Husten, Atemnot und B-Symptome bei einer 40-jährigen Frau

Zusammenfassung: Der Morbus Castleman ist eine seltene polyklonale, lymphoproliferative Erkrankung, bei der Mediatoren von Entzündungsreaktionen, v.a. Interleukin-6, eine wichtige pathophysiologische Rolle spielen. Zur Behandlung dieser Krankheit ist keine Standardtherapie etabliert. Wir berichten über den Fall einer 40-jährigen HIV-negativen Patientin mit primär pulmonaler Manifestation eines HHV-8-negativen, plasmazellreichen multizentrischen Morbus Castleman. Verschiedene Therapieversuche mit Immunmodulatoren wurden durchgeführt, bevor eine Behandlung mit dem Interleukin-6-Rezeptor-Antikörper Tocilizumab begonnen wurde. Seit 5Jahren ist der klinische Verlauf unter fortgesetzter Tocilizumabgabe stabi

Husten, Atemnot und B-Symptome bei einer 40-jährigen Frau

Multicentric Castleman's disease (MCD) is a rare polyclonal lymphoproliferative disorder that is typically accompanied by an overproduction of circulating cytokines (mainly interleukin-6). We here report the case of a 40-year-old HIV-negative woman with pulmonary manifestation of MCD. There is no standard treatment for MCD. In our patient, various treatment courses with immunomodulatory drugs were unsuccessful. Finally, treatment with the interleukin-6 receptor antibody tocilizumab has resulted in continual clinical improvement over the last 5 years. Der Morbus Castleman ist eine seltene polyklonale, lymphoproliferative Erkrankung, bei der Mediatoren von Entzündungsreaktionen, v. a. Interleukin-6, eine wichtige pathophysiologische Rolle spielen. Zur Behandlung dieser Krankheit ist keine Standardtherapie etabliert. Wir berichten über den Fall einer 40-jährigen HIV-negativen Patientin mit primär pulmonaler Manifestation eines HHV-8-negativen, plasmazellreichen multizentrischen Morbus Castleman. Verschiedene Therapieversuche mit Immunmodulatoren wurden durchgeführt, bevor eine Behandlung mit dem Interleukin-6-Rezeptor-Antikörper Tocilizumab begonnen wurde. Seit 5 Jahren ist der klinische Verlauf unter fortgesetzter Tocilizumabgabe stabil

Randomised controlled trial for emphysema with a selective agonist of the γ-type retinoic acid receptor

Palovarotene is an oral gamma-selective retinoid agonist. In animal emphysema models, palovarotene reduced inflammation, promoted structural repair and functional improvement. REPAIR (Retinoid treatment of Emphysema in Patients on the alpha(1)-antitrypsin International Registry), was an investigator-initiated, double-blind, placebo-controlled randomised study to assess the safety and efficacy of 5 mg.day(-1) palovarotene given for 1 year to 262 patients with severe alpha(1)-antitrypsin deficiency and emphysema confirmed by computed tomography. Change in volume-adjusted 15th percentile point lung density from baseline in 1 year was the primary endpoint; functional end-points were also regularly assessed. We randomly assigned 133 and 129 patients to placebo or palovarotene, respectively. Both groups were well matched for all baseline characteristics, including respiratory medications. 88% and 85% of patients completed 1 year of treatment with placebo and palovarotene, respectively. Palovarotene was generally well tolerated. In the study completers population, the placebo-corrected difference of lung density was -0.45 HU at week 28 (p=0.64) and -0.25 HU at week 52 (p=0.94). A nonsignificant treatment difference in most functional parameters of the lung in favour of the drug was observed over time suggesting potential pharmacological effects of palovarotene. Palovarotene 5 mg.day(-1) over 1 yr failed to show a significant benefit on lung density in moderate-to-severe emphysema secondary to severe alpha(1)-antitrypsin deficiency

Autologous fibrin sealant reduces the incidence of prolonged air leak and duration of chest tube drainage after lung volume reduction surgery: a prospective randomized blinded study

OBJECTIVE: Prolonged air leak is reported in up to 50% of patients after lung volume reduction surgery. The effect of an autologous fibrin sealant on the intensity and duration of air leak and on the time to chest drain removal after lung volume reduction surgery was investigated in a randomized prospective clinical trial. METHODS: Twenty-five patients underwent bilateral thoracoscopic lung volume reduction surgery. In each patient, an autologous fibrin sealant was applied along the staple lines on one side, whereas no additional measure was taken on the other side. Randomization of treatment was performed at the end of the resection on the first side. Air leak was assessed semiquantitatively by use of a severity score (0 = no leak; 4 = continuous severe leak) by two investigators blinded to the treatment. RESULT: Mean value of the total severity scores for the first 48 hours postoperative was significantly lower in the treated group (4.7 +/- 7.7) than in the control group (16.0 +/- 10.1) (P < .001), independently of the length of the resection. Prolonged air leak and mean duration of drainage were also significantly reduced after application of the sealant (4.5% and 2.8 +/- 1.9 days versus 31.8% and 5.9 +/- 2.9 days) (P = .03 and P < .001). CONCLUSIONS: Autologous fibrin sealant for reinforcement of the staple lines after lung volume reduction surgery significantly reduces prolonged air leak and duration of chest tube drainage