HISTORY AND CLINICAL FINDINGS:A 45-year-old woman presented with marked edema of both lower extremities over 6 weeks for a nephrological work-up; she had gained 8 kg of body weight. Voiding was asymptomatic and she had a stable diuresis. The patient took oestrogens for contraception over 10 years. Blood pressure was normotensive. Serum-creatinine was 0.8 mg/dl; a slight microalbuminuria was noted. Left and right ventricular systolic function were normal. DIAGNOSTIC FINDINGS, TREATMENT AND CLINICAL COURSE: Computed tomography of the abdomen revealed a hemodynamically relevant obstruction of the venous blood flow or the lymphatic vessels as cause for the edema of both legs. Masses of lymphangioleiomyomas located around the v. cava inferior were documented. Biopsy of the masses proved a massive proliferation of smooth muscle cells and epitheloid cells with an immunohistochemically typical staining. Furthermore, CT revealed multiple pulmonary cysts in both lungs, results which are pathognomic for lymphangioleiomyomatosis (LAM). In our patient, the structural impairment of the lungs was not substantiated clinically, i. e. she had only slight dyspnoe during exertion. By mild diuretic treatment with HCT and fluid control, a moderate regression of the edema was achieved. At present, the mTOR inhibitor rapamycin as antiproliferative treatment is considered to reduce the retroperitoneal LAM-related masses on an individual basis.CONCLUSION:LAM is a rare genetically determined progressive disease occurring frequently in women in childbearing age. LAM is characterized by a proliferation of smooth muscle cells, lymphangioma, renal angiomyolipoma, pulmonary cysts and progressive destruction of lung parenchyma. Refractory edema can result from an obstruction of the venous blood and lymphatic flow by lymphangioma masses located paracaval, which was the impressive and first clinical feature of LAM in our case report.

Individual immunoprint patterns of sodium dodecylsulfate-polyacrylamide gel electrophoresis-separated Aspergillus fumigatus (Af) allergens/antigens were evaluated in 28 patients with allergic bronchopulmonary aspergillosis in stages II to V. It could be demonstrated that active disease (stage III) is characterized by a very strong IgE response against a variety of Af components, whereas the IgE antibody pattern in corticosteroid-treated patients who have demonstrated improvement is much weaker. In patients in remission (stage II), it is minimal. In contrast, many patients in stage V without corticosteroid treatment demonstrated a strong reactivity of IgE antibodies, indicating persisting active disease. The pattern of IgG antibodies with individual Af components resembles, in general, that of IgE antibodies; however, discrimination between different stages and between treated patients is much weaker. Our results indicate that a certain relationship between the different stages of allergic bronchopulmonary aspergillosis and Af immunoprint patterns exists.

We describe the identification and characterization of antigens of the pearl-oyster shell which caused hypersensitivity pneumonitis in a 42-year-old worker. He was occupationally exposed to the dust of this shell in the manufacture of different ornaments. In addition to a typical history, the diagnosis was confirmed through a workplace-related exposure test and high titres of antigen-specific IgG antibodies. By means of the immunoblot technique, several relevant antigens could be detected in the shell of the pearl-oyster. Control sera also contained immunoglobulins that bound to some electrophoretically separated bands; these could represent antibodies that react with lectin-like components in the shell in a nonimmunologic manner.