Results Compared with IgG4-RD without mesenteritis, IgG4-SM predominantly affected elderly males and accompanied with more organ involvement ( P = 0.019) and elevated responder index (RI) scores and Physician Global Assessment (PGA) scores ( P = 0.005 and P = 0.018, respectively). Compared to the literature-reported cases, IgG4-SM patients in our cohort exhibited a lower prevalence of abdominal pain ( P < 0.001) but a higher frequency of submandibular gland ( P < 0.001) and lacrimal gland enlargement ( P < 0.001). Imaging features included a spectrum of mesenteric abnormalities, ranging from misty opacities to soft-tissue masses on computed tomography (CT), with significantly increased 18 F-fluorodeoxyglucose ( 18 F-FDG) uptake on positron emission tomography-computed tomography (PET-CT). Despite divergent treatments (glucocorticoids/immunosuppressants in our cohort vs . surgical resection in reported cases, P < 0.001), long-term relapse rates were comparable ( P = 0.17). Conclusions IgG4-SM is a significant but under-recognized manifestation of IgG4-RD, characterized by a predilection for elderly males, multi-organ involvement, higher disease activity and distinct imaging features. Systemic radiological evaluation, including PET-CT could assist diagnosis and treatment monitoring. Glucocorticoid-based therapy achieved outcomes comparable to surgery, challenging the necessity of invasive interventions. This study expands the clinical spectrum of IgG4-SM and advocates for personalized, non-surgical management strategies in most cases.

Background and Objectives Certain genetic traits increase the likelihood of developing rheumatoid arthritis (RA); nevertheless, the association between RA and polymorphisms in the CD209 gene is ambiguous. This study sought to investigate the correlation between RA susceptibility and the CD209 single nucleotide polymorphisms rs4804803 (AG) and rs735239 (AG) within the Egyptian population. Methods This case-control study was conducted between January and October 2024 and included 108 participants. Of these, 54 patients were diagnosed with RA according to the 2010 classification criteria of the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR). Two CD209 promoter regions rs4804803 (-336A/G) and rs735239 (-871A/G) were genotyped for single nucleotide polymorphisms (SNPs) using multiplex polymerase chain reaction (PCR) and double amplification refractory system (dARMS). Results Patients with RA showed significantly higher frequencies of the CD209 rs4804803 SNP in the AG ( P = 0.036) and GG ( P = 0.006) genotypes compared to controls. Under the dominant paradigm, those with CD209 rs4804803 genotypes (AG+GG) had an elevated risk of RA ( P = 0.003). In recessive inheritance model, RA patients had a greater frequency of the rs4804803 GG polymorphism than controls ( P = 0.017). Additionally, relative to the A allele, the rs4804803 G allele raised the risk of RA ( P = 0.001). No significant difference was observed between the two groups in the distribution of rs735239 genotypes. However, RA patients carrying the rs4804803 AG or GG genotypes exhibited significantly higher rates of morning stiffness ( P = 0.001) and elevated CRP levels ( P = 0.009). Conclusions This work highlights the significant role of the CD209 rs4804803 polymorphism, particularly the G allele, in the elevated susceptibility to RA among Egyptians.

Background and Objective Systemic lupus erythematosus (SLE) is a complex, heterogeneous autoimmune disease whose presentation can vary widely with patient age. While SLE in young adults has been extensively characterized, less is known about phenotypes of late-onset SLE. Methods This study aimed to characterize the features of late-onset SLE patients in a Chinese cross-sectional study. Patients diagnosed with SLE at age 50 years or older were classified as having late-onset SLE. Early-onset SLE patients from the same cohort were included as controls. Demographic, clinical, and laboratory data were collected, and a two-step cluster analysis was employed to categorize late-onset SLE patients. Results A total of 141 patients (27.48%) were classified as late-onset SLE. The onset of lupus in late-onset patients is more insidious, they exhibited lower systemic lupus erythematosus disease activity index-2000 (SLEDAI-2K) scores, and had significantly fewer instances of fever, mucocutaneous, and positive of antibodies compared to early-onset SLE (all P values < 0.05). However, late-onset SLE patients had a higher prevalence of comorbidities, particularly Sjögren’s syndrome ( P < 0.001). Additionally, late-onset SLE was associated with a high frequency of interstitial lung disease (ILD) and thrombotic events ( P < 0.001, P < 0.001; respectively). Two distinct clusters of late-onset SLE patients were identified. Cluster 1 was characterized by the presence of SLE-specific autoantibodies such as anti-double stranded DNA (anti-dsDNA), anti-Smith (anti-Sm) with higher SLEDAI-2K scores (11.8 ± 5.2). In contrast, cluster 2 presented with a high frequency of anti-Sjögren syndrome antigen A (anti-SSA) antibodies and Sjögren’s syndrome with a significantly lower SLEDAI-2K scores (8.8 ± 5.4) at diagnosis. Conclusion This study refines our understanding of late-onset SLE by delineating two subgroups and suggests that age-stratified approaches to diagnosis and management may improve patient care.