[IgG4-related systemic disease with autoimmune pancreatitis and lung involvement primarily presenting as pancreatic cancer with pulmonary metastases]. / IgG4-assoziierte Autoimmunpankreatitis und Lungenerkrankung mit der primären, klinischen Präsentation eines pulmonal metastasierten Pankreaskarzinoms.

BACKGROUND: After the first case publication using the term "autoimmune pancreatitis" in 1995 and the successful treatment with steroids we now can distinguish between two clinical und histopathological forms of autoimmune pancreatitis. Type 1 autoimmune pancreatitis (AIP) is usually part of an IgG4-related systemic disease. AIP Typ 2 is an IgG4-independent pancreatic disease. For both entities pancreas cancer is the most important differential diagnosis. CASE REPORT: We report the case of an 82-year-old male patient who primarily presented with obstructive jaundice. Computed tomography (CT) revealed the typical image of a small cancer of the head of the pancreas with pulmonary metastases. After endoscopic drainage of the bile duct a CT-guided biopsy of a pulmonary nodule was performed in which cancer was ruled out. Next the patient was treated with steroids because of "tumour-associated cachexia". In the follow-up the mass in the head of the pancreas like the lung nodules had surprisingly disappeared. In the complete work-up the immune histochemical staining of the lung biopsy revealed subsequently a typical IgG4-associated inflammation. After termination of the therapy the disease relapsed as sclerosing cholangitis. CONCLUSION: The IgG4-related systemic disease with AIP can present as cancer of the pancreas with lung metastases. Extrapancreatic IgG4-positive histopathology and response to therapy with steroids can help to diagnose the disease in complex clinical presentations.

[Complications following cell therapy]. / Komplikationen nach Zelltherapie.

So far, the law in the Federal Republic of Germany still allows the injection of fresh-cell preparations from animals as a roborant to increase the vitality of the organism and to strengthen the body's immune defense system. The use of "sicca-cell" preparations was provisionally forbidden in 1987 by the Federal Health Organization (Bundesgesundheitsamt; BGA). Prohibition of fresh-cell injections would have exceeded the authority of this office, although the same serious reservations also applied in the case of this treatment method. Several publications that have appeared since 1955 have reported serious complications of this therapy, some life-threatening and some even lethal. Two further cases are now added: (1) A woman aged 69 had been receiving treatment with cell injections for 9 years. Immediately after an injection of sicca cells she collapsed and was hospitalized; 7 days thereafter she developed an ascending paralysis with increasing inability to swallow or breathe. She died 25 days after the injection as a consequence of central and peripheral respiratory failure. Autopsy revealed the alterations typical for acute Landry-Guillain-Barré-Strohl syndrome. (2) A 76-year-old healthy woman had been receiving treatment with fresh-cell preparations for several years. After an injection of cell suspensions a painful local swelling was observed. The symptoms were interpreted as the consequence of an iatrogenic local hematoma, and repeated punctures were performed to obtain blood. The patient was transferred to a surgical department for further therapy. Two days after the injection she suddenly died with signs of acute cardiovascular failure. Autopsy revealed the signs of a fulminating clostridial infection and also the characteristic signs of Landry-Guillain-Barré syndrome with involvement of the autonomic nervous system. In both cases the development of an inflammatory process in the peripheral nervous system could be interpreted as an immune-mediated allergic disease, related to the repeated injection of heterologous antigenic material containing nervous tissues. This hypothesis would also explain the two other cases already published and would be consistent with the observed perivenous leukoencephalopathy of the central nervous system. The human disease pictures correspond to the well-established animal models of EAEM (experimental allergic encephalomyelitis) and EAN (experimental allergic neuritis). The pathogenesis is discussed; the major role of the central and peripheral nervous system is stressed, with special reference to the risk of acute autonomic failure. The need for specific autopsy techniques for the investigation of the entire nervous system, including spinal cord, roots, spinal ganglia and peripheral nerves with sympathetic chains, is raised.

[Steroid-refractory amiodarone-induced pulmonary fibrosis. Clinical features and morphology after an amiodarone-free interval of 3 months]. / Steroidrefraktäre Lungenfibrose durch Amiodaron. Klinik und Morphologie nach einem amiodaronfreien Intervall von 3 Monaten.

Amiodarone-induced bilateral diffuse pulmonary fibrosis developed in a 47-year-old woman with idiopathic hypertrophic subvalvular aortic stenosis who had been treated with amiodarone (Cordarex), 300 mg daily for about 18 months. Although the drug was discontinued and cortisone treatment begun, the pulmonary fibrosis did not regress. When gentamicin (Refobacin) and cefotaxime (Claforan) were administered for suspected fibrosis-induced right-sided bronchopneumonia, gentamicin-induced acute tubular renal damage occurred, requiring dialysis. The patient died soon after of myocardial electro-mechanical dissociation. At necropsy there was, in addition to the idiopathic hypertrophic subvalvular cardiomyopathy, extensive bilateral pulmonary fibrosis, lamellar bodies in foam-cell intraalveolar macrophages, in hepatocytes and in the epithelium of the proximal and distal tubules. Although amiodarone had been discontinued three months previously, high concentrations of the drug were still present, especially in both lungs, fat tissue and the liver.