OBJECTIVES:There are limited data on the additional diagnostic yield of axillary artery ultrasound (axUS) in addition to temporal artery ultrasound (tempUS) for the diagnosis of giant cell arteritis (GCA).METHODS:Retrospective study of consecutive patients with suspected GCA who underwent a standardized axUS and tempUS between 01/2015 and 03/2017. The diagnostic yield of axUS in addition to ultrasound of the temporal arteries with respect to the final clinical diagnosis was assessed, with a positive axUS defined as circumferential, hypoechogenic thickening of the far wall axillary artery intima media thickness (axIMT) ≥1.3 mm. A subgroup of patients underwent PET-CT within one week before or after the sonographic study. Separate analyses were performed regarding certain subgroups according to clinical presentation and to clinical pre-test probability for cranial GCA.RESULTS:Out of 228 patients, 92 received a final diagnosis of GCA. From the 92 patients with a final diagnosis of GCA, 50 (54.3%), 13 (14.1%) and 15 (16.3%) had a positive tempUS, positive axUS, and combined positive tempUS and axUS, respectively. The sensitivity of sonographic imaging for the final diagnosis of GCA increased from 69.6% to 84.8%, when axUS results were considered in addition to tempUS, while the specificity remained high (no false positive axUS). The diagnostic yield of axUS was highest in patients with a low clinical probability of cranial GCA and lowest in patients with symptoms of ocular ischemia. We observed a substantial rate (42.1%) of discordant results between axUS and PET-CT in a subgroup of 38 patients.CONCLUSIONS:In conclusion, axUS offers a substantial diagnostic yield in addition to tempUS in subjects with suspected GCA, mainly in those subjects with low clinical probability for cranial GCA.

Federführende Fachgesellschaft DeutscheGesellschaft fürRheumatologie (DGRh). Beteiligung anderer Fachgesellschaften Berufsverband Deutscher Pathologen e.V.; Deutsche Gesellschaft für Angiologie – Gesellschaft für Gefäßmedizin e.V. (DGA); Deutsche Gesellschaft für Gefäßchirurgie und Gefäßmedizin – Gesellschaft für operative, endovaskuläre und präventive Gefäßmedizin e.V. (DGG); Deutsche Gesellschaft für Innere Medizin (DGIM); Deutsche Gesellschaft für Neurologie (DGN); Deutsche Gesellschaft für Pathologie (DGP); Deutsche Ophthalmologische Gesellschaft (DOG); Deutsche Röntgengesellschaft e.V. (DRG); Österreichische Gesellschaft für Rheumatologie und Rehabilitation (ÖGR); Schweizerische Gesellschaft für Rheumatologie (SGR). J. H. Schirmer · P. M. Aries · K. Balzer · P. Berlit · T. A. Bley · F. Buttgereit · M. Czihal · C. Dechant · C. Dejaco · U. Garske · J. Henes · J. U. Holle · K. HollUlrich · P. Lamprecht · B. Nölle · F. Moosig · J. Rech · K. Scheuermann · M. Schmalzing · W. A. Schmidt · M. Schneider · H. Schulze-Koops · N. Venhoff · P. M. Villiger · T. Witte · M. Zänker · B. Hellmich 1 Klinik für Innere Medizin I, Sektion Rheumatologie, Exzellenzzentrum Entzündungsmedizin, Universitätsklinikum Schleswig-Holstein, Campus Kiel, Kiel, Deutschland; 2 Rheumatologie im Struenseehaus, Hamburg, Deutschland; 3 Abteilung für Gefäßund Endovaskulärchirurgie, St. Marien Hospital, GFO Kliniken Bonn, Bonn, Deutschland; 4 Deutsche Gesellschaft für Neurologie, Berlin, Deutschland; 5 Institut für Diagnostische und Interventionelle Radiologie, Universitätsklinikum Würzburg, Würzburg, Deutschland; Medizinische Klinik mit Schwerpunkt Rheumatologie und Klinische Immunologie (CCM), Charité Universitätsmedizin Berlin, Berlin, Deutschland; 7 Sektion Angiologie – Gefäßzentrum, Medizinische Klinik und Poliklinik IV, Klinikum der Universität München, München, Deutschland; 8 Sektion Rheumatologie und klinische Immunologie, Medizinische Klinik und Poliklinik IV, Klinikum der Universität München, München, Deutschland; 9 Klinische Abteilung für Rheumatologie und Immunologie, Medizinische Universität Graz, Landesweiter Dienst für Rheumatologie, Südtiroler Sanitätsbetrieb, Graz, Österreich; 10 Deutsche Rheuma-Liga Bundesverband e.V., Bonn, Deutschland; Medizinische Klinik II, Rheumatologie, Universitätsklinikum Tübingen, Tübingen, Deutschland; 12 Rheumazentrum Schleswig-HolsteinMitte, Neumünster, Deutschland; 13 Pathologie – Hamburg, Labor Lademannbogen MVZ, Hamburg, Deutschland; 14 Klinik für Rheumatologie und klinische Immunologie, Universität zu Lübeck, Lübeck, Deutschland; 15 Klinik für Ophthalmologie, Universitätsklinikum SchleswigHolstein, Campus Kiel, Kiel, Deutschland; Medizinische Klinik 3, Rheumatologie und Immunologie, Universitätsklinikum Erlangen, Erlangen, Deutschland; Medizinische Klinik II, Rheumatologie/Klinische Immunologie, UniversitätsklinikumWürzburg, Würzburg, Deutschland; 18 Rheumatologie und klinische Immunologie, Immanuel Krankenhaus Berlin-Buch, Berlin, Deutschland; 19 Poliklinik und Funktionsbereich für Rheumatologie, UniversitätsklinikumDüsseldorf, Düsseldorf, Deutschland; 20 Klinik für Rheumatologie und klinische Immunologie, Vaskulitis-Zentrum Freiburg, Department Innere Medizin, Universitätsklinikum Freiburg, Medizinische Fakultät, Albert-Ludwigs-Universität Freiburg, Freiburg, Deutschland; 21 Universitätsklinik für Rheumatologie, Immunologie und Allergologie, Inselspital, Bern, Schweiz; 22 Klinik für Immunologie und Rheumatologie, Medizinische Hochschule Hannover, Hannover, Deutschland; 23 Abteilung für Innere Medizin, Immanuel KlinikumBernau Herzzentrum Brandenburg, Bernau, Deutschland; Medizinische Hochschule Brandenburg, Neuruppin, Deutschland; 25 Klinik für Innere Medizin, Rheumatologie und Immunologie, Vaskulitiszentrum Süd, Medius Klinik, Kirchheim unter Teck, Deutschland

OBJECTIVES To identify independent risk factors for permanent visual loss (PVL) in patients with giant cell arteritis (GCA), with a special focus on sonographic findings of the temporal, carotid and subclavian/axillary arteries, and on established scoring systems of ischaemia risk assessment. METHODS Consecutive patients with a diagnosis of GCA between 2002 and 2013 were retrospectively identified from a prospectively maintained database. Data on clinical characteristics including ophthalmological findings, laboratory values, and sonographic findings of the temporal, carotid an axillary arteries were extracted. CHADS2- and CHA2DS2-VASc-score were calculated. Clinical, laboratory and sonographic characteristics of patients with and without PVL were compared. Multiple logistic regression models were calculated to identify variables independently associated with PVL. RESULTS One-hundred-fifty-two patients were included in the analysis. PVL occurred in 30.2% of patients, with anterior ischaemic optic neuropathy as predominant underlying cause (91.3%). The frequency of PVL was strongly dependent on the age at diagnosis, with a significant increase after the age of 70 years. In multivariate analysis, axillary artery vasculitis with an odds ratio (OR) of 0.3 and constitutional symptoms with an OR of 0.1 were negatively associated with PVL. A CHADS2-score of 1 (OR 10.7) or ≥2 (OR 25) was associated with a significantly increased risk of PVL. CONCLUSIONS The risk of PVL secondary to GCA increases with age but is lower in patients presenting with constitutional symptoms and/or exhibiting axillary artery involvement. The CHADS2-score may help to discriminate patients with low vs. high risk of PVL.

ZusammenfassungDas Raynaud-Syndrom ist eine durch Kälte oder Stress ausgelöste reversible Entfärbung der Akren, typischerweise einzelner Finger, durch einen Vasospasmus. Etwa 6 % der Bevölkerung sind betroffen, Frauen häufiger als Männer. Gesichert wird ein Raynaud-Syndrom durch die typische Anamnese und klinische Untersuchung. Bei 80 % der Betroffenen ist von einem primären Raynaud-Syndrom auszugehen. Insbesondere bei spätem Beginn und ausgeprägter Symptomatik kann ein sekundäres Raynaud-Phänomen vorliegen. Hierfür sind viele mögliche, oft seltene Ursachen beschrieben. Die Differenzialdiagnostik bedarf einer detaillierten Anamnese und Untersuchung. Die wichtigsten Instrumente bei Verdacht auf eine entzündlich rheumatische Erkrankung sind Antinukleare Antikörper und die Kapillarmikroskopie. Sind beide unauffällig, ist die Wahrscheinlichkeit sehr gering. Bei Nachweis spezifischer Autoantikörper oder kapillarmorphologischer Veränderungen werden Verlaufskontrollen empfohlen. Der Gefäßultraschall ist die am häufigsten genutzte angiologisch technische Diagnostik.

Der potentielle Stellenwert der hochauflösenden Kontrastmittelsonografie (CEUS) in der Aktivitätsbeurteilung der Takayasu-Arteriitis soll evaluiert werden.

Nailfold capillary microscopy is an imaging procedure of great diagnostic value in the differential diagnosis of microvascular skin pathologies. It enables the detection of specific changes in systemic diseases, especially connective tissue diseases, and is essential forthe differentiation between primary and secondary Raynaud's phenomenon. This interdisciplinary review provides a current overview of the most important applications of nailfold capillary microscopy as well as the chances and limitations of this procedure. Also it gives an outlook on the expected future development.

OBJECTIVES:We aimed to determine the diagnostic accuracy of B-mode compression sonography of the temporal arteries (tempCS) and B-mode sonographic measurement of the axillary artery intima media thickness (axIMT) for the diagnosis of giant cell arteritis (GCA). METHODS:After having established measurement of tempCS and axIMT in our routine diagnostic workup, 92 consecutive patients with a suspected diagnosis of GCA were investigated. Clinical characteristics were recorded and wall thickening of the temporal arteries (tempCS) and axillary arteries (axIMT) was measured (mm). Using the final clinical diagnosis as the reference standard, receiver operator characteristics (ROC) analysis was performed. In a subgroup of 26 patients interobserver agreement was assessed using Spearman's rank correlation. RESULTS:Cranial GCA, extracranial GCA, and combined cranial/extracranial GCA were diagnosed in 18, 7, and 9 individuals, respectively. For the diagnosis of cranial GCA, tempCS had an excellent area under the curve (AUC) of 0.95, with a cut-off of ≥0.7 mm offering a sensitivity and specificity of 85% and 95%. The AUC of axIMT for the diagnosis of extracranial GCA was 0.91 (cut-off ≥1.2 mm: sensitivity and specificity 81.3 and 96.1%). Applying a combined tempCS/axIMT cut-off of ≥0.7mm/1.2 mm, we calculated an overall sensitivity and specificity for the final clinical diagnosis of cranial and/or extracranial GCA of 85.3% and 91.4%. Interobserver agreement was strong for both parameters assessed (Spearman's rho 0.72 and 0.77, respectively). CONCLUSIONS:The combination of tempCS/axIMT allows objective sonographic assessment in suspected GCA with promising diagnostic accuracy.

OBJECTIVES:To determine sex differences in the clinical spectrum and disease pattern of cranial and extracranial giant cell arteritis (GCA).METHODS:Data on 153 consecutive patients with a confirmed diagnosis of GCA between 2002 and 2013 were retrospectively obtained from our database. For every male patient, two age-matched female patients were identified. Clinical symptoms, vascular physical examination findings, laboratory values, and the disease patterns as assessed by colour duplex sonography of the temporal and axillary arteries were compared between women and men. Subgroup analyses were performed for patients aged 50-69 years and ≥70 years at disease onset.RESULTS:No significant differences between sexes were noted with regard to cranial GCA. Female patients significantly more frequently had axillary artery involvement (48.9 vs. 27.5%, p=0.03), a difference mainly driven by a higher rate of axillary artery involvement in women ≥70 years of age (38.6 vs. 4.5%, p<0.01). Women aged 70 years or older significantly more frequently had axillary artery stenosis (27.3 vs. 0%, p<0.01), symptoms of upper extremity ischaemia (20.5 vs. 0%, p<0.01), and polymyalgia rheumatica (36.4 vs. 9.1%, p=0.02) compared to men. Significant sex differences were observed with regard to the frequency of anaemia and the mean platelet count.CONCLUSIONS:In GCA involvement of the cranial arteries does not differ between sexes. Female patients with GCA significantly more frequently exhibit extracranial (i.e. axillary) arterial involvement than men.

Objectives: Recent findings suggest that autoimmune disorders predispose to a diminished capacity to taste and smell. This has been shown for patients with systemic lupus erythematosus as well as for patients with rheumatoid arthritis (RA). Granulomatosis with polyangiitis (GPA), with its particular manifestations in the upper respiratory tract, may therefore have an even higher impact on these senses. The aims of this study were to evaluate the gustatory and olfactory function in patients with GPA, to compare them to sex- and age-matched healthy controls, and to correlate these findings with their GPA disease severity. Method: Patients with established GPA were analysed by standardized assessments for gustatory and olfactory functions and examined for disease activity, stage of disease, and treatment. Results: Forty-four GPA patients were tested for their chemosensory functions. Compared to age- and sex-matched healthy controls, GPA patients showed significantly decreased olfactory scores along with diminished scores for their gustatory functions. The diminished sense of smell in GPA patients correlated significantly with elevated C-reactive protein (CRP) values whereas the gustatory impairment correlated with the duration and extent of the disease. Conclusions: Our results indicate that olfactory and gustatory functions are significantly decreased in GPA. As the olfactory function of these patients was comparable to patients with RA, chemosensory impairment may not simply be a consequence of the involvement of the upper respiratory tract, but rather a common complication of systemic autoimmune diseases.

Sklerodermiforme Hautveränderungen können neben einer systemischen Sklerose auch im Rahmen etlicher anderer, z.B. genetisch, medikamentös oder infektiös vermittelter Erkrankungen auftreten.

Adult-onset Still's disease is a rare inflammatory systemic disease. Cardinal symptoms/manifestations are fever, arthralgias or arthritis, myalgias, the typical skin rash, sore throat, hepatosplenomegaly, lymphadenopathy and serositis. Several other symptoms and organ involvements are possible. The clinical picture is variable with mild to life-threatening courses. The disease is self-limiting, intermittently active or chronic. Because of the lack of a defined diagnostic test the diagnosis of AOSD can only be made after exclusion of several differential diagnoses in particular of infectious, malignant and autoimmune origin. For therapy non-steroidal anti-inflammatory drugs, glucocorticoids, disease modifying antirheumatic drugs and biologics can be used.

The role of Th17 cells in the pathogenesis of human autoimmune diseases is elusive. To gain insights into the role of Th17 cells in human autoimmune diseases, the authors analysed Th17 cells in patients with prototypic autoimmune diseases such as rheumatoid arthritis (RA) and psoriatic arthritis (PsA). Th17 cells were analysed in well-defined homogeneous cohorts …

Zusammenfassung Die Kapillarmikroskopie ist ein einfaches Verfahren, das eine den Patienten nicht belastende Beurteilung der Mikrozirkulation zulässt. Obwohl es sich um eine sehr alte Methode handelt, findet sie erst in den letzten Jahren wieder eine zunehmende Verbreitung in der klinischen Anwendung. Die Methode besitzt einen großen Stellenwert bei der differenzialdiagnostischen Einordnung des Raynaud-Phänomens.

CASE REPORT A 72 year old man presented with multiple purpuric and necrotising skin eruptions (sized 2–20 mm) covering mainly the extremities and, to a lesser extent, the trunk. He had noticed a weight loss of 12 kg in the past 2 months. There was a history of smoking with chronic obstructive pulmonary disease and partial colectomy because of a benign adenoma. Laboratory investigations showed a normal erythrocyte sedimentation rate (9 mm/1st h), but C reactive protein was raised (43 mg/l). Serological markers, such as antinuclear antibodies, antineutrophil cytoplasmic antibodies, cryoglobulins, and hepatitis B and hepatitis C were excluded. A diagnostic procedure was performed to exclude a neoplasm, but, chest radiography, abdominal ultrasonography, gastroscopy, and colonoscopy were normal. Prednisone was

Background and objective: Adult-onset Still's disease (AOSD) is a rare entity. The course of the disease can be mild or severe, acute or chronic. The intention of this survey was to evaluate the longterm efficacy of TNF blockade in patients with severe and active AOSD.Patients and methods: Eight patients with the diagnosis of AOSD, according to the diagnostic criteria developed by Yamaguchi in 1992, and pretreatment with either high dosage steroid or more intensive immunosuppressive therapy were treated with infliximab in a dosage of 3-5 mg/kg at time points-during week 0, 2 and 6. Later on, the treatment regimen was adapted to the individual needs of the patients. The follow-up period was between one and five years.Results: Seven patients responded to treatment with infliximab. Symptoms like fever, arthralgia, hepato-splenomegaly and serological parameters instantly improved. Five of these patients remained in remission over years after discontinuation of therapy. One of the responding patients needed permanent intensive treatment with TNF-blockers to control his severe chronic arthritis. Three patients experienced infusion reactions. One responding patient was therefore switched to etanercept and kept on this therapy. The one patient, who had not responded to infliximab also had no benefit from consecutive treatment with etanercept or adalimumab.Conclusion: Anti-TNF therapy can have a lasting beneficial effect on the course of AOSD. Five of eight patients remained in remission even after termination of therapy.