Bettina Mock

Interferon-αcon-1 Treatment of Three Patients with Severe Glucocorticoid-Dependent Asthma

We report herein the therapeutic effect of interferon (IFN)-αcon in three patients with severe persistent asthma and long-term oral glucocorticoid treatment. IFN-αcon (9 µg) administered subcutaneously thrice a week over a period of more than 24 months led to a substantial clinical improvement with regard to the number of daily asthma attacks, nighttime disturbance, emergency visits and hospitalizations. In addition, lung function and exercise capacity improved. At the same time, treatment with IFN allowed discontinuation of the daily glucocorticoid dose in all patients for the first time in more than 8 years. Our findings suggest that IFN-αcon leads to a significant clinical improvement while at the same time allowing reduction and discontinuation of the glucocorticoid treatment in severe persistent glucocorticoid-dependent asthma.

Interferon-γ-1b: Therapeutic Option in Advanced Idiopathic Pulmonary Fibrosis?

We read with great interest the paper by Nathan et al. [1] on using interferon (IFN)-Á-1b as a potential therapeutic option in delaying the progression of idiopathic pulmonary fibrosis (IPF)/usual interstitial pneumonitis (UIP). This retrospective study investigates the pulmonary function and diffusion capacity of 22 patients with IPF, most of whom had histologically proven UIP. The data presented suggest that IFN-Á-1b may have an overall beneficial effect on patients with IPF/UIP, in particular on patients with advanced disease, which is in contrast to the results from other studies on patients with mild-tomoderate disease [2]. Herein, we present a patient with advanced disease refractory to standard immune suppressive treatment with a significant clinical and lung functional response to IFN-Á-1b. A 59-year-old man was admitted to our hospital in October 1999 due to increasing exertion dyspnea. Physical examination showed bilateral, basilar end-inspiratory crackles and digital clubbing. Initial lung function tests showed a mild restrictive pattern (total lung capacity: 73% of predicted; vital capacity: 79%), and a moderately impaired diffusing capacity [carbon monoxide transfer factor (DLCO) 60%; DLCO corrected for alveolar volume (DLCO/VA) 74% predicted]. Arterial blood gas analysis at rest was normal while breathing room air, but hypoxemia developed following exercise (PaO2 59 mm Hg), whereas the alveolar arterial oxygen gradient (AaDPO2) increased from 23 to 42 mm Hg. The high-resolution computed tomography scan revealed bilateral, basilar subpleural reticular opacities and honeycombing without ground-glass opacities, most prominent in the basal regions of the lung. Since clinical symptoms and lung function continued to worsen, immunosuppressive treatment with oral methylprednisolone (20– 60 mg) was commenced in January 2000, but failed to cause any clinical or functional improvement (fig. 1). In August 2002, corticosteroid treatment was supplemented by 150 mg cyclophosphamide and

Might wasp venom desensitization induced Th2 to Th1 shift cause pregnancy failure

The case of a 28‐year‐old woman under wasp venom desensitization having a premature birth in her 24th week of pregnancy 16 days after the last injection is described. To test the hypothesis that a special profile of immune cells in the decidua may trigger abortions, placental and decidual tissue sections were stained with antibodies against T cells (CD3), cytotoxic cells (CD8), natural killer cells (CD56), and mast cells, and an in‐situ‐hybridization was performed for tumor necrosis factor‐α (TNF‐α). CD56+ Natural killer cells were the dominating population. In earlier analyses of healthy first trimester decidua the percentage of NK cells and T cells was in a similar range, but the CD8:CD3 ratio was only 2.2% in contrast to 27% in the present case. Mast cells, which are known to be able to secrete abortogenic TNF‐α, were only detectable in the decidua (10 cells/mm2) and decidua sections were TNF‐α positive. Since SIT induces a shift of the interleukin and functional profile from a Th2 type towards a Th1 type, and pregnancy is dependent on a Th2 pronounced profile, SIT may trigger abortions or immature births. This is supported by the present results and might have happened in this case.

Still-Syndrom des Erwachsenen

Zusammenfassung□ HintergrundDas Still-Syndrom des Erwachsenen ist eine seltene entzündlich-rheumatische Erkrankung mit systemischer Beteiligung unbekannter Ätiologie. Leitsymptome sind Fieber, Arthralgien/ Arthritiden und ein stammbetontes, meist makulöses Exanthem.□ Eigenes Krankengutund ErgebnisseVon 1980 bis 1996 sind an der eigenen Einrichtung acht Patienten (drei Männer, funf Frauen) mit adultem Still-Syndrom diagnostiziert und behandelt worden. Der Zeitraum zwischen erstem Symptom und Diagnose variierte von zwei bis 86 Monaten. Dies ist auf das Fehlen spezifischer laborchemischer Marker und die vielen differential diagnostischen Möglichkeiten, die ausgeschlossen werden müssen, zurückzuführen. Der in den letzten Jahren in der Literatur beschriebene erhöhte Serumferritinspiegel bei dieser Erkrankung fand sich bei sechs unserer acht Patienten und ist im akuten Stadium ein zusätzlicher Anhaltspunkt für die Diagnose. Unter effektiver Therapie normalisierte er sich rasch. Neben dem Stellen wert des Serumferritins als Diagnose- und Aktivitätsparameter werden unsere therapeutischen Strategien diskutiert und mit den Literaturangaben verglichen.□ SchlußfolgerungBei unklarem Fieber in Verbindung mit Exanthem und Arthralgien sollte ein adultes Still-Syn drom in die Differential diagnose einbezogen werden, dem Patienten können dadurch invasive diagnostische Eingriffe erspart werden. Im akuten Stadium ist ein erhöhter Serumferritinspiegel ein zusätzlicher Hinweis auf das Vorliegen eines Still-Syndroms.Summary□ BackgroundAdult onset Still’s disease (AOSD) is an uncommon, systemic, inflammatory disorder of unknown etiology, characterized by the trial offever, arthritis and rash.□ Patients and ResultsWe describe 8 cases of AOSD (3 male, 5 female) diagnosed and treated in the Department of Rheumatology from 1980 to 1996. The delay in reaching a firm diagnosis was between 2 and 86 months, due to both lack of specific serum markers and the abundance of possible differential diagnoses. Our therapeutic strategies and results are presented and the value of obtaining serum ferritin levels for both diagnosis and follow-up studies is discussed. The patients data are compared to those of the world’s literature on AOSD.□ ConclusionThe differential diagnosis of fever of unknown origin should always include AOSD, because these patients could be spared from invasive and unnecessary diagnostic measures. Increased serum ferritin levels are of particular value in the diagnosis of acute AOSD and the normalization of the serum ferritin value is a reliable indicator of therapeutic success.BACKGROUND Adult onset Stills disease (AOSD) is an uncommon, systemic, inflammatory disorder of unknown etiology, characterized by the triad of fever, arthritis and rash. PATIENTS AND RESULTS We describe 8 cases of AOSD (3 male, 5 female) diagnosed and treated in the Department of Rheumatology from 1980 to 1996. The delay in reaching a firm diagnosis was between 2 and 86 months, due to both lack of specific serum markers and the abundance of possible differential diagnoses. Our therapeutic strategies and results are presented and the value of obtaining serum ferritin levels for both diagnosis and follow-up studies is discussed. The patients data are compared to those of the worlds literature on AOSD. CONCLUSION The differential diagnosis of fever of unknown origin should always include AOSD, because these patients could be spared from invasive and unnecessary diagnostic measures. Increased serum ferritin levels are of particular value in the diagnosis of acute AOSD and the normalization of the serum ferritin value is a reliable indicator of therapeutic success.

POSTINFEKTIOSE BEINSCHWELLUNGEN BEIDSEITS UND LUMBOISCHIALGIEN

r f , E in 23j~ihriger Student bemerkte eine Woche nach einem Infekt der oberen Luftwege dumpfe, tiefsitzende P,.a und drei Tage sp~iter ziehende Schmerzen sowie Schwellungsgefa in beiden Waden. Klimsch fanden sich keine signifikanten U m fangsdifferenzen der Extremit~iten, aber geringe pr~itibiale Odeme. Laborchemisch waren deutlich erh6htes CrP (224 mg/l), beschteunigte BKS (60/1. h) und erh6htes Fibrinogen (6,75 g/l) auffiUig. Protein C und S waren nicht vermindert. Mittels P C K Analyse und P, estriktionsenzymandauung konnte eine angeborene I~esistenz (Mutation) gegena aktiviertem Protein C nachgewiesen werden. Die Phiebographie beider Beine zeigte links eine ausgedehnte Thrombose der tiefen Unterschenkelvenen, der Vena femoralis profunda sowie der Beckenvenen und rechts einen kompletten Verschlufl der Vena femoralis und ebenfalls der Beckenvenen mit Nachweis von Kollateralkreisl~iufen (Abbildung 1). [m Abdomen-MR.T (koronarer Schnitt /T2-Wichtung) Abbildung I