BACKGROUND: The clinical features of enthesitis in patients with psoriatic arthritis (PsA) have been reported in some Western countries, but data in China are very limited. This study aimed to describe the characteristics of enthesitis in Chinese patients with PsA and compared them with those in other cohorts. METHODS: Patients with PsA enrolled in the Chinese Registry of Psoriatic Arthritis (CREPAR) (December 2018 to June 2021) were included. Data including demographics, clinical characteristics, disease activity measures, and treatment were collected at enrollment. Enthesitis was assessed by the Spondyloarthritis Research Consortium of Canada (SPARCC), Maastricht ankylosing spondylitis enthesitis score (MASES), and Leeds enthesitis index (LEI) indices. A multivariable logistic model was used to identify factors related to enthesitis. We also compared our results with those of other cohorts. RESULTS: In total, 1074 PsA patients were included, 308 (28.7%) of whom had enthesitis. The average number of enthesitis was 3.3 ± 2.8 (range: 1.0-18.0). More than half of the patients (165, 53.6%) had one or two tender entheseal sites. Patients with enthesitis had an earlier age of onset for both psoriasis and arthritis, reported a higher proportion of PsA duration over 5 years, and had a higher percentage of axial involvement and greater disease activity. Multivariable logistic regression showed that axial involvement (odds ratio [OR] 2.21, 95% confidence interval [CI], 1.59-3.08; P <0.001), psoriasis area and severity index (PASI) (OR: 1.03, 95% CI: 1.01-1.04; P = 0.002), and disease activity score 28-C reactive protein (DAS28-CRP) (OR: 1.25, 95% CI: 1.01-1.55; P = 0.037) were associated with enthesitis. Compared with the results of other studies, Chinese patients with enthesitis had a younger age, lower body mass index (BMI), a higher rate of positive human leukocyte antigen (HLA)-B27, more frequent dactylitis, and a higher proportion of conventional synthetic disease-modifying antirheumatic drugs' (csDMARDs) use. CONCLUSIONS Enthesitis is a common condition among Chinese patients with PsA. It is important to evaluate entheses in both peripheral and axial sites.
Humans ; Arthritis, Psoriatic/drug therapy* ; East Asian People ; Enthesopathy/complications* ; Registries ; Severity of Illness Index ; Spondylarthritis/epidemiology*

Bucillamine is a disease modifying anti-rheumatic drug, structurally similar to D-penicillamine. Although D-penicillamine-induced pemphigus has been not infrequently demonstrated, pemphigus associated with bucillamine was rarely reported. We describe a patient complicating pemphigus vulgaris after bucillamine treatment in rheumatoid arthritis (RA) and polymyositis (PM) overlap syndrome. PM and RA overlap syndrome was diagnosed three years ago and bucillamine was administrated for 20 months. Skin lesions including erythematous flaccid blisters on her chest, axillae, and back were occurred and were compatible with pemphigus vulgaris by typical pathology. Withdrawal from bucillamine and prednisolone treatment made rapid improvement of pemphigus lesions.
Syndrome ; Skin/pathology ; Polymyositis/*complications/*drug therapy ; Pemphigus/*chemically induced/*pathology ; Middle Aged ; Humans ; Female ; Cysteine/adverse effects/*analogs & derivatives ; Biopsy ; Arthritis, Rheumatoid/*complications/*drug therapy ; Arthritis ; Antioxidants/*adverse effects

OBJECTIVETo study the efficacy of total knee anhmplasty (TKA) for the treatment of hemophilic knee arthropathy, and to explore the operative characteristics, the selection of prothesis, the effectiveness and safety of clotting factor replacement treatment.

METHODSFrom January 2008 to June 2010, 10 patients (12 knees)with hemophilic anhropathv underwent TKA. The average age was 33.6 years old (ranged, 17 to 49 years). There was 8 cases of type A hemophilia and 2 cases of type B hemophilia. According to Arnold and Hilgartner classification: 7 knees were IV degree and 5 knees were V degree. The level of VIII factor for replacement treatment was more than 80% on operation day, more than 60% within 3 days after operation, more than 40% from the third day to the second week after operation. Added prothrombin complex concentrate (PCC) to improve the level of IX factor, and the level of IX factor for replacement treatment was more than 40% on operation day, more than 30% within 3 days after operation, more than 20% from the third day to the second week after operation. Functional training was mainly based on continuous passive motion (CPM) device after surgery. Clinical assessment included hospital for special surgery knee score (HSS) and the individual scores (including pain, function, activity, muscle strength, flexion deformity and stability).

RESULTSTen patients (12 knees) were followed-up, and the average duration was 11 months (ranged, 6 to 24 months). The average preoperative HSS score was (44.9 +/- 12.5) (ranged, 29 to 62 scores), whereas the average postoperative HSS score was (84.4 +/- 10.2) (ranged 72 to 96 scores) (P < 0.01). The preoperative individual score including pain, function, activity, muscle strength flexion deformity and stability were significantly improved compared with pre-operation, the differences between them were statistically significant (P < 0.01 ). TKA had the distinct role in relieving pain from preoperative (8.5 +/- 4.1) to postoperative (24.5 +/- 4.4).

CONCLUSIONUnder the strict perioperative coagulation factor replacement therapy, TKA is a safe and an effective treatment for hemophmc anhmpathy of knee joint, whicht can effectively relieve pain and improve joint function.

Adolescent ; Adult ; Arthritis ; surgery ; Arthroplasty, Replacement, Knee ; methods ; Female ; Hemarthrosis ; surgery ; Hemophilia A ; complications ; drug therapy ; Humans ; Male ; Middle Aged

OBJECTIVEMacrophage activation syndrome (MAS) is a rare but life-threatening complication in children with rheumatic diseases, particularly systemic-onset juvenile idiopathic arthritis (SOJIA). Because of the potential fatality of this condition, prompt recognition and immediate therapeutic intervention are important. This study reviewed the data of MAS in 13 cases with SOJIA.

METHODSRetrospective review was performed on the precipitating events, clinical manifestations, laboratory data, treatment, and outcome of macrophage activation syndrome in 13 children with SOJIA seen from 1996 to 2005.

RESULTSOver the past 10 years the unit has had 90 new patients with SOJIA. Thirteen of those patients (14.4%) developed MAS during the course of their primary SOJIA, of whom ten were male. All patients were noted to have active SOJIA prior to developing MAS; 3 patients had medications, which were considered as trigger factors; 8 had infections prior to MAS, in two of them the infections were possible triggers. All the patients had high grade fever; 12 cases (92.3%) had hepatomegaly; 10 patients (76.9%) had coagulopathy, and eight patients (61.5%) had central nervous system dysfunction. The counts of platelet, white blood cells and the mean erythrocyte sedimentation rate fell dramatically in all patients; hyperferritinemia was identified in 8 patients, in 5 of whom serum ferritin (SF) was >or= 10,000 microg/L; in 8 (72.7%) of 11 cases fibrinogen was or= 2.5 mmol/L in 9 (69.2%) of 13 cases.

CONCLUSIONMAS is a rare and potentially fatal complication of children with SOJIA. Primary disease activity, medications and infections preceding MAS were all important triggers. The strongest clinical discriminators were hepatomegaly, hemorrhages and central nervous system dysfunction. The strongest laboratory tests were decreased counts of platelet and white blood cells, decreased ESR and fibrinogen, dramatically increased SF and TG. It calls for the immediate treatments, particularly with cyclosporin A, which are often effective.

Arthritis, Juvenile ; complications ; drug therapy ; pathology ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Macrophage Activation Syndrome ; drug therapy ; etiology ; pathology ; Male ; Retrospective Studies

OBJECTIVETo review and analyze the clinical features, treatment, and outcome of macrophage activation syndrome (MAS) in children with systemic onset juvennil rheumatoid arthritis (SOJRA).

METHODRetrospective review and analysis were performed on cases with MAS from a prospectively collected database of children with SOJRA from the year of 2003 to 2006 in the Hospital.

RESULTSTwenty four patients (21 boys, 3 girls) were diagnosed as having MAS with SOJRA. Mean age of the patients with MAS at diagnosis was 7 years, and the duration prior to diagnosis of MAS was 12 months. No trigger factors were found except in one case whose MAS was triggered by use of methotrexate and in another by parvovirus B19 infection. High grade fever, new onset hepatosplenomegaly and lymphadenopathy, pancytopenia, liver dysfunction were common clinical features in all the 24 cases (100%). Bleeding from skin, mucous membrane and gastrointestinal tract were noted in 9 cases (38%). Twelve (50%) cases had CNS dysfunction (high intracranial pressure, seizure and coma). Six cases (25%) developed ARDS. One patient suffered from renal damage. The laboratory test revealed elevated live enzymes and ferritin, decreased value of ESR, albumin, complete blood count and fibrinogen in all the 24 cases. Bone marrow examination supported the diagnosis of definite hemophagocytosis in the 24 cases. Lymph node biopsy was done for one case and histopathological examination showed that the node was full of activated macrophage. As to treatment, five cases only received high dose steroids (three of them died), 14 cases were treated with high dose steroids plus cyclosporine (one died), two were treated with steroids plus cyclosporine and etoposide (none died). The causes of deaths were ARDS and CNS involvement. In three of the cases who died, treatment was given up by their parents.

CONCLUSIONSMAS is a rare and potentially fatal complication of SOJRA. Most of our patients were male. Bone marrow studies support the diagnosis. CNS involvement and ARDS were poor prognostic signs. Early diagnosis and aggressive therapy are essential.

Adolescent ; Arthritis, Juvenile ; complications ; drug therapy ; pathology ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Macrophage Activation Syndrome ; drug therapy ; etiology ; pathology ; Male ; Retrospective Studies

The triad of rash, arthritis, and uveitis seems to be characteristic for early-onset childhood sarcoidosis. We describe an interesting case of early-onset childhood sarcoidosis coexisting enchondromatosis, which clinically masquerade as Langerhans cell histiocytosis. A 33 months old girl presented with skin rash, subcutaneous nodules with polyarthritis, and revealed the involvement of lymph nodes as well as spleen during work-up. She also presented with multiple osteolytic lesions which pathologically proven enchondromatosis. Oral prednisone was prescribed at 2 mg/kg/day for 2 months until when subcutaneous nodules and joint swellings almost disappeared, and then slowly tapered over a period of 5 months. We report an unusual case of early-onset childhood sarcoidosis presented with osteolytic bone lesions which were irrelevant to sarcoidosis.
Administration, Oral ; Anti-Inflammatory Agents/therapeutic use ; Arthritis/complications ; Child, Preschool ; Diagnosis, Differential ; Enchondromatosis/*complications/*diagnosis/drug therapy/radiography ; Exanthema/etiology ; Female ; Humans ; Positron-Emission Tomography and Computed Tomography ; Prednisone/therapeutic use ; Sarcoidosis/*complications/*diagnosis/drug therapy/radiography ; Whole Body Imaging

Most reports on serious MTX toxicity have focused on hepatic abnormalities, while other effects, including hematologic reactions, have not been emphasized. We experienced a case of pancytopenia secondary to MTX therapy in a patient with RA and renal insufficiency. A 67-year-old woman with a 12-year history of active seropositive RA that was a response to non-steroidal anti-inflammatory drugs, hydroxychloroquinine and intra-articular steroid injections, had been followed up and was diagnosed as early chronic renal failure in October, 1993. Recently, because of significant morning stiffness and polyarthralgia, the decision was made to institute MTX treatment. This was begun as a single oral dose of 5mg/week. After 2 doses, the patient was admitted to the hospital with general weakness. Laboratory tests showed a hemoglobin level of 7.9 g/dl, WBC count 1800/mm3 and platelet count of 64000/mm3. The serum creatinine level was 6.1 mEq/dl and the BUN level was 82 mEq/dl. Liver function test results were normal, but the serum albumin level was 2.7 g/dl. The patient subsequently developed fever and blood transfusions, granulocyte colony stimulating factor (G-CSF) and intravenous prophylactic antibiotic therapy were required. Her condition was improved. In summary, Low-dose MTX-related adverse hematologic side effects, including fatal pancytopenia, are rare but are a cause of increasing concern in patients with RA and renal insufficiency. Close monitoring of associated risk factors, particularly impaired renal function, should be mandatory for all patients who are receiving MTX therapy.
Aged ; Antirheumatic Agents/adverse effects* ; Antirheumatic Agents/administration & dosage ; Arthritis, Rheumatoid/drug therapy ; Arthritis, Rheumatoid/complications ; Case Report ; Female ; Human ; Kidney Failure, Chronic/complications ; Methotrexate/adverse effects* ; Methotrexate/administration & dosage ; Pancytopenia/chemically induced* ; Risk Factors

OBJECTIVETo investigate the effects of methanol extract of Celastrus orbiculatu (MECO) on synovial hyperplasia and cartilage erosion and degradation in rheumatoid arthritis (RA), and explore the possible mechanisms to provide clues for new drug development for RA treatment.

METHODSThe articular synovium from patients with RA and normal articular cartilage were co-implanted into the back of severe combined immunodeficient (SCID)mice to establish the chimeric model SCID- HuRAg. Four weeks later, the mice were given MECO intragastrically at 30 mg/day, leflunomide at 500 microg/day or distilled water, respectively, for 4 consecutive weeks. After completion of the treatments, the histological scores of the grafts for synovial hyperplasia, cartilage invasion by synoviocyte and cartilage degradation around the chondrocytes were evaluated, and serum level of tumor necrosis factor-alpha (TNF-alpha) was measured with radioimmunoassay. The expression of TNF-alpha mRNA and the cell apoptosis in the synovium were detected with in situ hybridization (ISH) and TUNEL, respectively, and the results were analyzed with the image analysis system.

RESULTSThe grafts survived in the mice till the end of experiment. MECO and leflunomide, in comparison with distilled water, significantly lowered the scores for synovial hyperlasia (2.00+/-0.76 and 2.25+/-0.89 vs 3.63+/-0.52), cartilage erosion (1.69+/-0.80 and 2.00+/-1.36 vs 3.75+/-0.53), cartilage degradation (1.88+/-0.83 and 2.13+/-0.83 vs 3.63+/-0.74) and serum TNF-alpha level (0.84+/-0.09 and 0.83+/-0.12 vs 0.99+/-0.11 ng/ml). Cell apoptosis of the synovium increased significantly with MECO and leflunomide treatments, but the expression of TNF-alpha mRNA in the synovium decreased significantly in MECO group.

CONCLUSIONMECO can effectively suppress synovial hyperplasia and cartilage erosion and degradation SCID-HuRAg mice by reducing TNF-alpha production in the synovium and promoting synovial apoptosis. MECO can be comparable with leflunomide in their effect, but the former is more effective in suppressing TNF-alpha expression in the synovium.

Animals ; Apoptosis ; drug effects ; Arthritis, Rheumatoid ; complications ; drug therapy ; metabolism ; pathology ; Cartilage Diseases ; complications ; drug therapy ; metabolism ; pathology ; Celastrus ; chemistry ; Cell Transplantation ; Female ; Gene Expression Regulation ; drug effects ; Humans ; Hyperplasia ; complications ; drug therapy ; Male ; Methanol ; chemistry ; Mice ; Plant Extracts ; isolation & purification ; pharmacology ; therapeutic use ; RNA, Messenger ; genetics ; metabolism ; Synovial Membrane ; drug effects ; pathology ; transplantation ; Tumor Necrosis Factor-alpha ; blood ; genetics

OBJECTIVETo study clinical characteristics of children with enthesitis related arthritis (ERA).

METHODSTwelve patients fulfilling the international league of associations for rheumatology (ILAR) criteria for classification of juvenile idiopathic arthritis (JIA) and ERA were referred to our department between August and November, 2002. Their gender, age, family history, clinical manifestations, imaging data, laboratory data and treatment regimens were analyzed retrospectively.

RESULTSOf the 12 patients, 11 were male, only one was female; their age ranged from 4 to 16 years, and the median age was 10.5 years. Ten (83%) of the patients were older than eight years. Among their first degree relatives, one had definite ankylosing spondylitis (AS), and 3 presented with a history of inflammatory low back pain. Enthesitis occurred in 9. Synovitis occurred in 11, most of which were oligoarthritis, predominantly affecting large joints of the lower limbs in an asymmetric pattern. Seven patients underwent CT scan, and only one had erosions of the sacroiliac joints to achieve a diagnosis of juvenile AS. Ten had fever at the onset, and one had a history of diarrhea preceding the symptoms of arthritis. Urethritis and balanitis circinata occurred in 3 cases fulfilling the classification criteria of Reiter's syndrome, with conjunctivitis in 2 and corneitis in one. Elevated inflammatory indicators such as white blood cell, neutrophil, platelet, erythrocyte sedimentation rate, C-reactive protein, immunoglobulins and serum complement C3 were common during the acute illness. Mild anemia was found in 4, and reactive hemophagocytosis in 4 bone marrow specimens. DNA of human parvovirus B19 was detected in sera of 2 cases. One had positive IgM antibody to coxsackie virus B. All 12 cases were rheumatoid factor negative and HLA-B27 positive. Nonsteroidal anti-inflammatory drugs and sulfasalazine were the mainstay of treatment. Corticosteroids were added in 3 cases as a "bridge" therapy due to the severe systemic inflammation. Methotrexate was used in 4 cases with refractory disease or with the hip involved, and in 2 of them cyclophosphamide was added.

CONCLUSIONThe clinical characteristics of ERA can facilitate an early diagnosis so as to avoid joint damage and disabilities.

Adolescent ; Anti-Inflammatory Agents, Non-Steroidal ; therapeutic use ; Arthritis ; drug therapy ; etiology ; Child ; Child, Preschool ; Female ; Humans ; Joint Diseases ; complications ; Joints ; pathology ; Male ; Retrospective Studies

Antirheumatic Agents ; therapeutic use ; Arthritis, Rheumatoid ; complications ; drug therapy ; Atlanto-Axial Joint ; injuries ; Humans ; Joint Dislocations ; etiology ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Syringomyelia ; diagnosis ; etiology ; physiopathology