Arthritis Cartilage

Parry-Romberg syndrome (PRS) is an uncommon disorder and characterized by a slowly, an acquired progressive atrophy involving skin, soft tissue, cartilage, and bony structures. Accompanying atrophies of the other parts of the body are rarely reported.

The aim of this study is to report a case that had contralateral lower extremity atrophy with PRS, and to review the related etiologic features, physiopathology, and mechanism.The patient who admitted for his facial atrophy also had atrophy of his contralateral extremity. This extremity was also short in length when compared with other extremity.

To obtain detailed information regarding the severity of involvement routine laboratory investigations including antinuclear antibody (ANA), magnetic resonance imagine (MRI) of the craniofacial region and lower extremities, MR angiography of the lower extremities and brain, 3-dimension computed tomography (CT) scan of the craniofacial region were performed.

Normal or negative laboratory findings included results of blood count, renal and hepatic function biochemical tests, rheumatoid factor, C-reactive protein, anti-dsDNA antibody except ANA that were positive. His lower extremity and brain MR angiography were normal. In the 3-dimensional cranial CT, there was no abnormality or defect in the bony structures.

His brain MRI showed no pathologic changes, and his facial MRI demonstrated noteworthy atrophy to the sternocleidomastoid, masseter, pterygoid muscles, and subcutaneus soft tissues on the right side of the face. Additionally, MR investigation of his lower extremities revealed decreased volume in muscles and bony structures of the effected extremity compared with the other extremity, but pathologic evidence of fatty degeneration associated with muscle atrophy was not demonstrated.

The patient had isolated contralateral lower extremity involvement combined with hemifacial atrophy (without affecting any other part of the body). Although more accepted theory is the sympathic nervous system dysfunction, autoimmunity may play a roll in the etiology of our case as ANA abnormality was found in multiple tests.



Parry-Romberg Syndrome: Facial Atrophy and Relationship With the Other Part of the Body Involvement.
Ann Plast Surg. 2009 Sep 4; Duymaz A, Karabekmez FE, Keskin M, Tosun Z

Based on recent analyses, the measures of short-term responsiveness of magnetic resonance imaging (MRI) derived cartilage morphometry may not be as large as earlier studies had suggested. We examined if by selecting regions of interest with denuded cartilage, the remaining cartilage within this region of interest was susceptible to greater rates of cartilage loss.

METHODS:
Subjects included for this analysis are a subset of the approximately 4700 participants in the Osteoarthritis Initiative (OAI) Study. Bilateral radiographs and 3T MRI (Siemens Trio) of the knees and clinical data are obtained at baseline and annually in all participants.

Hundred and fifty subjects from the OAI progression subcohort all of whom had both frequent symptoms and, in the same knee, radiographic osteoarthritis (ROA defined as definite tibio-femoral osteophytes on X-ray) based on a screening reading done at the OAI clinics. One knee from each subject was selected for analysis.

Using sagittal 3D DESSwe MR images from the baseline and 12 follow-up month visit, a segmentation algorithm was applied to the cartilage plates of the index knee to compute the cartilage volume, normalized cartilage volume (volume normalized to bone surface interface area), and percent denuded area (Total Cartilage Bone Interface area denuded of cartilage). Summary statistics of the changes (absolute and percentage) from baseline at 1 year and the standardized response mean (SRM), i.e., mean change divided by the standard deviation (SD) of that change were calculated.

Analyses are stratified into three groups according to baseline assessment of denuded area: those with no denuded area in the region of interest at baseline, and then two groups (intermediate denuded area (/=median) denuded area) of equal sample size.

RESULTS:
On average the subjects were 60.9 years of age and obese with a mean body mass index (BMI) of 30.3kg/m(2). For the combined central medial femur and tibia the mean volume change for the whole sample was -48.2 (SD 159.8) mm(3), which gives an SRM of -0.30. In the subsample of knees with no denuded area the SRM was -0.25, in the knees with intermediate denuded area the SRM was -0.30, and in knees with severe denuded area the SRM was -1.00.

For normalized volume of the central medial femur in the subsample of knees with no denuded area the SRM was -0.22, in the knees with intermediate denuded area the SRM was -0.26, and in knees with severe denuded area (n=23) the SRM was -0.71. The magnitude of the SRMs was generally smaller in participants with no denuded area. In contrast, the SRMs in participants with denuded area were larger.

CONCLUSION:
By selecting participants with the presence of cartilage regions with denuded area the ability to demonstrate change in cartilage loss in that specific location is markedly improved compared to persons without a full thickness lesion in that cartilage plate. This option for screening during recruitment in clinical trials could facilitate the detection of participants at greater risk of subsequent cartilage loss.


Region of interest analysis: by selecting regions with denuded areas can we detect greater amounts of change?
Osteoarthritis Cartilage. 2009 Aug 29; Hunter DJ, Li L, Zhang YQ, Totterman S, Tamez J, Kwoh CK, Eaton CB, Hellio Le Graverand MP, Beals CR,

Abstract The aim of this study was to record growth-related changes in collagen network organization and proteoglycan distribution in intermittently peak-loaded and continuously lower-level-loaded articular cartilage.

Cartilage from the proximal phalangeal bone of the equine metacarpophalangeal joint at birth, at 5, 11 and 18 months, and at 6-10 years of age was collected from two sites. Site 1, at the joint margin, is unloaded at slow gaits but is subjected to high-intensity loading during athletic activity; site 2 is a continuously but less intensively loaded site in the centre of the joint.

The degree of collagen parallelism was determined with quantitative polarized light microscopy and the parallelism index for collagen fibrils was computed from the cartilage surface to the osteochondral junction. Concurrent changes in the proteoglycan distribution were quantified with digital densitometry.

We found that the parallelism index increased significantly with age (up to 90%). At birth, site 2 exhibited a more organized collagen network than site 1. In adult horses this situation was reversed. The superficial and intermediate zones exhibited the greatest reorganization of collagen.

Site 1 had a higher proteoglycan content than site 2 at birth but here too the situation was reversed in adult horses. We conclude that large changes in joint loading during growth and maturation in the period from birth to adulthood profoundly affect the architecture of the collagen network in equine cartilage.

In addition, the distribution and content of proteoglycans are modified significantly by altered joint use. Intermittent peak-loading with shear seems to induce higher collagen parallelism and a lower proteoglycan content in cartilage than more constant weight-bearing.

Therefore, we hypothesize that the formation of mature articular cartilage with a highly parallel collagen network and relatively low proteoglycan content in the peak-loaded area of a joint is needed to withstand intermittent stress and shear, whereas a constantly weight-bearing joint area benefits from lower collagen parallelism and a higher proteoglycan content.



Changes in collagen fibril network organization and proteoglycan distribution in equine articular cartilage during maturation and growth.
J Anat. 2009 Aug 27; Hyttinen MM, Holopainen J, René van Weeren P, Firth EC, Helminen HJ, Brama PA

The gamma isoform of phosphoinositide 3-kinase (PI3Kgamma) has been viewed as restricted to leukocytes mediating the regulation of chemokine-induced migration and recruitment of neutrophils, monocytes, and macrophages.

In line with the observation that PI3Kgamma-deficient mice display defects in adaptive immunity, inhibition of PI3Kgamma reduces synovial inflammation in the collagen-induced arthritis mouse model of inflammatory arthritis [rheumatoid arthritis (RA)], which has been attributed to reduced influx of inflammatory cells.

Challenging the concept of leukocyte-restricted PI3Kgamma function, we report here a novel, nonredundant function of PI3Kgamma as an important regulator of fibroblast-induced cartilage destruction during chronic destructive arthritis.

We show that in human tumor necrosis factor transgenic mice, the loss of PI3Kgamma leads to a milder inflammatory arthritis. Interestingly, PI3Kgamma deficiency does not alter the recruitment of inflammatory cells, but significantly reduces cartilage damage through reduced expression of matrix metalloproteinases in fibroblasts and chondrocytes.

In vitro analyses demonstrate that the decreased invasiveness of fibroblasts is mediated by reduced phosphorylation of Akt and extracellular signal-regulated kinase. Using a PI3Kgamma specific inhibitor, these data are confirmed in human synovial fibroblasts from patients with RA who exhibit a disease-specific up-regulation of PI3Kgamma.

Our data indicate that in addition to mediating the recruitment of inflammatory cells, PI3Kgamma is an important regulator of fibroblast-mediated joint destruction in RA and suggest that specific inhibitors of PI3Kgamma will interfere with the activation of RA synovial fibroblasts and reduce cartilage destruction in RA.-Hayer, S., Pundt, N., Peters, M. A., Wunrau, C., Kühnel, I., Neugebauer, K., Strietholt, S., Zwerina, J., Korb, A., Penninger, J., Joosten, L. A. B., Gay, S., Rückle, T., Schett, G., Pap, T. Phosphatidylinositol 3-kinase-gamma regulates cartilage damage in chronic inflammatory arthritis.




PI3K{gamma} regulates cartilage damage in chronic inflammatory arthritis.
FASEB J. 2009 Sep 4; Hayer S, Pundt N, Peters MA, Wunrau C, Kühnel I, Neugebauer K, Strietholt S, Zwerina J, Korb A, Penninger J, Joosten LA, Gay S, Rückle T, Schett G, Pap T

Mod Pathol. 2009 Sep 4; Rozeman LB, de Bruijn IH, Bacchini P, Staals EL, Bertoni F, Bovée JV, Hogendoorn PCDedifferentiated peripheral chondrosarcoma is a rare subtype of chondrosarcoma arising superimposed on the cartilage cap of a preexisting osteochondroma. It consists of two clearly defined components, a low-grade malignant, well-differentiated cartilage component and a high-grade non-cartilaginous sarcoma. Signaling pathways having a role in normal cartilage development were analyzed in these tumors, and compared with available data of other cartilaginous tumors. Sixteen well-characterized dedifferentiated peripheral chondrosarcomas were immunohistochemically analyzed for parathyroid hormone-like hormone (PTHLH)-BCL-2, fibroblastic growth factor (FGF), and transforming growth factor-beta signaling molecules, as well as matrix molecules and p53, comparing the chondrogenic component of dedifferentiated peripheral chondrosarcomas with the anaplastic component and with previously published data obtained from conventional grade I and II secondary peripheral chondrosarcomas. Results were correlated with clinical outcome. In the anaplastic component, various lines of differentiation could be found (collagen I (6/16), CD31 (1/16), smooth muscle actin (12/16), muscle-specific actin (12/16) and desmin (2/9)). Compared with the anaplastic component, the chondrogenic component of dedifferentiated peripheral chondrosarcomas shows more often expression of cyclin D1 (P=0.05), p53 (P=0.008), plasminogen activator inhibitor 1 (PAI-1) (P=0.005), and CD44 (P=0.030). Compared with secondary peripheral chondrosarcomas, more samples were positive in the chondrogenic component of dedifferentiated peripheral chondrosarcomas for FGF signaling (FGF receptor 3 P=0.000; bFGF P=0.003) and CD44 (P=0.000). Lower expression of BCL-2 (P=0.025) and absence of CD44v3 (P=0.000), a splice variant of CD44, was observed in the chondrogenic component of dedifferentiated peripheral chondrosarcomas compared with secondary peripheral chondrosarcomas. With regard to clinical data, PAI-1 expression in the chondrogenic component of dedifferentiated peripheral chondrosarcomas correlated with better survival (P=0.019). In conclusion, in the chondrogenic component of dedifferentiated peripheral chondrosarcomas, FGF signaling pathway is active, whereas PTHLH signaling seems to be low/downregulated. Interestingly, although the chondrogenic component of dedifferentiated peripheral chondrosarcoma is CD44+/CD44v3-, secondary peripheral chondrosarcomas is CD44-/CD44v3+, which suggest different splicing (preference). The prognostic value of PAI-1 in dedifferentiated peripheral chondrosarcomas might also be of interest for the more common dedifferentiated central chondrosarcomas.Modern Pathology advance online publication, 4 September 2009; doi:10.1038/modpathol.2009.120.

Skeletal Radiol. 2009 Sep 3; Krug DK, Vinson EN, Helms CAOBJECTIVE: Luxatio erecta humeri is a rare type of inferior glenohumeral dislocation with a unique radiographic appearance; however, the magnetic resonance imaging findings associated with this dislocation have not been described in the radiology literature. The purpose of this study is to identify magnetic resonance imaging findings associated with this uncommon type of glenohumeral dislocation. MATERIALS AND METHODS: The magnetic resonance imaging features of four patients with clinical and radiographic evidence of luxatio erecta humeri were reviewed retrospectively by two musculoskeletal-trained radiologists. The reported mechanism of injury in all four patients was falling. The MR imaging examinations were evaluated for the presence of rotator cuff and biceps tendon pathology, glenoid labrum pathology, joint capsule and glenohumeral ligament injury, fractures and bone marrow contusions, articular cartilage injury, and joint effusions. RESULTS: All four patients demonstrated pathology of the glenohumeral joint. Three of the four patients demonstrated rotator cuff tears, including large full thickness tears of the supraspinatus and infraspinatus tendons in two patients, and small full thickness tear of the supraspinatus tendon with partial thickness tear of the infraspinatus tendon in the third patient. In the two patients with large full thickness tears of the supraspinatus and infraspinatus tendons, one patient demonstrated tearing of the subscapularis tendon with dislocation of a partially torn long head of the biceps tendon, and the second patient demonstrated full thickness tearing of the intra-articular biceps tendon. All four patients demonstrated injuries to the glenoid labrum and both anterior and posterior bands of the inferior glenohumeral ligament. Contusions or fractures of the humeral head were seen in two of the patients. Three of the four patients demonstrated cartilage abnormalities including a focal cartilage defect in the anterior inferior glenoid in one patient, and cartilage surface irregularity of the glenoid in the other two patients. CONCLUSION: Common magnetic resonance imaging findings in patients with a prior luxatio erecta humeri dislocation include rotator cuff tears, injury to the glenoid labrum, and injury to both the anterior and posterior bands of the inferior glenohumeral ligament. These findings are compatible with the mechanism of dislocation in luxatio erecta, and noting these findings on magnetic resonance imaging may suggest that the patient has sustained a prior inferiorly directed glenohumeral dislocation such as luxatio erecta.

Zhongguo Xiu Fu Chong Jian Wai Ke Za Zhi. 2009 Aug; 23(8): 1002-6Liu T, Tan B, Luo J, Deng L, Xie HOBJECTIVE: To explore the method of preparing spongy and porous scaffold materials with swine articular cartilage acellular matrix and to investigate its applicability for tissue engineered articular cartilage scaffold. METHODS: Fresh swine articular cartilage was freeze-dried and freeze-ground into microparticles. The microparticles with diameter of less than 90 microm were sieved and treated sequentially with TNE, pepsin and hypotonic solution for decellularization at cryogenic temperatures. Colloidal suspension with a mass/volume ratio of 2% was prepared by dissolving the microparticles into 1.5% HAc, and then was lyophilized for molding and cross-linked by UV radiation to prepare the decellularized cartilage matrix sponge. Physicochemical property detection was performed to identify aperture, porosity and water absorption rate. Histology and scanning electron microscope observations were conducted. The prepared acellular cartilage matrix sponge was implanted into the bilateral area of spine in 24 SD rats subcutaneously (experimental group), and the implantation of Col I sponge served as control group. The rats were killed 1, 2, 4, and 8 weeks after operation to receive histology observation, and the absorption and degeneration conditions of the sponge in vivo were analyzed. BMSCs obtained from femoral marrow of 1-week-old New Zealand white rabbits were cultured. The cells at passage 3 were cultured with acellular cartilage matrix sponge lixivium at 50% (group A), acellular cartilage matrix sponge lixivium at 100% (group B), and DMEM culture medium (group C), respectively. Cell proliferation was detected by MTT method 2, 4, and 6 days after culture. RESULTS: The prepared acellular cartilage matrix sponge was white and porous. Histology observation suggested that the sponge scaffold consisted primarily of collagen without chondrocyte fragments. Scanning electron microscope demonstrated that the scaffold had porous and honeycomb-shaped structure, the pores were interconnected and even in size. The water absorption rate was 20.29% +/- 25.30%, the aperture was (90.66 +/- 21.26) microm, and the porosity of the scaffold was 90.10% +/- 2.42%. The tissue grew into the scaffold after the subcutaneous implantation of scaffold into the SD rats, angiogenesis was observed, inflammatory reaction was mild compared with the control group, and the scaffold was degraded and absorbed at a certain rate. MTT detection suggested that there were no significant differences among three groups in terms of absorbance (A) value 2 and 4 days after culturing with the lixivium (P > 0.05), but significant differences were evident among three groups 6 days after culturing with the lixivium (P < 0.05). CONCLUSION: With modified treatment and processing, the cartilage acellular matrix sponge scaffold reserves the main components of cartilage extracellular matrix after thorough decellularization, has appropriate aperture and porosity, and provides even distribution of pores and good biocompatibility without cytotoxicity. It can be used as an ideal scaffold for cartilage tissue engineering.