[ADAPTHERA-Statewide cross-sectoral care network for patients with early rheumatoid arthritis shows sustained remission in standard care]. / ADAPTHERA ­ Landesweit transsektorales Versorgungsnetzwerk für Patienten mit früher rheumatoider Arthritis zeigt anhaltende Remissionen in der Regelversorgung.

BACKGROUND/OBJECTIVE: The majority of patients in Germany miss out on the necessity of early diagnosis and initiation of therapy for rheumatoid arthritis (RA) caused by considerable structural deficits in the health care system. The challenge is to reconcile the individual demand for the best possible therapy result with a sustainable expenditure of resources. METHODS: The cross-sectoral regional care network ADAPTHERA aims to improve early RA diagnosis and treatment in Rhineland-Palatinate. The retrospective triage analyses of suspected early onset RA patients was performed by tracing the selection process of all available enquiries (n = 1045). For analysis of the clinical course of the disease, a subset comprising 143 patients with a minimum observation time of 12 months (5 consecutive visits) was available. Clinical and laboratory parameters were collected quarter yearly, self-administered questionnaires were filled out and the treatment was adapted if necessary. RESULTS: A total of 454 patients were included. The mean waiting time was 23.9 (SD = 18) days. The mean observation period in the subcohort was 29.2 (SD = 12.7) months, with about 50% of the patients presenting within 3 months. Almost 75% of the patients were in remission after 2 years. A sustained remission could be described for 74.8% (6 months) and 53.5% (12 months), respectively. Especially patients with rapid remission induction benefited in terms of longer remissions (p = 0.03). A very early stage of the disease (VERA) was associated with a rarely necessary biologic therapy (p = 0.022). DISCUSSION: The approach of a supply network is not a panacea, but it might improve healthcare for patients with early onset RA. In order to minimize resource utilization, a pinpoint referral and accurate triage of potential cases are crucial.

[The regional network ADAPTHERA : Rheumatology care through coordinated cooperation: comprehensive, trans-sectoral, covering all health insurance. Initial results]. / Das landesweite Netzwerk ADAPTHERA : Erste Ergebnisse einer flächendeckenden, krankenkassenübergreifenden und transsektoralen rheumatologischen Versorgung.

The aim of the rheumatology network ADAPTHERA ("risk-adapted rheumatology therapy") is to achieve a comprehensive improvement in rheumatology care by coordinating treatment in a regional, trans-sectoral network. Accompanying biomedical research projects, training concepts, and the construction of a rheumatology register (gathering data and biomaterials) should furthermore ensure the stable and sustainable optimisation of care. In the pilot phase (2012-2015) the focus of the ADAPTHERA network, required as a "regional key project" within the framework of the Initiative on Health Economy of Rheinland-Palatinate (RL-P), Germany, was placed on the optimisation of the early diagnosis of rheumatoid arthritis, where it is well-known that there is a significant care deficit.Through the intensive, stable, and coordinated cooperation of all health care partners in the field of rheumatology (registered general practitioners and orthopaedic specialists, registered core rheumatologists as well as the Association of Rheumatology of RL-P) a unique regional, comprehensive offer with verifiable care optimisation has been established in RL-P. The network is supported by outstanding collaboration with the Association of Statutory Health Insurance Physicians and the self-help organisation Rheumatology League.The aims that were established at the start of the project will be achieved by the end of the pilot phase:- significant improvement in the early diagnosis of rheumatoid arthritis (an average of 23.7 days until diagnosis by rheumatologists)- access covering all health insurance (regardless of the particular scheme the patients belong to)- comprehensive (verifiable participation of general practitioners from all over RL-P)- data and biomaterials collection, established as a basis for biomarker research, and a rheumatology register for RL-P.

Serum antibody titers in a systemic lytic therapy with streptokinase.

BACKGROUND: Anaphylactic reactions to streptokinase are rare but potentially life-threatening complications. Gamma E immunoglobulin (IgE) mediated mechanisms, probably due to streptococcal infections, have been implicated. We investigated the value of in vitro laboratory or dermatologic tests in predicting anaphylactic reactions due to streptokinase and the value of antistreptolysin titers (ASL) in predicting the amount of specific IgE (sIgE) and specific gamma G immunoglobulin (sIgG) neutralizing antibodies to streptokinase. METHODS: We measured serum levels of total IgE, streptokinase sIgE and sIgG, and ASL in 16 patients before and 9 and 41 days after streptokinase therapy. Immediately before therapy, intracutaneous testing with 100 IU streptokinase was done. RESULTS: Dermatologic testing did not identify patients prone to allergic reactions. Moreover, not all patients with increased sIgE levels had allergic reactions. These reactions were independent of the dose of streptokinase given. In spite of steroid prophylaxis, allergic reactions occurred in 3 of 16 patients, but none showed life-threatening anaphylaxis. Streptokinase sIgE and sIgG concentrations were closely related to ASL titers. CONCLUSIONS: Plasma levels of sIgG, sIgE, and ASL titers showed a good correlation. We believe ASL titers can be used for the estimation of neutralizing antibodies instead of streptokinase sIgG antibodies. Currently, no laboratory or dermatologic test allows reliable predictions of allergic reactions to streptokinase.

[Anaphylactic reaction in streptokinase therapy]. / Anaphylaktische Reaktion unter Streptokinase-Therapie.

Streptokinase is used worldwide as thrombolytic agent. Indications are deep vein thrombosis, arterial thrombosis and embolism, arteriovenous cannula occlusions, acute coronary artery thrombosis, and acute renal vein thrombosis. The major drawback in comparison with other thrombolytic drugs is its antigenicity. Allergic reactions have been reported but the underlying mechanisms have not yet been fully elucidated. A 58 year-old man with acute myocardial infarction developed an anaphylactic reaction immediately after commencement of streptokinase infusion, although immunological laboratory findings were not affected. The patient had no history of prior exposure to streptokinase, infections with streptococci, or chronic allergic reactions. 2 months after streptokinase therapy increased plasma levels were detected of specific IgG antibodies to streptokinase. The levels of specific IgE antibodies to streptokinase and other anti-streptococcal antibodies were within the normal range. A prick test performed 4 months later with 100 I.U. streptokinase was unremarkable. The problems of such allergic reactions are discussed on the basis of this case report and a review of the literature.

Animal poxviruses transmitted from cat to man: current event with lethal end.

We report about the infection of an 18-year-old man with an orthopox virus (OPV) which was transmitted by a cat. The infectious route from cat to man could be proved by epidemiological, virological and serological methods. The corresponding techniques are described. The patient had not been vaccinated against smallpox and was intensively immunosuppressed by medication on account of a severe endogeneous eczema combined with an allergic asthma bronchiale. A cyclic poxvirus disease developed with a generalised, partly confluent pox virus exanthema disseminated over the body. The clinical symptoms were similar to a "variola pustulosa haemorrhagica". The young man died of a lung embolism in the course of the intensive medical therapy. The haemorrhagic character of the pox virus pustules with central necrosis (pox navel) could be reproduced in the rabbit skin and on chorioallantois membranes. The pox virus isolated from the patient could be differentiated from variola, vaccinia and monkeypox virus. It is a member of the group of "cowpox-like viruses". The environmental importance of these OPVs is discussed.

[Lethal animal pox virus infection in an atopic patient simulating variola vera]. / Letale Tierpockeninfektion bei einem Atopiker unter dem Bild einer Variola vera.

An 18-year-old patient requiring steroid treatment for severe bronchial asthma and with atopic dermatitis acquired a cowpox-like virus infection clinically similar to smallpox from a domestic cat as carrier. In spite of intensive care, with controlled pressure breathing and the last available vaccinia hyperimmunoglobulin, the patient died of pulmonary embolism although viral spread had ceased some days before.

[Two cases of side effects of a fumaric acid ester--local therapy]. / Zwei Fälle von Nebenwirkungen einer Fumarsäureester--Lokaltherapie.

We report on two cases of adverse reactions to topical treatment with monoethyl fumarate. One patient suffering from atopic dermatitis reacted with contact dermatitis; the other, suffering from psoriasis, developed a generalized, partly pustulous exanthema as well as signs of systemic involvement, such as tachycardia and dyspnea. As the causative mechanism we suggest non-immunological contact urticaria syndrome.

[Malignant melanoma in café-au-lait spot]. / Malignes Melanom in Café-au-lait-Fleck.

A 17-year-old female patient developed a superficially spreading malignant melanoma (level III, thickness 0.91 mm) on a typical café-au-lait spot. There were no metastases, as yet. On the basis of this unusual case, we discuss the incidence of development of malignant melanoma on melanotic spots.

[Malignant fibrous histiocytomas]. / Maligne fibröse Histiozytome.

Malignant fibrous histiocytomas of the storiform pleomorphic type were found in the lower legs of two patients, 79 and 42 years of age. In the first, dermatofibrosarcoma protuberans, in the second, squamous cell carcinoma, had to be discussed. In the first patient, the tumor arose in the area of a preexisting paresis of the superficial peroneal nerve; the second patient developed the tumor in fistulous osteomyelitis existing since childhood.

[Cutaneous sarcoidosis]. / Kutane Sarkoidosen.

We report on three clinical cases of cutaneous sarcoidosis. One of them was associated with erythema nodosum, another with erythema annulare centrifugum. The third case showed an annular lesion having persisted for 10 years; X-ray of the lungs revealed stage 3 disease.

[Syringocystadenoma papilliferum as a differential diagnosis of a carcinoma of the lower lip]. / Syringocystadenoma papilliferum als Differentialdiagnose eines Unterlippencarcinoms.

A 56-year-old patient showed a skin-colored tumor of the lower lip which had been photographically documented since his 10th year of age. On account of the localisation, increase of size during the last few months, as well as irregular epithelial hyperplasia observed in a punch biopsy from the periphery, we suspected the tumor to be a squamous cell carcinoma. Histological examination, however, revealed syringocystadenoma papilliferum.

[Contact dermatitis due to chloroacetophenone (tear gas)]. / Kontaktdermatitis auf Chloracetophenon (Tränengas).

We report on 3 patients suffering from allergic contact dermatitis to chloracetophenone, two of them policemen. Besides toxic local and systemic effects, one possible risk of using tear gas is sensitization.

Klinefelter's syndrome and Breast cancer.

Breast cancer in a patient with Klinefelter's syndrome is reported. Possible correlation between testosterone and estradiol serum levels after testosterone-enanthate substitution, estrogen receptors in tumor tissue and clinical symptomatology are discussed. The various theories of etiology concerning breast cancer in this syndrome are reviewed. The increased conversion of testosterone to estradiol at the therapy with androgens might be responsible for the development of breast cancer in Klinefelter's syndrome. The current way of treatment is described.

[Cystic acne and clinocomptodactyly in Klinefelter's syndrome (46XY/47 XXY mosaic)]. / Zystische Akne und Klinokamptodaktylie bei Klinefelter-Syndrom (46 XX/47 XXY Mosaik).

Klinefelter's syndrome was found in a 18 year old patient, suffering for four years from nodulocystic acne. Serum gonadotropins were within normal range, while histology of the testes revealed beginning tubular hyalinization. The case report shows that young patients with Klinefelter's syndrome may present symptoms that seem to exclude this syndrome. Furthermore, our patient exhibited klinokamptodaktylia of hands and feet, a congenital anomaly occurring very rarely in Klinefelter's syndrome.