Axial involvement with facet joint arthropathy and bony ankylosis in a case of camptodactyly, arthropathy, coxa vara, pericarditis (CACP) syndrome

2013 ◽  
Vol 80 (5) ◽  
pp. 520-522 ◽  
Author(s):  
Yasser Emad ◽  
Yasser Ragab ◽  
Maher Khalifa ◽  
Iman Bassyouni ◽  
Nashwa El-Shaarawy ◽  
...  
2016 ◽  
Vol 27 (5) ◽  
pp. 801-805 ◽  
Author(s):  
Haku Iizuka ◽  
Yoichi Iizuka ◽  
Koichi Okamura ◽  
Yukio Yonemoto ◽  
Tokue Mieda ◽  
...  

Reumatismo ◽  
2015 ◽  
Vol 67 (2) ◽  
Author(s):  
Y. Ragab ◽  
M. Nabih ◽  
I. Aly ◽  
A. Kamal ◽  
M. A. Abd-Allah ◽  
...  

Hip disorders in a pediatric population are a diagnostic challenge. The aim of the study is to assess the role of magnetic resonance imaging (MRI) in the evaluation of non-traumatic hip disorders in a series of Egyptian patients and to review the literature on the most common hip conditions. Seventy two consecutive patients [40 males (55.6%) and 32 females (44.4)] with acute onset of hip complaints unrelated to trauma or falls were recruited. All patients underwent an initial full clinical assessment and blood tests as well as contrast enhanced MRI of both hips. The most common diagnosis in this group of Egyptian patients was transient synovitis in 29 (40.3%) cases, followed by seronegative enthesopathy and arthropathy syndrome in 8 (11.1%), septic arthritis in 10 (13.9%), tuberculous arthritis in 4 (5.6%), sickle-cell disease in 7 (9.7%), complicated with septic arthritis in 3 (4.2%), transient bone marrow microenvironment (BME) in 3 (4.2%), osteomyelitis in 2 (2.8%), osteosarcoma in 2 (2.8%), sciatic nerve injury in 1 (1.4%), leukemia with BME in 1 (1.4%), coxa vara of both hips and L5/S1 facet joint ankylosis in 1 (1.4%), and a benign bone cyst in 1 (1.4%). MRI studies showed hip effusion in a total of 51 patients (70.8%), joint space narrowing in 9 (12.5%), and BME in 15(20.8%). MRI is a sensitive tool for assessing hip disorders in a pediatric population and can play an important role in both diagnosis and management of different hip disorders, irrespective of the underlying pathology.


2009 ◽  
Vol 18 (8) ◽  
pp. 1130-1134 ◽  
Author(s):  
Haku Iizuka ◽  
Masahiro Nishinome ◽  
Yasunori Sorimachi ◽  
Tsuyoshi Ara ◽  
Takashi Nakajima ◽  
...  

2007 ◽  
Vol 46 (01) ◽  
pp. 38-42 ◽  
Author(s):  
V. Schulz ◽  
I. Nickel ◽  
A. Nömayr ◽  
A. H. Vija ◽  
C. Hocke ◽  
...  

SummaryThe aim of this study was to determine the clinical relevance of compensating SPECT data for patient specific attenuation by the use of CT data simultaneously acquired with SPECT/CT when analyzing the skeletal uptake of polyphosphonates (DPD). Furthermore, the influence of misregistration between SPECT and CT data on uptake ratios was investigated. Methods: Thirty-six data sets from bone SPECTs performed on a hybrid SPECT/CT system were retrospectively analyzed. Using regions of interest (ROIs), raw counts were determined in the fifth lumbar vertebral body, its facet joints, both anterior iliacal spinae, and of the whole transversal slice. ROI measurements were performed in uncorrected (NAC) and attenuation-corrected (AC) images. Furthermore, the ROI measurements were also performed in AC scans in which SPECT and CT images had been misaligned by 1 cm in one dimension beforehand (ACX, ACY, ACZ). Results: After AC, DPD uptake ratios differed significantly from the NAC values in all regions studied ranging from 32% for the left facet joint to 39% for the vertebral body. AC using misaligned pairs of patient data sets led to a significant change of whole-slice uptake ratios whose differences ranged from 3,5 to 25%. For ACX, the average left-to-right ratio of the facet joints was by 8% and for the superior iliacal spines by 31% lower than the values determined for the matched images (p <0.05). Conclusions: AC significantly affects DPD uptake ratios. Furthermore, misalignment between SPECT and CT may introduce significant errors in quantification, potentially also affecting leftto- right ratios. Therefore, at clinical evaluation of attenuation- corrected scans special attention should be given to possible misalignments between SPECT and CT.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1633.1-1633
Author(s):  
D. Cici ◽  
C. Rotondo ◽  
A. Corrado ◽  
S. Berardi ◽  
N. Mansueto ◽  
...  

Background:Occasional findings of anti-citrullinated-protein-antibodies (ACPA) and anti-nuclear-antibodies (ANA) were rarely described in literature on Psoriatic Arthritis (PsA) and on Spondyloarthritis (SpA) in general. How these autoimmune dysregulations can affect the course of them is not yet understood.Objectives:The aim of our study is to evaluate if the presence of ACPA and ANA can determine different disease subsets and influence the DMARDs monotherapy (methotrexate) drug survival (DSM) and b-DMARDs multi-failure patients (MF).Methods:We conducted a retrospective study on patients with Psoriatic Arthritis (PsA) and Spondyloarthritis that fulfilled the ASAS and CASPAR criteria. Patients with diagnosis of connective tissue disease and rheumatoid arthritis and patients ≤ 18 years old were excluded from the study. For each patient, the following variables were considered: age, ACPA, ANA, time between arthritis onset and start of DMARDs (start-time), DSM, switch to b-DMARDs (sw-bDMARDs), arthritis subset (oligoarticular (OA), polyarticular (PA), enthesitis (EA), axial involvement (AI)), number of comorbidities (NC), Charlson Comorbidity Index (CCI).Results:150 patients (55% with PsA and 45% with another SpA) were included in the study. No differences were found in age, ANA rate, ACPA rate, start-time, OA, PA, EA, AI, NC and CCI between the PsA and SpA groups.In the whole group of patients, the ACPA+ subjects(11%) had a significant increase of NC (2.47 ± 1.5 vs 1.6 ± 1.4, p=0.035), a trend to higher CCI, to switch to b-DMARDs, and to be MF compared to those without ACPA. In the same group, the ANA+ patients (12%) showed shorter DSM (233.5 wk ± 45.9 vs 548.0 wk ± 56.8, p=0.362) with similar trend in each subgroup (PsA and SpA).In SpA group, the ACPA+ patients(6,3%) had a trend to shorter DSM (269.0 weeks ± 125vs 603.96 wk± 92.8, p=0.492),to higher sw-bDMARDs, and to be MF, higher NC and CCI compared to those without ACPA. No differences in clinical subset (OA, PA, EA, AI) were observed. In the same group the ANA+ patients had significant higher rate of PA (100% vs 65%, p=0.026) rather than OA (0% vs 35%, p=0.025). No significant differences were found in NC, CCI, MF.In the PsA group, ACPA+ patients showed a trend to develop PA and EA subsets, shorter DSM (187.5 wk ± 48.7 vs 299.6 wk ± 31.4, p=0.415), higher rate to sw-bDMARDs and to be MF. The ANA+ PsA patients had higher trend to develop PA and AI subsets rather than OA and EA. All ANA+ patients were MF (100% vs 42%, p=0.046).Conclusion:The ACPA and ANA positivity in PsA and SpA patients could be suggestive of more severe clinical disease manifestation, higher frequency of comorbidities and lower predicted 10-year survival (CCI). Moreover, this autoimmune dysregulation could be associated with worse drug survival in monotherapy with methotrexate and higher chance to be MF. Therefore, they can be taken into account for clinical management of these patients.Disclosure of Interests:None declared


2021 ◽  
pp. 1-6
Author(s):  
Rabia Miray Kisla Ekinci ◽  
Sibel Balci ◽  
Haldun Dogan ◽  
Serdar Ceylaner ◽  
Celal Varan ◽  
...  

Camptodactyly-arthropathy-coxa vara-pericarditis (CACP) syndrome, caused by biallelic pathogenic mutations in the <i>PRG4</i> gene, is characterized by early-onset camptodactyly, noninflammatory arthropathy, coxa vara deformity, and rarely, pericardial effusion. Herein, we report 3 patients with CACP syndrome from 2 unrelated families. All patients are female, born to consanguineous parents, and had camptodactyly since the first years of their lives. Two patients had a prior diagnosis of juvenile idiopathic arthritis. Hip changes were present in 2 patients, and 2 of 3 patients had undergone surgery for camptodactyly. Routine echocardiographic evaluations were normal during the 2-year follow-up. This paper represents the third study including CACP patients from Turkey. Clinically, all 3 patients resembled juvenile idiopathic arthritis cases and received unnecessary medication. There is also an ongoing need for improving awareness of CACP and an effective treatment focusing on the lubrication of the joint space in CACP patients.


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