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On blood lymph nodes and spleen cells of 4 patients with leukemic reticuloendotheliosis (LR) the binding of 125-J-labelled IgG aggregates as well as binding of 125J-labeled anti immunoglobulin sera was studied. Moreover the capacity to form spontaneous rosettes with sheep red blood cells to bind immune complement complexes as well as the proliferative response in the presence of PHA were investigated. On the surface of hairy cells a variety of immunoglobulins were demonstrable. IgG of both types was found to be the predominant surface bound immunoglobulin and evidence was obtained, that these antibodies were adsorbed from the serum via Fc-receptors. In all LR-cases the percentages of T-cell rosettes as well as the PHA responses were decreased when compared with the normal controls. The numbers of immune complement complex binding cells were in the range of the normal controls. Results are discussed with respect to the origin of hairy cells in LR.

Two cases of the development of acute myeloid leukemia (AML) after treatment with alkylating agents are reported. In Case 1, melphalan and then cyclophosphamide had been given for multiple myeloma. 46 months after onset of cytostatic treatment AML occurred, as confirmed cytochemically and by qualitative determination of urinary lysozyme. In Case 2, cyclophosphamide had been given for rheumatoid arthritis. After a latency of 34 months 'smouldering leukaemia' developed with an atypical monocytic leukaemic cell population. In a third case, multiple myeloma and monocytic leukaemia developed synchronously. The causative role of melphalan and cyclophosphamide in the development of AML seems securely established. Despite the risk of alkylating agents in the treatment of multiple myeloma or Hodgkin's disease causing AML, they should not be replaced, as other drugs have been shown to be less beneficial. On the other hand, alkylating agents should be used with great caution in the treatment of non-malignant diseases.

The Henoch-Schönlein-syndrome, or anaphylactoid purpura, is usually a childhood disease. The progess of this syndrome in adults was observed in 4 cases at our clinic. An elevated IgA level in the serum with a normal complement value seems to be of immunological interest. By immunohistological methods fibrin and IgA could be demonstrated in skin and kidney biopsies; in glomerulae C3c was observed, occasionally accompanied by Properdin-factor-B. These findings differentiate this disease from other allergic superficial vasculitids by the detection of IgG and complement; while at the same time some similarities are indicated to the so called "Berger Nephritis" with comparable kidney findings.

Goodpasture's syndrome occurred in six patients (four men and two women) aged 18-32 years. Haemoptysis, anaemia and abnormal urinary findings with erythrocyturia were the first signs of the disease in five instances, in one the renal signs predominated. In five patients the disease took a malignant course with uraemia developing within six weeks. One patient is in a spontaneous remission eleven months after the onset of the disease. Three patients died of recurrent haemoptysis (non-nephrectomised), two nephrectomised patients are still alive half a year and three and a half years later, respectively. A typical linear IgG-, beta1A, alpha2D and beta1E deposition along the glomerular capillaries was demonstrated immunohistologically in all subjects. Of three lung biopsies one also had typical IgG and beta1A-positive linear fluorescence along the alveolar basal membrane. Immunoglobulins with basal membrane antibody activity were eluted from renal homogenates in two patients, basal membrane antibody activity in serum was demonstrated only in one of three patients.

Cytochemical and electron-microscopic studies have been carried out on leukemic monocytes and ‘hairy cells’ (HC), ‘reticulosarcoma’ (RS) cells and cells of cases of ‘reticulosis’ and ‘reticulosarcoma cell leukemia’. Additional investigations included quantitative determinations of the urinary lysozyme excretion, skin window studies, testing of the phagocytosis of ferritin by HC, and labelling of the Fc receptors on HC at the ultrastructural level. Clear evidences against any cytological relationship among leukemic HC and monocytes have been provided. Further results argued also against the frequently stressed relationship among leukemic monocytes and RS cells. Cases of ‘RS cell leukemia’ and ‘reticulosis’ had to be reclassified as lymphosarcoma cell leukemia, acute lymphatic, and myeloblastic leukemias. Besides distinct ultrastructural differences among HC, RS cells, and lymphocytes, mainly gradual differences have been noted using cytochemical methods and by evaluating the phagocytosis of ferritin particles. A further common trait of HC, RS cells, and B lymphocytes seems to be the presence of surface Fc receptors. A more precise classification instead of the diagnosis ‘reticulosarcoma’ and ‘reticulosarcoma cell leukemia’ is required, and the use of the term ‘hairy cell’ leukemia is suggested instead of the misleading term ‘leukemic reticuloendotheliosis’.

On 6 patients suffering from rubeola or morbilli we examined the portion of IgG-containing cells and the number of DNA-synthesizing lymphocytes repeatedly during the course of the disease using immunofluorescent and autoradiographic methods. In virus infections we found a marked increase of both cellpopulations by comparison with normals. During the course of the disease we observed a simultaneous increase and decrease of both IgG-containing and DNA-synthesizing lymphocytes. At the beginning of that reaction, DNA-synthesizing cells prevailed upon IgG-positive cells. In 2 cases, a small portion of IgG-containing cells was found to be in the stage of DNA-synthesis

Es wurde der Wert immunhistologischer Untersuchungen fur die nephrologische Diagnostik an Hand von 50 Nierenbiopsien bei Patienten mit verschiedenen Nierenerkrankungen uberpruft. Dabei zeigte sich: 1. Der immunhistologische glomerulare Gamma-G-Globulinnachweis gelingt lediglich bei glomerulonephritischen Veranderungen und ermoglichen damit die Abgrenzung von Nierenlasionen bei chronischen Pyelonephritiden und essentieller Hypertonie. Das immunhistologische Bild der membranosen Glomerulonephritis zeigt eine spezifische granulare Fluorescenz entlang der Basalmembran der Glomerulumcapillaren und erlaubt damit eine Differenzierung des nephrotischen Syndroms anderer Atiologie mit immunhistologisch negativem Befund. Die Fruhdiagnose einer Nierenbeteiligung bei Patienten mit LED gelingt durch einen positiven Gamma-Globulinnachweis manchmal noch vor Auftreten lichtoptisch fasbarer pathologischer Veranderungen. Schlieslich weist ein negativer immunhistologischer Befund auf eine endgultige Ausheilung von akuten Glomerulonephritiden, die offenbar spater als die klinisch fasbare Normalisierung eintritt. Eine Unterscheidung von persistierender akuter und chronische wenig aktiver Glomeruloakuter und chronischer, wenig aktiver Glomerulonephritis ist hingegen nach unseren Erfahrungen immunhistologisch nicht moglich. 2. Daruber hinaus wird der pathogenetische Prozes morphologisch fasbar. Die Differenzierung einer immunologischen Grundkrankheit beim Zustandekommen von pathologischen Nierenveranderungen ist meist moglich, wobei besonders dem Gamma-G-Globulinnachweis Bedeutung zukommt. 3. Bei 3 Fallen (2 mit subakuter GLN und einem mit ungeklarter Proteinurie) fand sich eine scharfe, lineare Nachzeichnung der Basalmembran der Glomerulumcapillaren durch spezifische Fluorescenz, wie sie fur Basalmembranantikorper als charakteristisch beschrieben wird. 2 Falle von membranoser GLN und 1 Fall mit LED zeigten Veranderungen, die fur eine Immunkomplexnephritis sprechen. Diesen unterschiedlichen immunhistologischen Bildern durften verschiedene pathogenetische Mechanismen entsprechen, ohne das sich daraus bisher therapeutische Konsequenzen ergeben. 4. Komplementablagerung ohne analogen Gamma-Globulinnachweis wurde haufig in Nierenarteriolen beobachtet, besonders wenn eine Hypertonie bestand. Die pathogenetische Bedeutung dieser Befunde ist bisher ungeklart.

Zirkulierende Lymphocyten setzen sich aus funktionell heterogenen Zellen zusammen, welche mit morphologischen Methoden allein nur ungenugend differenziert werden konnen. Unter Verwendung immunologischer und autoradiographischer Methoden wurden Lymphocyten mit Determinanten leichter (kappa) Ketten, Lymphocyten mit Membranreceptoren fur Immun-Komplement-Komplexe, lymphatische Zellen mit cytoplasmatischem Immunglobulingehalt und Lymphocyten in DNS-Synthese bestimmt. Gegenuber Normalpersonen waren bei Patienten mit chronischer Lymphadenose Abweichungen an mehreren dieser Lymphocytenpopulationen nachweisbar. Die Ergebnisse werden im Hinblick auf das Konzept thymus- und knochenmarkabhangiger Lymphocyten diskutiert, deren Differenzierung mit immunologischen Methoden erleichtert wird.

Aus verschiedenen Organen isolierte lymphatische Zellen wurden auf das Vorhandensein von Immunglobulindeterminanten an ihrer Oberflache und auf Receptoren fur Antigen-Antikorper-Komplementkomplexe untersucht. Durch simultane Untersuchungen konnte gezeigt werden, das beide untersuchten Zellpopulationen in der Milz und im Lymphknoten in Ahnlicher Haufigkeit nachgewiesen werden konnen, wahrend sie in Thymuszellsuspensionen weitgehend fehlten. Wurden Lymphocyten mit Immunglobulindeterminanten durch Zusatz von Anti-leichte Ketten-(Kappa-)Antiseren und Komplement lysiert, so erniedrigte sich der Prozentsatz von CRL unter den uberlebenden Zellen deutlich. Im Gegensatz dazu war der Anteil von CRL nach Vorbehandlung von Lymphocyten mit Anti-Thymuszellen-Antiserum oder mit Anti-θ-Antiserum und Komplement stark erhoht. Wurden Lymphocytensuspensionen durch Zentrifugation in einem diskontinuierlichen Albumingradienten von CRL gereinigt, so konnte ein gleichzeitiger Verlust von Zellen mit Immunglobulindeterminanten beobachtet werden. Im Gegensatz dazu stieg in den so behandelten Zellsuspensionen der Anteil von Lymphocyten, die mit Anti-Thymuszellen-Antiserum oder mit Anti-θ-Antiserum reagierten, deutlich an. Schlieslich konnten Immunglobulindeterminanten durch eine kombinierte Methode an etwa 85% der CRL dargestellt werden, wahrend dies nur an einem vergleichsweise geringen Teil der non-CRL (etwa 15%) beobachtet werden konnte. Aus diesen Befunden schlossen wir, das beide untersuchten Eigenschaften Marker ein und derselben „knochenmarksabhangigen“ Lymphocytenpopulation darstellen und diskutierten die Bedeutung dieser Zellen bei der Anreicherung von Antigenen in bestimmten Abschnitten des lymphatischen Systems.

Bei Erkrankungen mit vermehrtem Auftreten lymphatischer Reizformen im peripheren Blut wurde die immunzytologisch bestimmte Zahl gamma-Ghaltiger peripherer Lymphozyten mit der von3H-Thymidin-markierten Lymphozyten verglichen. Bei Patienten mit Mononucleosis infectiosa Hepatitis epidemica, Morbilli und Rubeolen ergab sich eine Zunahme beider Zellpopulationen, bei Patienten mit Morbus Hodgkin wurde eine isolierte Vermehrung der3H-Thymidin-markierten Lymphozyten beobachtet. Im zeitlichen Ablauf erreichten zuerst die3H-Thymidin-markierten, dann die gamma-G-haltigen Zellen die hochsten Werte. Bei der direkten Zuordnung zeigte sich, das bis zu einem Funftel der IgG-haltigen Zellen3H-Thymidin-markiert waren und somit teilungsfahig, sein durften. Eine der Zahl der IgG-haltigen Lymphozyten vergleichbare, wenn auch etwas grosere Anzahl lymphoider Zellen wies bei der elektronenmikroskopischen Klassifizierung vermehrt Ergastoplasma auf.

Es wird uber einen Patienten mit IgAK+IgMK-Doppelparaproteinamie und Bence-Jones-Proteinurie vom Kappa-Ketten-Typ berichtet. Histologisch fanden sich im Knochenmark neben einer diffusen Vermehrung polymorpher Plasmazellen, knotchenformig angeordneten Infiltrate lymphozytoider Zellen sowie im Becken Veranderungen, welche fur einen M. Paget typisch waren. Bei dem vorliegenden Fall bestehen somit drei Krankheiten nebeneinander: Plasmozytom, M. Waldenstrom und M. Paget. Auf Grund der immunologischen, histologischen sowie fluoreszenzmikroskopischen Untersuchungen mus mit Sicherheit angenommen werden, das die IgA-Paraproteine in Plasmazellen und die IgM-Paraproteine in lymphoiden Zellen gebildet werden. Somit liegt hier eine echte diklonale Gammopathie vor. Die Entstehungsmoglichkeiten der biklonalen und monoklonalen Doppelparaproteinamien werden diskutiert.

Bei 23 Patienten mit verschiedenen Leukamieformen wurde in Verlaufsbeobachtungen bis zu einem Jahr gleichzeitig die Muramidaseaktivitat (MA) von Cytolysaten peripherer Leukocyten, Serum und Harn gemessen. Die Werte der leukocytaren MA schwankten bei Normalpersonen nur gering (1,0–2,8 µg/106), bei Patienten mit Leukamien zwischen kaum mesbaren Werten und 26,0. Die Leukocyten-MA-Anderungen im Krankheitsverlauf waren sowohl leukamischen Monocyten, Promyelocyten, Myelocyten als auch reifen Neutrophilen bei Patienten in Remission zuzuordnen. In den meisten untersuchten Fallen waren Leukocyten-MA und Serum-MA gleichsinnig verandert, wenn auch in unterschiedlich starkem Ausmas. Lediglich bei je cinem Patienten mit myelomonocytarer und chronisch myeloischer Leukamie wurden extrem erhohte Serum- und Harn-MA-Werte trotz wiederholt normaler Leukocyten-MA beobachtet. Erniedrigte MA-Werte wurden regelmasig beim Auftreten von Stammzellen und Lymphoblasten in der Peripherie beobachtet, wobei die Leukocyten-MA empfindlicher als die Serum-MA reagierte. Patienten mit chronisch myeloischer Leukamie wiesen eine geringere Serum-MA auf als aus der Berechnung der peripheren leukocytaren MA zu erwarten gewesen ware. Eine MA im Harn wurde bei Patienten mit Monocytenleukamie, Promyelocytenleukamie, myelomonocytarer Leukamie und auch vereinzelt bei Patienten mit chronisch myeloischer Leukamie gefunden. Eine Beziehung zwischen peripherer Blastenzahl und Harn-MA konnte nur bei Patienten mit Monocytenleukamie und myelomonocytarer Leukamie erstellt werden. 10 Patienten mit einer Uberlebenszeit von weniger als 3 Monaten zwischen Diagnosestellung und Tod wurden einer Gruppe von 6 Patienten mit einer Uberlebenszeit von mehr als 6 Monaten gegenubergestellt. In der ersten Gruppe war der mittlere Serum-MA-Spiegel signifikant hoher als in der zweiten Gruppe.

The production of characteristic granules is one of the most specialized functions concerned with the development of granulocytes and monocytes. Leukemia-associated defects in the production of these highly specialized cell organelles reflect the perturbation of the RNA metabolism within the leukemic cells. Pathologic granulation can be of some clinical importance as a diagnostic aid in identifying various cytological types of acute leukemia as well as giving an indication for the profound perturbation in the intrinsic leukocyte regulation, as it occurs in preleukemic states or following treatment with some cytotoxic agents. The defects in granule production may lead either to a partial or complete reduction of a single or of several granular enzymes as well as in the partial or complete failure of the production of a given type of granules. On the other hand unusual increases of specific enzyme activities can be found as well as combination of characteristic granules of different cell types within one homogenous leukemic cell population. Ultrastructural evidence has been obtained that the production of granules may be disturbed at any morphologically identifiable stage of granulogenesis. Pathological agglomeration or fusion of granules with atypical cytosomes may lead to further abnormal subcellular structures exhibiting granular enzyme activities.

The cellular exudates in skin windows of 10 normal controls, 3 patients with myeloblastic leukemia, 3 patients with acute monocytic leukemia and 4 patients with symptomatic monocytosis were evaluated by cytochemical methods (naphthol-AS-esterase, acid phosphatase, naphthylamidase, sudan-black B) and by immunofluorescence using a specific antiserum against human lysozyme. Monocytes and derived cells were identified by their content of characteristic NaF (1.5 mg./ml.) -sensitive naphthol-AS-esterase, whose presence was demonstrated by a double incubation method. After an initial decrease, corresponding to degranulation, the acid hydrolases appeared again in the Golgi zone. Macrophages with NaF-sensitive esterase and strong lysosomal enzyme activities became apparent after 12-16 hours, and there was no indication of a development of these cells from lymphocytes. In myeloblastic leukemia and symptomatic monocytosis the cellular exudate was comparable to that of normal humans. In monocytic leukemia an early exudation, predominantly of monocytoid cells, (instead of granulocytes) was a consistent finding. The transformation of monocytoid cells to typical macrophages with an increase of the lysosomal enzymes occurred with enzyme activities remaining lower than in normals.

Bei 19 Kaninchen wurde 59–80 Tage nach umschriebener Hautverbrennung und anschlie\ender Injektion von Freundschem Adjuvans eine intradermale Injektion des Verbrennungs-Antigens (Extrakt aus ihrer verbrannten Haut) vorgenommen; es entwickelte sich daraufhin an der Injektionsstelle eine proliferative Entzundung mit Erythem und Induration wie bei einer uberempfindlichkeitsreaktion vom verzogerten Typ. Bei 33 nicht hautverbrannten Kaninchen kam es nach einer solchen Injektion lediglich zu einer exsudativen Entzundung der Injektionsstelle. Durch eine intradermale Injektion lebensfAhiger lymphoider Milzzellen zusammen mit einer intravenosen Einverleibung des entsprechenden Verbrennungs-Antigens konnte dann diese Reaktion vom verzogerten Typ von den hautverbrannten Kaninchen auf gesunde, nicht hautverbrannte Kaninchen ubertragen werden. Kontrollen ergaben, da\ zu diesem ubertragungsversuch die Milzzellen lebensfAhig sein und von einem hautverbrannten Kaninchen stammen mussen.

Four cases of acute myelogenous leukemia characterized by peculiar leukemic cell populations are presented. The cytochemical, ultrastructural, biochemical, and functional features Electron microscopy

In 3 patients with myeloma and paraprotein containing cells in the peripheral blood, we demonstrated by an immunofluorescent technique an increase in the number of lymphocytes and plasma cells with immunoglobulin determinants of paraprotein class specificity. Lymphocytes found with mebranous and intracytoplasmic paraprotein content were considered as intermediate forms in plasma cell development. Some possible disturbances of cell proliferation known in lymphoproliferative diseases are discussed.

A rabbit antiserum was obtained against a protein isolated from membranes of bovine chromaffin granules. Immunohistochemical evidence indicates that this protein is present in the adrenal medullae of several species and in bovine sympathetic nerve.

Seren von Patienten mit Lupus erythematodes, mit primar chronischer Polyarthritis, mit lymphoproliferativen Erkrankungen sowie verschiedenen Erkrankungen mit antinuclearen Antikorpern wurden auf das Vorhandensein von Antikorpern gegen autologe Lymphocyten, sog. „Lymphocytotoxine“ getestet. Diese Lymphocytotoxine konnten fast ausschlieslich bei Patienten mit LE nachgewiesen werden. Von 12 Patienten mit LE fehlten diese Antikorper nur in einem Fall. Ihr Auftreten war unabhangig von der Aktivitat der Erkrankung. Die Virusatologie als Ursache der Entstehung des LE wird diskutiert.

About 5% of all human glomerulonephritides are induced by basal membrane antibodies. Immunohistologically they are characterized by a linear fluorescence along the basal membrane of the glomerulum, when cuts of the kidneys of bioptic material are incubated with fluorescent anti-human-IgG-serum. Basal membrane antibodies circulating in the serum as well as antibodies eluted from renal homogenates are nephritogenic. Also recidivations of nephritis on renal homografts confirm the pathogenic significance. Basal membrane antibodies of patients with rapidly progressing glomerulonephritis seem to differ only by a non-existing cross-reaction to the antigen of the basal membrane of the lungs from Goodpasture basal membrane antibodies. Only a glomerulonephritis with pulmonary haemorrhage induced by basal membrane antibodies should be called Goodpasture syndrome.

The clinical, immunological and histological findings in a patient suffering the acquired immunodeficiency syndrome and Pneumocystis carinii pneumonia are discussed. At the time of diagnosis HTLV III antibodies were not demonstrable. Treatment with trimethoprim sulfamethoxazole achieved a remission lasting 7 months so far. HTLV III antibodies became demonstrable after 5 months; the markedly decreased Helper/Suppressor ratio of 0.30 remained unchanged.

Goodpasture's syndrome occurred in six patients (four men and two women) aged 18-32 years. Haemoptysis, anaemia and abnormal urinary findings with erythrocyturia were the first signs of the disease in five instances, in one the renal signs predominated. In five patients the disease took a malignant course with uraemia developing within six weeks. One patient is in a spontaneous remission eleven months after the onset of the disease. Three patients died of recurrent haemoptysis (non-nephrectomised), two nephrectomised patients are still alive half a year and three and a half years later, respectively. A typical linear IgG-, beta1A, alpha2D and beta1E deposition along the glomerular capillaries was demonstrated immunohistologically in all subjects. Of three lung biopsies one also had typical IgG and beta1A-positive linear fluorescence along the alveolar basal membrane. Immunoglobulins with basal membrane antibody activity were eluted from renal homogenates in two patients, basal membrane antibody activity in serum was demonstrated only in one of three patients.

Two patients with rare localizations of extrapulmonary tuberculosis are presented. In a 55-year-old man admitted with septic fever and a low neutrophil count, ultrasonography of the spleen revealed splenomegaly with numerous hypodense foci. Fine needle biopsy of one of the foci disclosed tuberculosis. Persistent splenogenic neutropenia and thrombocytopenia, despite tuberculostatic therapy, required splenectomy which confirmed the initial diagnosis. At laparotomy no other abdominal foci of tuberculosis were found. In a second 57-year-old man admitted for ascites and weight loss, a biopsy specimen of the liver showed normal liver parenchyma but, by chance, peritoneal tuberculosis. Tuberculostatic therapy brought about cure.

Immunological, immunofluorescence and electromicroscopic studies were performed in a case of atypical myeloma. The 77-year-old patient presented with skeletal pain, multiple osteolytic lesions and bone marrow infiltration by atypical plasma cells. Monoclonal light chains kappa were confined to the plasma cells, as shown by immunofluorescence. No monoclonal immunoglobulin or fragments were detected in plasma or concentrated urine, even by highly sensitive immunological methods. The concentration of the immunoglobulins G, A and M in the plasma was markedly reduced. The plasma cells contained very little sarcoplasmatic reticulum. The simultaneous occurrence of monoclonal light chains kappa in the plasma cells and the absence of monoclonal immunoglobulins or fragments in plasma and urine suggest a non-secretory myeloma.

Essential mixed cryoglobulin associated with rapidly progressive glomerulonephritis has been observed in a 64-year-old patient. The cryoglobulin consisted of monoclonal IgM-kappa and polyclonal IgG and displayed rheumatoid-factor activity. The antinuclear antibody test was negative. In the immunofluorescent method renal biopsy showed positive staining for IgG; IgM and the early and late complement components. The rheumatoid factor activity of the renal IgM deposits was demonstrated by binding of fluorescent aggregated IgG. There is thus evidence that the vasculitis observed in essential mixed cryoglobulin is caused by IgM-IgG complexes. The differentiation of essential mixed cryoglobulin from lupus nephritis with cryoglobulins is discussed.

Two patients with IgD-lambda myelomatosis are presented and the differential diagnosis is discussed. Typical features of this disease are the high incidence of Bence-Jones proteinuria, osteolytic lesions, amyloidosis and the predominance of male patients. Furthermore, an augmentation of serum IgD level to 165 mg% was observed in a 22-year-old female patient with presumed Coxsackie myocarditis. The theories in regard to IgD function are discussed.

Two cases of the development of acute myeloid leukemia (AML) after treatment with alkylating agents are reported. In Case 1, melphalan and then cyclophosphamide had been given for multiple myeloma. 46 months after onset of cytostatic treatment AML occurred, as confirmed cytochemically and by qualitative determination of urinary lysozyme. In Case 2, cyclophosphamide had been given for rheumatoid arthritis. After a latency of 34 months 'smouldering leukaemia' developed with an atypical monocytic leukaemic cell population. In a third case, multiple myeloma and monocytic leukaemia developed synchronously. The causative role of melphalan and cyclophosphamide in the development of AML seems securely established. Despite the risk of alkylating agents in the treatment of multiple myeloma or Hodgkin's disease causing AML, they should not be replaced, as other drugs have been shown to be less beneficial. On the other hand, alkylating agents should be used with great caution in the treatment of non-malignant diseases.

Course and treatment of a case of Wegener's granulomatosis are reported. A 39-year-old man admitted to the hospital because of abnormal densities demonstrated by the X-ray examination of the chest. The patient developed a rapidly progressive renal failure with renal insufficiency. Diagnosis was obtained by biopsys from the upper respiratory tract and from the kidney. During hemodialysis and immunosuppressive therapy renal function improved and hemodialysis could be stopped. The patient was discharged from the hospital and kept on immunosuppressive therapy. The patient omitted the medication by himself and renal insufficiency reappeared. The disease was progressive and the patient died because of intestinal bleeding. Apart from the description of the symptoms of this rare disease, problems of diagnosis and treatment are discussed.