Panarteritis nodosa – ein „klassischer“ Fall

Diagnosis of polyarteritis nodosa is often delayed due to the vast heterogeneity of initial clinical symptoms. The case presented shows the clinical image of the disease, leading from the first symptoms up to verification of the diagnosis by sural-nerve biopsy. We discuss the classification of the disease among other types of vasculitis, the classification criteria proposed by the American College of Rheumatology (ACR) as well as current therapeutic options. This case underlines the interdisciplinary character of the disease, challenging neurologists, dermatologists, rheumatologists and orthopedics alike.     

Was ist „Schock“?

Zusammenfassung Schock, abgeleitet vom französischen choc, ist definiert als unzureichende Durchblutung vitaler Organsysteme unterschiedlicher Ausprägung mit nachfolgender Gewebehypoxie als Ausdruck des Missverhältnisses zwischen Sauerstofftransport und Sauerstoffaufnahme. Da der Schock mit einer hohen Morbidität und Letalität einhergeht, ist für die weitere zielgerichtete Therapie die Zuordnung der Schockform zu einer der 4 Hauptkategorien (kardiogener, obstruktiver, distributiver und hypovolämischer Schock) von besonderer Bedeutung. Pathophysiologisch stehen beim Schock die Aktivierung des sympathischen Nervensystems sowie renale, neurohumorale und lokale vasoregulatorische Mechanismen im Vordergrund. Ziel der Gegenregulationen ist die Wiederherstellung und Aufrechterhaltung eines ausreichenden Herzzeitvolumens mit suffizienter Organperfusion. Eine Laktatazidose ist Ausdruck einer zellulären Hypoxie. Die Minderperfusion und Ischämie führt zu einer systemischen inflammatorischen Reaktion, die, wird der Schockzustand überlebt, sekundäre Organschäden und ein Multiorganversagen verursachen kann.     

Autoimmunpankreatitis – „the new kid on the block“

Autoimmune pancreatitis (AIP) is a rare form of chronic pancreatitis which is characterized by diffuse or focal pancreatic enlargement, a lymphoplasmacytic infiltrate with a storiform fibrosis and a dramatic response to steroid treatment. AIP can be classified into three subtypes: lymphoplasmacytic sclerosing pancreatitis (LPSP; AIP type 1), idiopathic duct centric pancreatitis (IDCP; AIP type 2), and not otherwise specified (NOS). AIP type 1 is the pancreatic manifestation of IgG4-related disease which also involves other organs; AIP type 2 is a pancreas-specific, IgG4-negative disorder and is associated with inflammatory bowel disease. Epidemiological studies from Japan estimate an incidence of approximately 0.82 per 100,000 inhabitants; in Europe 5–6% of all patients with chronic pancreatitis are diagnosed with AIP. The pathomechanism of AIP remains enigmatic, but a mulitfactorial etiology with genetic predisposition and environmental influences has been proposed. Clinically, patients can present with jaundice, epigastric pain, weight loss and new-onset diabetes, thus, raising suspicion for pancreatic malignancy. A combination of the HISORt (Histology, Imaging, IgG4 Serology, other Organ involvement and Response to steroid therapy) criteria can be used to definitively diagnose AIP in most cases. The greatest challenge is to differentiate AIP from pancreatic cancer and cholangiocarcinoma, since it often presents with a pancreatic mass, lymphadenopathy and bile duct obstruction. The prognosis of AIP is usually favorable; in over 90% of cases remission can be induced by steroid treatment.     

Sind Mitglieder von Fibromyalgiesyndrom-Selbsthilfegruppen „anders“?

Background

No data were available on demographic and clinical characteristics of members of fibromyalgia syndrome (FMS) self-help groups in Germany.

Material and methods

The study was carried out from November 2010 to April 2011. A set of questionnaires was distributed by the German League Against Rheumatism and the German Fibromyalgia Association to members and to all consecutive FMS patients at nine clinical centres of different levels of care. The set included a self-developed questionnaire on demographic and medical data and on previously and currently used therapies, the patient health questionnaire (PHQ 4) and the fibromyalgia survey questionnaire.

Results

Members of FMS self-help groups (N?=?1,014) were older and reported a longer duration of chronic widespread pain, less anxiety and depression and a more frequent current use of aerobic exercise, relaxation training and complementary alternative medication than participants not affiliated with FMS self-help groups (N?=?630).

Conclusions

Membership in FMS self-help groups was associated with less psychological distress and a more frequent use of active self-management strategies.     

Dopamin in „Nierendosis“ beim akuten Nierenversagen – Wirksamkeit eindeutig widerlegt

Ohne Zusammenfassung     

Die „Empfehlungen zur Strukturierung der Herzschrittmacher- und Defibrillatortherapie“ – was können sie leisten?

Implantable devices in cardiac electrophysiology have developed from simple machines that prevent bradycardia to complex systems that treat malignant tachyarrhythmias and severe heart failure. Optimal therapeutic utilization of these systems requires profound knowledge of those who treat patients with devices. To account for that, the German Cardiac Society has published“Recommendations for the structure of cardiac pacemaker and defibrillator therapy” to achieve an expertise in 2 levels. These define topics for interdisciplinary education to ensure implantation and follow-up of high quality, as well as infrastructural requirements such as staff and operation rooms. The implementation of these guidelines offers a great opportunity for good quality in device therapy and may enhance the results of the compulsory German quality registry of the respective institution. The established courses of the German Cardiac Society to achieve competence in pacemaker and ICD therapy according to the curricula should be complemented by practical training in accredited centers. Minimum numbers for implantation and follow-up are necessary to ensure a continuously high quality.