Arthroscopic synovectomy and radiosynoviorthesis: a treatment option for recurrent arthritis symptoms in patients with pachydermoperiostosis.

Pachydermoperiostosis as the primary form of hypertrophic osteoarthropathy is a rare hereditary disorder with a number of characteristic findings, e.g. periosteal hypertrophy, digital clubbing and pachydermia. Further typical symptoms include arthritic manifestations in all major joints. Literature review showed that therapeutical approaches usually focus on medical therapy alleviating chronic polyarthritis by controlling inflammation. In the case presented in this report anti-inflammatory therapy appeared to have been insufficient since knee arthritis persisted. Arthroscopic synovectomy and radiosynoviorthesis were performed consecutively. Postoperative and follow-up examinations indicate that this combined therapy reduces pain, increases range of motion and improves overall mobility, and is thus of lasting benefit to the patient.

[Amendment of the structural quality for inpatient rheumatology. A forward-looking concept]. / Neufassung der Strukturqualität der akut-stationären Rheumatologie. Ein zukunftsweisendes Projekt.

In 2010 a total of 9 guidelines on structural quality were endorsed by the Association of Rheumatology Clinics in Germany (VRA). These 9 structural criteria replace the regulations published in 2002 and were elaborated with the support of the German Rheumatology League. With guideline number 9 even the structural requirements for university hospitals are defined for the first time.Along with taking part in the quality project "Kobra" (continuous outcome benchmarking in rheumatology inpatient treatment) compliance with the new structural criteria constitutes a prerequisite for acquiring a quality certificate, which is awarded by an external institution.By this means the VRA sets the stage for its members to be prepared for future challenges and quality competition among hospitals. Furthermore, the provision of a high quality treatment for chronically diseased patients in rheumatology clinics will be effectively supported.

Hypogonadism in Wegener's granulomatosis.

OBJECTIVE: Current developments in the management of Wegener's granulomatosis (WG) focus on adoption of therapeutic strategies to avoid complications of both the disease and its therapy. Systematic analyses with respect to the reproductive system in WG are missing. METHODS: Data of a cross-sectional study of sexual hormones in 19 male WG patients were analysed. Disease extension was classified according to the ears, nose, and throat (E), lungs (L), and kidneys (K) classification (ELK classification) and to the disease extent index (DEI). Laboratory investigation included measurement of gonadotrophins, oestradiol, and total serum testosterone. Hypogonadism was defined by an increase in follicle-stimulating hormone (FSH) beyond twice the upper reference range in the presence of low serum testosterone. Thirty-eight age-matched men served as controls. RESULTS: Hypogonadism was found in 52.6% of the patients but was not detectable in any of the controls (p<0.0001). No significant correlation to any clinical factor of systemic vasculitis, current or past medication was detected. In particular, testosterone or FSH levels were not correlated with current or cumulative use of cyclophosphamide or corticosteroids (p = 0.417, p = 0.293; p = 0.893, p = 0.317). CONCLUSION: Data of our study revealed an unexpected high rate of hypogonadism irrespective of cyclophosphamide use, and subclinical involvement of the testes by the vasculitis itself might be an alternative explanation. Screening during the course of the disease is reasonable as hypogonadism might severely affect patients' quality of life. Further prospective studies with respect to gender-specific changes in the reproductive system are warranted.

Peripheral circulating activated b-cell populations are associated with nephritis and disease activity in patients with systemic lupus erythematosus.

B-cell-dependent autoantibody production is a hallmark of systemic lupus erythematosus (SLE) which requires costimulatory molecules. The aim of the study was to analyse the expression of costimulatory molecules on B cells in patients with SLE. Twenty-six patients with SLE (four male, 22 female, mean age 46 +/- 15 years) as defined by the American College of Rheumatology criteria and 13 healthy controls (three male, 10 female, mean age 43 +/- 15 years) were included in the study. In a subgroup analysis, SLE patients were divided according to renal involvement due to SLE (10 with and 16 patients without renal involvement). Clinical disease activity was assessed according to the systemic lupus erythematosus disease activity index (SLEDAI). Blood B-cell populations were analysed by FACS for the cell surface marker expression of CD27, CD38, CD71, CD80, CD86 and CD137 ligand. The expression levels of CD71, CD80 and CD86 on B cells were significantly enhanced in SLE patients when compared with healthy controls (27 +/- 3% versus 11 +/- 2%, P = 0.0003, 55 +/- 2% versus 28 +/- 4%, P < 0.0001, 34 +/- 3% versus 12 +/- 2%, P < 0.0001). CD86 expression was significantly elevated in patients with renal involvement when compared with patients without renal disease (43 +/- 6% versus 28 +/- 3%, P < 0.05). There was a significant correlation between the expression levels of CD80 and CD86 on CD19(+) B cells and disease activity. Moreover, prednisone dose significantly correlated with SLEDAI (r = 0.5, P = 0.02) and with the expression levels of CD86 (r = 0.47, P = 0.02). A pathological B-cell population is associated with disease activity and renal involvement in SLE which are obviously resistant to therapy with medium doses of prednisone.

Rituximab as a treatment option for refractory endogenous anterior uveitis.

BACKGROUND: To report on anti-CD20 antibody therapy in a patient with uveitis refractive to immunosuppression therapy. METHODS: Case report with ophthalmoscopic, optical coherence tomography and fluorescein-angiographic findings. RESULTS: A 49-year-old woman was suffering from bilateral, noninfectious chronic anterior uveitis refractive to corticosteroids and immunosuppressive drugs. Bilateral visual acuity was 20/100 due to cataract and cystoid macular edema (CME). After treatment with rituximab, vision and CME improved, and uveitis was stable until the final visit (follow-up at 12 months). CONCLUSION: The case report suggests that rituximab may be helpful for selected patients with chronic anterior uveitis refractive to corticosteroids and immunosuppressive medication.

CD134 expression on CD4+ T cells is associated with nephritis and disease activity in patients with systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is characterized by a deviation of the immune system that involves T cell-dependent autoantibody production. The aim of this study was to investigate the role of co-stimulatory markers on T cells in this disease. Twenty-eight patients with SLE as defined by the American College of Rheumatology (ACR) criteria and 11 healthy controls were included into the study. Eleven patients had biopsy-proven lupus nephritis while 17 patients had no clinical evidence of lupus nephritis. Clinical disease activity was assessed according to the systemic lupus erythematosus disease index (SLEDAI). CD4+ T cell populations in the peripheral blood were analysed for the expression of co-stimulatory markers CD45RO, CD70, CD80, CD86, CD137, CD137L, CD134, CD152, CD154 and ICOS. SLE patients showed an increased frequency of peripheral CD4+ T cells expressing high levels of CD80, CD86 and CD134 compared to healthy controls (7.1 +/- 1.5% versus 1.7 +/- 0.9%; P < 0.005; 2.3 +/- 0.4% versus 1.0 +/- 0.2%; P = 0.008, 20.2 +/- 2.0% versus 10.6 +/- 1.9%; P < 0.005, respectively). Significantly higher levels of CD80 on CD4+ T cells were detected in SLE patients with lupus nephritis compared to patients without nephritis (11.9 +/- 3.3% versus 4.0 +/- 0.7%; P < 0.005). There was an increased presence of CD134+ CD4+ cells in SLE patients with lupus nephritis (27.5 +/- 4.0% versus 15.5 +/- 1.3%; P < 0.005). CD80 and CD134 expression was significantly correlated with SLEDAI (r = 0.42, P = 0.03; r = 0.56, P < 0.005). Co-stimulatory molecules on CD4+ T cells are associated with renal disease and disease activity in patients with systemic lupus erythematosus.

Acquired factor VIII inhibitors in nonhemophilic patients.

Antibodies against factor VIII occur in about 15-35% of hemophilia A patients and induce refractoriness to factor VIII substitution. In most cases, these antibodies are of the IgG class. Strategies to avoid or to treat such inhibitors are controversial. In very rare cases, factor VIII inhibitors also develop in nonhemophilic patients. Although there are anecdotal reports that these antibodies may disappear spontaneously without occurrence of bleeding tendencies, in the majority of patients the clinical course is characterized by severe hemorrhages. From 1980 to 1995, we observed ten nonhemophilic patients with acquired factor VIII inhibitors at our hospital. In most cases, a sudden bleeding tendency was observed shortly after an injury or surgery. Coagulation tests showed a prolonged aPTT and a decreased F VIII level. Other deficiencies of blood-clotting factors and acquired or hereditary von Willebrand's disease were excluded. Therapy with F VIII concentrates did not produce the expected increase. Measurement of F VIII inhibitor levels in Bethesda units/ml (BU/ml) revealed maximal values in the range of 2-128 BU/ml. Immunosuppressive therapy with azathioprine or cyclophosphamide in combination with methylprednisolone led to complete disappearance of the inhibitor, normalization of the coagulation tests, and complete remission of the bleeding tendency in seven treated patients within 6 weeks. Although the clinical course is not predictable and inhibitors may disappear spontaneously, combined therapy with methylprednisolone and azathioprine or cyclophosphamide is recommended for patients with bleeding tendency. In pregnancy, therapy should be started only with methylprednisolone; post-partum, azathioprine should be used additionally if methylprednisolone as a single drug does not lead to complete remission. In emergency situations, therapy with high doses of human factor VIII concentrate may be used. When bleeding does not cease, the additional use of activated prothrombin-complex concentrates or porcine factor VIII is indicated. Possible side effects may include hepatitis and short-lived intravascular thrombin production.

Effects of glucocorticoids in rheumatoid arthritis. Diminished glucocorticoid receptors do not result in glucocorticoid resistance.

OBJECTIVE: Lymphocytes of patients with rheumatoid arthritis (RA) have diminished receptor density; thus, patients with RA should show partial resistance to glucocorticoids. We investigated the glucocorticoid sensitivity of lymphocytes in RA patients compared with healthy subjects. METHODS: We determined the effects of glucocorticoids on lymphocyte proliferation and cytokine release. RESULTS: Proliferation and cytokine release were inhibited in RA patients to the same extent as in healthy controls. CONCLUSION: Diminished receptor density in RA patients does not result in glucocorticoid resistance.

HLA antigens in ankylosing spondylitis associated with Crohn's disease. Increased frequency of the HLA phenotype B27,B44.

In a consecutive case study 231 patients with Crohn's disease were investigated for ankylosing spondylitis (AS) and HLA-A, B, C, DR antigen association. Eighteen patients (7.8%) had definite AS according to the New York criteria; 13 (72%) were HLA-B27 positive. The phenotype B27,B44 was seen in 8 patients (44%) compared to only 3 (1%) of 300 controls (p less than 10(-7), and 1 (0.5%) of 213 patients with Crohn's disease without AS (p less than 10(-7). We conclude that patients with the phenotype B27,B44 are highly at risk (relative risk = 68.8) for the common manifestation of Crohn's disease and AS.