RESUMO
PURPOSE: To describe an unusual case of Whipple's disease (WD) complicated by uveitis, and subsequent paradoxical worsening after effective antibiotic treatment targeting Tropheryma whipplei (TW). METHODS: Case report. RESULTS: A 53-year-old male presented with bilateral knee arthritis, weight loss, chronic low-grade fever, and cognitive disorders. He was under treatment with tumor necrosis factor α inhibitors (TNFi) for seronegative spondyloarthritis. Given this unusual clinical presentation, further investigations were performed and revealed blood, saliva, stool, synovial fluid and cerebrospinal fluid positivity for TW, confirming the diagnosis of systemic WD. Ophthalmologic examination revealed bilateral posterior uveitis and an aqueous humor sample confirmed the presence of intraocular TW. TNFi were stopped, and the patient was subsequently treated with adequate antibiotics (ceftriaxone, followed by doxycycline and hydroxychloroquine), and subconjunctival corticosteroid injections. After a transient improvement of the ocular symptoms, he presented a recurrence of posterior segment inflammation, leading to repeated PCR testing for TW which were negative. Therefore, paradoxical worsening of the inflammation in the context of immune recovery uveitis (IRU) was thought to be the culprit. The patient was treated with systemic corticosteroid therapy, allowing for rapid improvement of the ocular findings. CONCLUSIONS: This case underlines the possibility of IRU complicating WD. Ophthalmologists, rheumatologists, and internists should be aware of this rare complication, particularly in the context of previous immunosuppressive therapy.
RESUMO
PURPOSE: Bone fragility in sickle cell disease (SCD) has been previously reported even in young patients, but the clinical consequences and specific management remain unclear. The objective of this study was to assess the prevalence of bone fragility in sickle cell patients and to evaluate the potential risk factors and associated complications. METHODS: We conducted a single-center cross-sectional study. Bone mineral densitometry (BMD) at the lumbar spine and the hip, Vertebral Fracture Assessment (VFA) and biological measurements were performed in patients aged between 20 and 40 years. RESULTS: One hundred and thirty-eight patients with sickle cell disease were included between June 2020 and December 2021. One hundred and one patients (73.2 %) were from Sub-Saharan Africa, 13 from North Africa (9.4 %), 11 from the Caribbean (7.9 %), 6 from the Indian Ocean. A Z-score < -2 was found in 43 patients (31.2 %) at the lumbar spine, in 4 patients (3 %) at the total hip, and in 5 patients (3.7 %) at the femoral neck. 59 patients (46.8 %) had vertebral deformities. Fragility fractures were recorded in 9 patients (10.8 %). Patients with low BMD had lower BMI (21.3 (19.0, 24.0) versus 24.0 (20.7, 26.1) Kg/m2, p = 0.003), lower osteonecrosis history (7 % versus 25.3 %, p = 0.011) and lower hemoglobin levels (9.0 (8.0, 10.0) versus 10.0 (9.0, 11.0) g/dL, p < 0.01). No association was found between history of fracture and low BMD. CONCLUSION: Young patients with SCD commonly have low BMD at the lumbar spine, but the prevalence of fragility fracture was low. Low BMD - specifically at the spine - may not be tantamount to bone fragility.