[If digital clubbing appears bear Marie-Bamberger disease in mind]. / Bei neu aufgetretenen Trommelschlegelfingern daran denken: Marie-Bamberger-Syndrom.

Secondary hypertrophic osteoarthropathy, also known as Marie-Bamberger disease, occurs in up to 5 % of patients with non-small cell lung cancer (NSCLC). If the syndrome constellation of finger clubbing and bone pain on palpation is recognized early, lung cancer can be diagnosed at an early stage. This article reports the case of a 52-year-old male patient with knee pain, distal edema, watchglass nails and finger clubbing which first appeared in August 2013. Following angiologic, cardiologic and orthopedic consultations radiographs of the hands, feet and lungs were taken. Following the presumptive diagnosis of hypertrophic osteoarthropathy, the patient was referred to a specialist pulmonary clinic where an adenocarcinoma of the lungs was diagnosed.

[Regression of peripheral and pulmonary rheumatoid nodules under therapy with rituximab]. / Regression von peripheren und pulmonalen Rheumaknoten unter Rituximab-Therapie.

We report on a retrospective study of 16 rheumatoid arthritis (RA) patients with reduction in size of pulmonary and peripheral rheumatoid nodules following treatment with rituximab (RTX). The 8 female and 8 male patients had an average disease duration of 12.2 years, 88 % were anti-CCP positive and 94 % seropositive. Prior treatment included an average of 2.9 DMARD and 1.4 biological therapies. On average 6.1 rheumatoid nodules were found on hands and elbows and 5 patients had pulmonary nodules. In 6 out of 16 patients the nodules disappeared completely, in 2 patients a pulmonary nodule disappeared. In 10 out of 16 patients the size of the nodules decreased by approximately 50%, 1 out of the 16 patients with significant increase in size and number of nodules prior to RTX therapy showed a reduction in size but no new nodules occurred. The regression in size of the nodules occurred 34.2±39.1 weeks following RTX therapy, correlating with 1.3±0.59 RTX infusion cycles. Overall, increases in size or new nodules were reported in none of the patients. One nodule examined histologically following RTX therapy did not show any specific differences. RTX may lead to a marked reduction in size of rheumatoid nodules in RA patients. More studies are necessary to confirm whether this is an RTX-specific effect.

[Development and/or increase of rheumatoid nodules in RA patients following leflunomide therapy]. / Neuauftreten und/oder Zunahme von Rheumaknoten unter Leflunomidtherapie bei RA-Patienten.

This is the first description of the development of rheumatoid nodules in 3 rheumatoid arthritis patients following leflunomide therapy. The nodules were localized at typical sites with preference of the extensor side of hands and elbow. One nodule examined histologically revealed the typical architecture of RA nodules. In all 3 patients the time of onset of nodulosis was about 6 months after initiating the leflunomide therapy. In all 3 patients leflunomide was clinically efficacious concerning RA: remission or near remission was achieved. Due to the extent of nodulosis, the leflunomide therapy had to be stopped in 2 patients. Progression and acceleration of nodulosis is well known following MTX therapy in RA patients. It is caused by adenosine A1 receptor promotion of multinucleated giant cell formation by human monocytes. Leflunomide has no known influence on adenosine metabolism, so different pathogenetic mechanisms must be assumed for the induction of nodulosis by leflunomide.

[Listeria arthritis in chronic polyarthritis during low dose prednisolone and methotrexate therapy. Case report and review of the literature]. / Listerienarthritis bei chronischer Polyarthritis unter Low-dose-Prednisolon- und Methotrexat-Therapie. Kasuistik und Literaturübersicht.

We report about a patient with polyarticular rheumatoid arthritis taking methotrexat and 5 mg prednisolone who developed in the course of a RA flare a septic arthritis in the right shoulder. Listeria monocytogenes could be identified as the causative bacteria. Clinically, the Listeria-induced septic arthritis could not be differentiated from rheumatoid arthritis; fever was not present. The synovial analysis showed a granulocytic effusion with 19,000 cells/ml; there was no microbiological growth within the first 24 hours. Only the low glucose level indicated a possible septic arthritis. After 48 hours, gram-positive bacterial growth was evident and Listeria monocytogenes could be isolated after 72 hours. Therapy was initiated by antibiotic treatment and arthrotomy with synovectomy followed by extensive irrigation which proved effective in bacterial elimination but joint destruction resulted. During the whole course, Listeria antibodies were negative and proved to be too insensitive. The incidence of Listeria-induced arthritis is very low; a review of the literature revealed only 24 reported cases. It occurs primarily in patients with rheumatic diseases under immunosuppression and in prosthetic joints. The diagnosis is based on cultural detection. It is important to cultivate synovial effusions for longer than 24 hours in order to identify Listeria. This is of relevance since Listeria serology is not sensitive.

Monoclonal antibody WGM1 directed against proteinase 3: an immunohistochemical marker for naphthol ASD chloroacetate.

Enzyme histochemistry for naphthol ASD chloroacetate (NASDCA, 'Leder's stain') is used to identify the granulocyte lineage ranging from promyelocytes to mature neutrophils and is an additional tool for the characterization of leukemias. We demonstrated for the first time that NASDCA activity can be detected by routine immunohistochemistry and immunocytochemistry using the monoclonal antibody WGM1 directed against proteinase 3 (PR3; synonyms: Wegener's autoantigen, myeloblastin). Immunohisto- and immunocytochemical staining with WGM1 against PR3 and enzyme histochemistry for NASDCA produced identical staining patterns in normal myelomonocytic cells and cells of myeloid leukemia. This was additionally proven by double immunostainings. We have also shown that PR3 is one of the specific proteinases responsible for hydrolysis of NASDCA.

Incidence, target antigens, and clinical implications of antineutrophil cytoplasmic antibodies in rheumatoid arthritis.

OBJECTIVE: To determine the incidence of antineutrophil cytoplasmic antibodies (ANCA) in rheumatoid arthritis (RA), to detect the target antigens of ANCA, and to compare clinical and laboratory data of ANCA+ with ANCA- patients with RA. METHODS: 385 sera of patients with RA were screened for ANCA by indirect immunofluorescence. ANCA+ sera were further analyzed for target antigens by ELISA: The ANCA+ patients were compared to randomly selected ANCA- patients with RA with respect to serological and radiological variables and extraarticular involvement. RESULTS: ANCA were found in 16% of patients with RA (61/385 sera). All sera showed a perinuclear (pANCA) pattern. Antibodies directed against proteinase 3 were not observed. The analysis of ANCA+ and ANCA- patients revealed that the pANCA+ group exhibited significantly higher serological markers of inflammation (p < 0.005) and a higher incidence of rheumatoid factor (p < 0.005). Furthermore, vasculitic involvement was found at a higher frequency (p < 0.05) in the pANCA+ group. Five patients in the pANCA+ group had pulmonary involvement, but none in the pANCA- group. CONCLUSION: pANCA in RA may be a marker for a more aggressive course of disease in respect to serological variables and extraarticular manifestations including rheumatoid vasculitis and lung involvement.

Proteinase 3, the target antigen of anticytoplasmic antibodies circulating in Wegener's granulomatosis. Immunolocalization in normal and pathologic tissues.

Proteinase 3 was identified as the target antigen of anticytoplasmic antibodies (ACPA/c-ANCA) in Wegener's granulomatosis (WG). Using the murine monoclonal antibody (MAb) WGM2, the authors localized proteinase 3 on normal and pathologic tissue and in various human cell lines. Proteinase 3 was found in all granulocytes, mast cells, and a subset of monocytes (2-10% of peripheral blood monocytes). No crossreactivity of the MAb with other cells or tissue components was observed. Comparing various vasculitic and granulomatous tissues, WG granulomas contained more granulocytes. In tissue of WG, no crossreactivity of the MAb with tissue components such as blood vessel wall or endothelium was seen. The monocytic line THP-1 and the promyelocytic line HL 60 contained proteinase 3. Since proteinase 3 was only found in neutrophil granulocytes, mast cells, and a subset of monocytes, the presented data provide further evidence that granulocytes play a central role in the pathogenesis of WG.

Morphological differentiation of human lymphocyte subpopulations following polyclonal stimulation with bacteria and lectin.

Mononuclear cells (MNC) were stimulated with different heat-treated gram-positive and -negative bacteria, and the lymphocyte subpopulation interaction, the proliferative response, and the immunoglobulin secretion were analyzed. It can be demonstrated that beta haemolytic streptococci and Staphylococcus aureus have a similar stimulation pattern: early stimulation of helper T cells, cell contacts of helper T cells and B cells, maximum proliferation on day 5 and 7, and Ig secretion peak on day 7. Klebsiella pneumoniae and Yersinia enterocolitica do not cause proliferation, while Ig secretion is seen on day 5. No cell contacts and no T cell stimulation are seen in the Klebsiella culture, whilst Yersinia causes slight helper T cell activation. In contrast, PHA induces strong T cell stimulation, proliferation and expansion of the suppressor T cell subpopulation. Leu 7-positive lymphocytes are not activated by any of these stimulants.