[Risk factors for the development of vision disorders in polymyalgia rheumatica with giant cell arteritis]. / Risikofaktoren für die Entwicklung von Sehstörungen bei Polymyalgia rheumatica mit Riesenzellarteriitis.

BACKGROUND AND OBJECTIVE: Visual disorders, even blindness, are serious complications of polymyalgia rheumatica (PMR) associated with temporal arteritis. Their early recognition in patients at high risk is essential to avoid the development of such visual disorders. It was the aim of this study to identify these risk factors. PATIENTS AND METHODS: Clinical and laboratory data and biopsy findings in 131 patients (94 women, 37 men; mean age 74 years) with PMR and concomitant temporal arteritis were analysed retrospectively. RESULTS: Visual disorders occurred in 61 of the 131 patients. Temporal artery biopsy was not sufficient to detect those at high risk. But this was possible by identifying a typical clinical pattern in that most patients with visual disorders had severe cerebral symptoms, while they had only minor forms of PMR and few generalized symptoms. There was no correlation between any of the laboratory tests and high risk. CONCLUSIONS: A typical pattern of clinical manifestations can provide early identification of those patients who have PMR associated with temporal arteritis and are at high risk of developing visual disorders.

[The rare differential diagnosis of a mediastinal space-occupying lesion]. / Seltene Differentialdiagnose einer mediastinalen Raumforderung.

HISTORY AND CLINICAL FINDINGS: Two months before admission a 31-year-old man first noted a painless swelling on the right side of his neck without any associated symptoms. Physical examination revealed a painless right cervical node 4 cm in diameter. INVESTIGATIONS: Magnetic resonance imaging demonstrated a large right-sided cervical tumour which extended into a mediastinal mass 11 cm in diameter. The excised cervical node showed a Hodgkin's lymphoma. Further tests to stage the disease revealed a 1.5 cm tumour in the right testis. Removal of the latter showed a mixed testicular tumour. Mediastinoscopic biopsy confirmed Hodgkin's lymphoma of the mediastinal mass. TREATMENT AND COURSE: Standard chemotherapy of the Hodgkin's lymphoma was undertaken, followed by "extended field" radiation which has so far secured a remission of two years. CONCLUSION: Histological diagnosis is always essential in the case of an unusual tumour location so that a synchronous second tumour may be revealed. If there is a second tumour, exact histological classification with definitive staging of the tumours is necessary to ensure adequate treatment.

Interferon alpha (IFN alpha 2a) therapy for herpes virus-associated inflammatory bowel disease (ulcerative colitis and Crohn's disease).

BACKGROUND/AIMS: The etiology and pathogenesis of ulcerative colitis and Crohn's disease remain unclear, so that exact causal therapy is not yet possible. In our UC and CD patients, viral infections, particularly of the upper respiratory tract, aggravated the underlying disease. This had led us to use in-situ hybridization to investigate intestinal mucosa for viral agents such as HSV I + II- and Epstein-Barr virus DNA. We found these DNA in the cell nuclei in the surface and glandular epithelia of the affected mucosa of the small intestine and the colon. These findings indicated that viruses may exacerbate these inflammatory bowel diseases. METHODOLOGY: Over a period of 1-4.7 years, we treated 16 patients aged 25-65 with Crohn's disease (12 patients) or ulcerative colitis (four patients). In 14 patients, inflammatory bowel disease had been diagnosed years before (mean, 15.3 years). When we started therapy, 75% of the patients with Crohn's disease had extra-intestinal manifestations; and the CDAI after Best averaged considerably above 150. All patients had been taking either prednisone or prednisolone and/or 5-ASA or SASP and/or azathioprine or metronidazole for many years. Using PCR, mucosal specimens of the small intestine and/or the colon were tested for EBV-, HSV I + II, HHV6- and CMV DNA. In 12 of the 16 patients. EBV- and/or HHV6 DNA were found in the affected mucosa. Since interferon alpha administration has proven effective in chronic hepatitis-B therapy, we decided to administer interferon alpha 2a (13,46,47,55). After stopping the above-mentioned basic therapies, we commenced treatment with 6 million units of interferon alpha 2a subcutaneously 3 times per week for at least six months. Four of the patients showed no signs of improvement, and their therapy was stopped after three months. For the others, therapy was continued until patients were clinically symptom-free and viral DNA could no longer be traced in their mucosal biopsies. RESULTS: With interferon therapy, 12 of the 16 patients showed slow but continual improvement. Particularly impressive was the remission of the extra-intestinal manifestations, which did not recur in any patient during interferon therapy. Four patients did not show any improvement, and the clinical symptom of diarrhea continued. Two patients with ulcerative colitis suffered relapses three and four years later, after severe bouts of para-influenza of the upper respiratory tract. In these two patients, EBV- and HHV6 DNA was found in the inflamed mucosa of the colon. Renewed therapy with interferon alpha 2a successfully cleared up the inflammation. The patient group needed an average of eight weeks to become clinically symptom-free, and an average of six months to achieve complete virus elimination in the pathologically altered mucosa. CONCLUSIONS: For herpesvirus-associated ulcerative colitis and Crohn's disease, interferon alpha 2a treatment should be started as early as possible to prevent disease becoming chronic. Whether this kind of antiviral treatment will be as effective in the long term, and whether malignant transformation (herpes viruses are potential tumor inducers) will be delayed or prevented, are questions that can be answered only by future long-term studies.

[The synchronous occurrence of acute myeloid leukemia and kidney carcinoma]. / Synchrones Auftreten von akuter myeloischer Leukämie und Nierenkarzinom.

HISTORY AND CLINICAL FINDINGS: During the past year a 52-year-old man had developed quarterly bouts of fever, up to 38.5 degrees C, associated with fatigue and arthralgia. The fourth bout continued as undulating periods of fever with markedly impaired general state. Physical examination was unremarkable except for mild generalised lymphadenopathy. INVESTIGATIONS: Extensive imaging and serological studies failed to find an infectious or autoimmunological cause. But computed tomography and angiography revealed a space-occupying lesion in the left kidney, yet the suspected diagnosis of renal carcinoma insufficiently explained the clinical picture. DIAGNOSIS TREATMENT AND COURSE: Haematological tests having shown anaemia (Hb 8.1 g/dl) and blast cells (10%) in the peripheral blood a bone marrow biopsy was performed which indicated acute myeloid leukaemia. Three courses of intensive chemotherapy brought about only partial remission and the patient died 6 months later from a cerebral haemorrhage. An autopsy confirmed both the acute myeloid leukaemia and a renal carcinoma. CONCLUSION: The possibility of synchronous occurrence of two malignancies should always be kept in mind especially if the initially diagnosed tumor cannot explain the clinical symptoms.

Successful treatment of stage-IIIB seminoma with single-agent carboplatin therapy.

Single-agent carboplatin chemotherapy has recently been introduced into the therapy of limited seminoma. Because of poor compliance due to Down's syndrome we successfully treated a 32-year-old man with relapsed stage-IIIB seminoma with a dose-modified carboplatin monotherapy schedule leading to complete remission even after a follow-up of 4 years.

Acquired C 1-inhibitor deficiency with angioedema due to pleomorphic immunocytoma in a patient with three malignant tumors: long-term follow-up data and presentation of an additional case.

Two cases of lymphoma-associated acquired C 1-inhibitor deficiency are described. In both patients, C 1-inhibitor deficiency and related symptoms preceded the diagnosis of the underlying neoplasm by several months. C 1-inhibitor deficiency was most likely due to consumption following immunocomplex formation. In both patients, a close relationship between low levels of C 1-inhibitor and tumor relapse was observed during follow-up. These findings indicate that measurement of C 1-inhibitor and complement factor C4 can be used as markers of disease activity in affected patients.

Color-coded Doppler sonography in the diagnosis of abdominal masses.

A man in his forties was operated on for testicular cancer on the right-side with subsequent radiotherapy. Two years later, another tumor was found on the opposite side and surgically removed, followed by polychemotherapy. In the follow-up period, CT scanning and ultrasound showed large abdominal masses which were suspected to be metastases. Before initiating four cycles of chemotherapy, we checked their nature by Doppler sonography and found them to be recently developed abdominal varices due to an alcoholic liver cirrhosis. We were able to demonstrate that Doppler sonography can provide further information and is easy to use.

Efficacy of antiestrogen treatment in a benign metastasizing leiomyoma with paraneoplastic estradiol production.

Benign metastasizing leiomyoma (BML) is a rare hormone-dependent disease which occurs predominantly in women during their child-bearing years. After our patient had refused ablative hormone therapy (bilateral ovarectomy), evidence of estrogen and progesteron receptors in tumor tissue taken from the lung sites, as well as extremely high estradiol serum levels, led us to conduct high-dosage antiestrogen therapy for 5 years; daily administration of 250 mg of Tamoxpuren(R) resulted in stable disease of the pulmonary sites without any side effects. This also significantly lowered estradiol serum levels, which improved clinical symptoms. Five years later, the patient's vision suddenly deteriorated due to bilateral macula degeneration. This forced us to stop the antiestrogen therapy and commence alternative treatment with LHRH analogue (3.6 mg Goreselin). We observed stable disease of the pulmonary metastases and low estradiol serum levels during the first 6 months of Goserelin treatment. The response to antiestrogen therapy in BML suggests that the muscular component of these disorders is responsive to estrogen ablation.

Successful interferon-alpha 2 a therapy for a patient with acute mumps orchitis.

Involvement of one or both gonads in postpuberty mumps is common and can often lead to infertility. Our report discusses the course of disease of a patient with acute mumps orchitis. Only 2 days after administration of interferon, clinical symptoms had drastically improved, and the infection subsided without any after-effects. We therefore recommend immediate interferon therapy, as soon as postpuberty mumps manifests, to prevent testicular atrophy and resulting infertility.

Role of human chorionic gonadotropin in patients with pure seminoma.

Human chorionic gonadotropin (beta-hCG) and alpha-fetoprotein (AFP) are widely established specific and sensitive tumor markers for nonseminomatous testicular cancer. In 106 patients with pure seminoma, a highly sensitive method detected beta-hCG both before and repeatedly during therapy. The low detection limit of the test (0.3 IU/l) coincided with the 95 percentile of a group of 60 healthy blood donors. Its 100 percentile of < 1.0 IU/l was applied as the upper limit of the normal range. In 30.2% of our patients with pure seminoma, elevated beta-hCG levels were noted prior to orchiectomy. The levels returned to normal in 76% of these patients thereafter, and in 34% after additional irradiation or chemotherapy. During an observation period of 2-84 months, all beta-hCG-positive patients were in complete remission. Prior to semicastration, 1 patient showed extremely high beta-hCG levels, while in another patient, beta-hCG and AFP were elevated simultaneously. In both cases, tumor marker levels did not seem to agree with the histology of 'pure seminoma' and rather suggested the presence of nonseminomatous tumor cells. Increased AFP levels contradict the presence of a pure seminoma and indicate a nonseminomatous testicular tumor. The same holds true for strongly elevated beta-hCG levels, whereas levels of up to 200 IU/l correlate with the diagnosis of pure seminoma.

Intra- and perivesical tumor growth in preoperative staging of bladder cancer: the role of transrectal ultrasonography and high resolution magnetic resonance imaging.

Bladder tumors were staged preoperatively before and/or after M-VEC poly-chemotherapy using transrectal ultrasonography and magnetic resonance imaging in 46 patients. The resultant findings were each compared with histomorphologic results. The sensitivity of both methods compared with pathohistology was 88%. Using this technique, the degree of tumor invasion of the bladder wall could be evaluated and the appropriate therapy selected. Further applications included the monitoring of transurethral resection determine its adequacy as a treatment modality.

Spontaneous splenic rupture in a patient with multiloculated signet ring cell carcinoma of the urinary bladder.

We report a case of spontaneous splenic rupture caused by signet ring cell carcinoma of the urinary bladder. Rupture of the spleen usually stems from trauma and only rarely from solid tumor metastasis. Early diagnosis by ultrasound is essential since mortality is high without prompt splenectomy.

[Herpes simplex-associated exacerbation of Crohn's disease. Successful treatment with acyclovir]. / Herpes-simplex-assoziierte Exazerbation eines Morbus Crohn. Erfolgreiche Behandlung mit Aciclovir.

Seven years ago, a now 25-year-old man was found to have Crohn's disease of the terminal ileum as well as of the transverse and sigmoid colon. Despite treatment with both corticosteroids and mesalazine the disease progressed and led to almost complete stenosis of the lumen of the sigmoid colon so that surgical intervention was planned. But because immunohistological examination of the small and large intestinal mucosa had demonstrated herpes simplex virus (HSV I + II) DNA, additional treatment with acyclovir appeared worth-while. The morphological and clinical findings indeed changed decisively, obviating surgery. If a virus infection is found to be a pathogenetic co-factor in a case of Crohn's disease, a therapeutic trial with a suitable antiviral agent seems reasonable.

Rectal pseudotumor due to Chlamydia trachomatis in a male homosexual.

Chlamydia trachomatis causes a wide spectrum of human genital infections including lymphogranuloma venereum. We describe an unusual case of Chlamydia trachomatis proctitis in a homosexual man presenting with a large, solid tumor in the distal rectum, which was treated successfully by long-term oral doxycycline therapy.

[Polychemotherapy using the M-VEC protocol (methotrexate, vinblastine, epirubicin, cisplatin) in advanced urinary bladder cancer--effectiveness and toxicity]. / Polychemotherapie nach dem M-VEC Schema (Methotrexat, Vinblastin, Epirubicin, Cisplatin) beim fortgeschrittenen Harnblasenkarzinom--Effektivität und Toxizität.

We report on preliminary experience with a modified M-VAC (methotrexate, vinblastine, adriamycin and cisplatin) regimen in which adriamycin was replaced by the less toxic 4-epirubicin at equal doses (M-VEC). This study includes 58 patients suffering from advanced bladder cancer, with a minimum observation time of 12 months; each patient received at least two courses of M-VEC (mean follow-up 22 months, average 3.9 cycles). Most (22; 37.9%) of the tumors were T3-4 NO MO; 20 (34.4%) were T3-4 N1-2 MO; and 16 (27.7%) were T3-4 NO-2 M1. Microscopically, 52 (89.6%) were pure transitional cell carcinoma, 5 were (8.6%) squamous cell/carcinomatous transformation; 1 (1.8%) sarcoma was found. Chemotherapy was given as palliative treatment in 34 (58.6%) patients, as neo-adjuvant therapy in 19 (32.8%) cases and as adjuvant therapy in 5 (8.6%) patients. The overall response rate was 72.3% (CR = 51.7%), with a mean duration of response of 18+ months. The disease-free survival so far amounts to 24/58 (41.4%). Squamous cell carcinoma does not respond to M-VEC. Locally advanced bladder cancer (T3-4 NO-2 MO) responds significantly better than metastatic (M1) disease (78.5% vs 56.2%), resulting in an increased survival rate (57% versus 12.5%) after 22 months. The toxicity of M-VEC is considerably lower than has been reported for other regimens (M-VAC, CMV, CM). The toxic effects included mucositis (3%), nadir sepsis (2.4%) and drug-related death (2.4%).

[Successful antibiotic treatment of a pulmonary infection with Nocardia asteroides (biovariety A3)]. / Erfolgreiche antibiotische Behandlung einer pulmonalen Infektion mit Nocardia asteroides (Biovarietät A3).

An open lung biopsy in a 67-year-old man revealed nocardiosis as the cause of a treatment-resistant pulmonary infection. His resistance had been weakened by a non-Hodgkin lymphoma, polychemotherapy and long-term steroid medication. The nocardiosis was cured by a 26-day high-dosage regimen of imipenem and amikacin in combination. A six-month period of co-trimoxazole followed to ensure treatment success. Nocardia asteroides (biovariety A3) was found to be the causative organism, the second time that this has been described as cause of a human infection.

[Immunocytoma with acquired C1-esterase inhibitor deficiency and recurrent angioneurotic edema]. / Immunozytom mit erworbenem C1-Esterase-Inhibitor-Mangel und rezidivierendem Angioödem.

Four attacks of mucosal angioneurotic oedema occurred in a 57-year-old woman, requiring tracheotomy for one episode. Decreased or non-measurable levels of complement components C1q, C-1-INH and C4 pointed to a complement consumption. The basic disease was a pleomorphic immunocytoma with the production of monoclonal IgM. Treatment with danazol, 600 mg daily, raised the serum level of C-1-INH, but not of C4.