Sven Süfke

IgE-Specific Immunoadsorption for Treatment of Recalcitrant Atopic Dermatitis

Report of Cases Two consecutively recruited patients with atopic dermatitis (AD) (a man in his 50s [patient 1] and a man in his 40s [patient 2]) displayed high serum IgE levels of 40 848 and 111 696 μg/L (to convert to milligrams per liter, multiply by 0.001), Scoring Atopic Dermatitis (SCORAD) scores of 66 and 77, respectively, and a history of no substantial or long-term SCORAD reduction after use of topical corticosteroids and calcineurin inhibitors, UV light therapy, systemic corticosteroids, and cyclosporine A.

Adjuvant treatment of severe/refractory bullous pemphigoid with protein A immunoadsorption: Immunoadsorption in bullous pemphigoid

While depletion of circulating autoantibodies using immunoadsorption (IA) is an established therapeutic approach in patients with pemphigus vulgaris, IA has only sporadically been used in other autoimmune bullous disorders. Although bullous pemphigoid (BP) usually responds well to topical and systemic corticosteroids, rapid depletion of serum autoantibodies may be an effective adjuvant treatment option in patients with severe and/or refractory disease.

A hypertensive patient presenting with paraneoplastic perimyocarditis and myositis due to pheochromocytoma

Hypertension is a frequent condition in the general population, and may lead to severe end organ damage. Various endocrine disorders cause hypertension, requiring specific attention and treatment. Paragangliomas (PGLs) are catecholamine producing chromaffin cell tumors developing from the sympathetic and parasympathetic ganglia throughout the abdomen and head and neck area, respectively. When arising from the adrenal gland, PGLs are referred to as pheochromocytomas (PHEOs). PGLs/PHEOs have an incidence of 1–2 per 100,000 and a prevalence of 0.1–0.4% in patients with hypertension [1]. They synthesize and secrete catecholamines and more rarely dopamine, causing a variety of clinical symptoms. The classic triad of symptoms consists of episodic headache (90%), sweating (60–70%) and heart palpitations. Moreover, most patients with PGL/PHEO suffer from paroxysmal or sustained hypertension. However, 5–15% of patients present with normal blood pressure. Other symptoms might be dyspnea, weakness, weight loss, psychiatric disorders or alterations in carbohydrate metabolism (hyperglycemia, insulin resistance). Several genetic disorders are associated with PHEOs (MEN 2a/b, von Hippel– Lindau disease, SDH gene mutations, MAXmutations, Neurofibromatosis Type 1). We here report the case of a hypertensive patient with a PHEO presentation with myositis and perimyocarditis. A 76 year-old female patient with a history of hypertension was admitted to the hospital with progressive severe pain and weakness of the shoulder girdle and thighs for several weeks. Laboratory

Adjuvante Behandlung des schweren/refraktären bullösen Pemphigoids mit Protein-A-Immunadsorption: Immunadsorption beim bullösen Pemphigoid

Mittels Immunadsorption (IA) können Immunglobuline und Immunkomplexe aus dem Plasma entfernt werden. Während dieses therapeutische Verfahren beim Pemphigus vulgaris bereits etabliert ist, wird es bei anderen blasenbildenden Autoimmundermatosen bislang nur sporadisch eingesetzt. Das bullöse Pemphigoid (BP) spricht zwar meist gut auf eine Therapie mit topischen und systemischen Kortikosteroiden an, jedoch könnte bei Patienten mit ausgedehnten Läsionen oder bei einem Rezidiv die rasche Reduktion der pathogenen Autoantikörper eine effektive adjuvante Therapie darstellen.

Safety Aspects of Immunoadsorption in IgG Removal Using a Single‐Use, Multiple‐pass Protein A Immunoadsorber (LIGASORB): Clinical Investigation in Healthy Volunteers

Therapeutic apheresis and immunoadsorption are used to deplete efficiently pathogenic autoantibodies in crises in several acute autoimmune driven diseases. This prospective, non‐comparative cohort study was conducted at a single study center under standardized conditions in 10 healthy volunteers. Efficient immunoglobulin G (IgG) removal (−86% versus baseline) was achieved after 3 apheresis treatments on 3 consecutive days. The treatments were well tolerated. Safety laboratory parameters did not show unexpected or pathological changes. The effects were transient, with most parameters exhibiting complete recovery between treatments. Minimal complement activation and moderate transient fibrinogen depletion were observed. Immunoadsorption with LIGASORB® provides a safe and effective treatment alternative to TPE in acute episodes of peripheral neurological diseases mediated by pathogenic IgG autoantibodies.