Nebennierentuberkulose als seltene Ursache einer adrenocorticalen Insuffizienz (Morbus Addison).

Adrenocortical insufficiency (Addison's disease) is a functional diagnosis. At the beginning of this century, tuberculosis was recognized as the main etiological cause (50-70%). Today, however, tuberculosis represents only 10% of the cases. Adrenocortical insufficiency is mainly caused by autoimmune adrenalitis (more than 50%). Unilateral adrenocortical disease is usually asymptomatic or presents, as in the case described, with minor symptoms. This case report of adrenal tuberculosis illustrates the current challenges of diagnosis and therapy.

Lethal complications in a case of sigmoid diverticulitis. A case report.

A 61-year-old female complaining of arthralgia was repeatedly treated with antibiotics and also with prednisolone. A chronic polyarthritis was assumed. In hospital, leukocytosis of 21.000 was found one day before death as well as moderate anemia. Colonoscopy was rejected by the patient. A computer tomography revealed destructive arthritis of the symphysis, vertebral osteochondrosis L5/S1, and sigmoid diverticulosis. The patient died with clinical signs of central dysregulation. At autopsy, a covered perforation of a sigmoid diverticulum with purulent peridiverticulitis was found. The 5th lumbal vertebra and the symphysis showed hematogenic abscesses. Microabscedating pneumonia, purulent meningitis and hypophysitis, and mycotic aneurysm of the basilar artery with lethal rupture were further results of hematogenic spread. Death was caused by massive subarachnoidal hemorrhage. This history is not untypical for elder patients with complicated diverticular disease. The intestinal perforation is often clinically occult due to only few and unspecific symptoms which cannot be exactly attributed to the colon. In the last ten years, we have found lethal complications of sigmoid diverticulitis at a frequency of 0.32% (5 cases in 1.557 subsequent autopsies). The clinical differential diagnosis included diverticulitis in none of the cases. This underlines the importance of autopsies for quality control in medicine, because modern diagnostic methods such as computer tomography were not able to give the correct diagnosis in these cases.

Autoimmune hepatitis in inflammatory bowel disease: report of two unusual cases.

Elevated transaminases occur in up to 17% in patients with inflammatory bowel disease. Primary sclerosing cholangitis (PSC) is an important cause for elevated liver enzymes in these patients whereas autoimmune hepatitis is rare. Both diseases can overlap. We report two patients with an autoimmune hepatitis. One patient had Crohn's disease and arthritis with morphological liver changes typical for autoimmune hepatitis but without the characteristic autoantibody pattern. The other patient suffered from ulcerative colitis. He had antinuclear and antiactin antibodies as in autoimmune hepatitis type I. however, histological examination of the liver showed bile duct changes. Transaminases declined significantly in both patients after onset of steroid treatment. Therefore, the diagnosis of autoimmune hepatitis in patients with inflammatory bowel disease must not be missed, as immunosuppressive therapy improves the prognosis of the illness.

Impaired pancreatic function in patients with Crohn's disease with and without pancreatic autoantibodies.

Pancreatic autoantibodies (PABs) are found in 31% of patients with Crohn's disease (CD), but they do not correlate with the activity of intestinal disease or the incidence of acute pancreatitis. Exocrine pancreatic insufficiency has been observed in patients with CD. The aim of our study was to correlate the occurrence of PABs with exocrine pancreatic function to explore the clinical significance of these antibodies. Serum samples of 64 patients with CD were tested for PABs by indirect immunofluorescence. In addition, all patients were tested for exocrine pancreatic insufficiency by a fluorescein dilaurate test. PABs were detected in 26 of 64 patients (40%). The PAB-positive and -negative groups did not differ in clinical characteristics, such as age, sex, involvement of intestine, previous surgical interventions, drug therapy, and disease activity. Seven of the antibody-positive patients (27%) had impaired pancreatic function, in contrast to three of 38 PAB-negative patients (8%) (p < 0.05). In conclusion, exocrine pancreatic function is impaired significantly more often in PAB-positive than in PAB-negative patients. A prospective follow-up is required to determine whether PAB-positive patients are more likely to develop pancreatic insufficiency later in their course of disease.

[Magnetic resonance imaging in perianal Crohn's disease]. / Kernspintomographie bei perianalem Morbus Crohn.

Magnetic resonance imaging (MRI) was undertaken in a prospective study of 34 consecutive patients (21 women, 13 men; median age 31 [18-53] years) suspected of having active perianal Crohn's disease. The results of the investigation were compared with those obtained by independent observers on proctological and intraoperative examination (n = 31). A total of 58 fistulas and 21 abscesses were noted intraoperatively, 47 fistulas and all 21 abscesses by MRI, and 40 fistulas and 13 abscesses proctologically. The proctological examination proved to be more sensitive in demonstrating short subcutaneous or anovaginal fistulas (three of four subcutaneous and two of five anovaginal fistulas were not shown by MRI). Intersphincteric, ischiorectal and supralevator involvement was shown better by MRI. These results indicate that in perianal Crohn's disease MRI is a useful addition to proctological examination.

Acute pancreatitis in Crohn's disease.

Pancreatitis as an extraintestinal manifestation of Crohn's disease (CD) is controversial. We review the episodes of acute pancreatitis in patients with CD. Of 852 patients, 12 developed clinically overt pancreatitis, representing a frequency of 1.4% in a follow-up period of 10 years. In 10 patients, common causes of pancreatitis were excluded. In 2 patients, drug-induced disease (azathioprine, sulfasalazine) could not be ruled out. Recurrence of pancreatitis was observed in only 2 patients. Younger patients and those with active disease seemed more at risk for development of pancreatitis. If prednisolone was needed for treatment of active CD, no adverse effect was observed for the pancreatitis. Along with the clinical features, we studied autoantibodies against exocrine pancreas; the incidence of autoantibodies in patients with pancreatitis was the same as in the controls who did not develop pancreatic abnormalities. This does not support the hypothesis that acute pancreatitis in CD is associated with the formation of pancreatic autoantibodies.

[Polypoid pansinusitis in an unusual, extra-intestinal manifestation of Crohn disease]. / Polypöse Pansinusitis als eine ungewöhnliche, extraintestinale Manifestation des Morbus Crohn.

A 17-year-old female patient with known Crohn's disease developed a polypoid pansinusitis and a peritonsillitis during an acute inflammatory phase of her disease. The symptoms observed in the paranasal sinuses and in the oropharynx disappeared after treatment with cortisone and antibiotics was started. The histological findings in the biopsy specimens taken from the affected regions as well as the course of the disease suggest the extraintestinal manifestation of Crohn's disease.

Filiform polyposis: a case report describing clinical, morphological, and immunohistochemical findings.

Filiform polyposis (FP) is a rare condition of uncertain pathogenesis, 28 cases of which have been published since it was first described in 1965. It is usually found in association with chronic inflammatory bowel disease, especially Crohn's disease and ulcerative colitis. The condition is characterized by the presence of numerous, densely packed, filiform polyps in the colon, which may resemble villous adenomas on endoscopy. We describe a case of FP occurring in a 33-year-old man with a 5-year history of Crohn's disease, in whom subtotal colectomy was performed because of perforation of the sigmoid colon. Microscopy revealed inflammatory pseudopolyps covered by largely normal and non-dysplastic colonic epithelium. The neuroendocrine system of the intestine in FP was investigated for the first time in this case: marked hyperplasia of endocrine cells immunoreactive for serotonin, somatostatin and enteroglucagon and of neural structures immunoreactive for substance P and vasoactive intestinal peptide was noted in the polyps and the adjacent intestinal mucosa. The patient has experienced no further complications in the 12 months since the operation. Medication administered in FP depends mainly on the nature of the underlying disease, and the amount of information published about this condition is as yet insufficient to allow any one specific type of treatment to be recommended. FP alone is not an indication for bowel resection but complications, such as massive haemorrhage or intestinal obstruction, may necessitate surgical intervention.

Microbic superinfection in relapse of inflammatory bowel disease.

To assess the association between symptomatic relapse of inflammatory bowel disease (IBD) and superinfection with enteropathogenic microorganisms, we determined prospectively the incidence of infections with enteropathogenic bacteria, protozoa, and helminths in patients with confirmed longstanding IBD. Sixty-four patients with IBD (49 with Crohn's disease [CD] and 15 with ulcerative colitis [UC]) were consecutively enrolled in the study when relapse occurred. Multiple biopsies for histological and microbiological investigations were taken from all patients who were evaluated by colonoscopy. Parallel stool specimens were investigated for the presence of enteropathogenic bacteria, protozoa, and helminths. In six patients, we detected Clostridium difficile or toxin B (five CD, one UC), in one patient Campylobacter jejuni (CD), and in another patient Salmonella typhimurium (UC). Enteropathogenic Escherichia coli were isolated from three patients. Investigation of biopsies for Mycobacteria, microscopic examination of stool samples for helminths, and immunofluorescence for chlamydia were negative in all patients. In summary, as we found enteropathogenic microorganisms so infrequently in patients with relapse of IBD, despite intensive microbiological screening by tissue sampling for detection of gut adherent bacteria, we believe that microorganisms play only a minor role in the exacerbation of IBD.

[Do perioperative blood transfusions affect the postoperative recurrence rate in Crohn disease?]. / Beeinflussen perioperative Bluttransfusionen die postoperative Rezidivrate bei Morbus Crohn?

The influence of perioperative blood transfusion on postoperative recurrence rate was investigated in 109 patients with Crohn's disease after primary surgery. 52 of the 109 patients had received transfusions during the perioperative period. The symptomatic recurrence rate 5 years after surgery was 31% in transfused and 33% in non-transfused patients (p = 0.99). The reoperation rates were 10% (transfused) and 12% (non-transfused) after 5 years respectively (p = 0.70). Therefore, the finding of decreased postoperative recurrence rates in patients who received perioperative blood transfusions cannot be supported by our results.

Antibodies to a trypsin sensitive pancreatic antigen in chronic inflammatory bowel disease: specific markers for a subgroup of patients with Crohn's disease.

The presence of antibodies against pancreatic juice (PAB) in patients with Crohn's disease has recently been reported. In our study sera from 273 patients with inflammatory bowel disease (222 with Crohn's disease, 51 with ulcerative colitis) have been examined for PAB and also for antibodies against gut tissues by means of indirect immunofluorescence. PAB were found in 68 of the 222 patients with Crohn's disease (31%), with titres ranging from 1/10 to 1/1280, and in only two patients with ulcerative colitis (4%), with titres of 1/20. None were found in 198 patients with various chronic inflammatory diseases and healthy control subjects. No differences were found between the PAB positive and negative patients when the following parameters were compared: disease activity (Crohn's disease activity index), involvement of bowel segments, incidence of extraintestinal disease, or treatment with anti-inflammatory drugs. Only seven of the patients with Crohn's disease had a history of pancreatic disease and of these, four had detectable pancreatic antibodies. Longitudinal observations of 40 patients with Crohn's disease showed a stable pattern for PAB, independent of disease activity and treatment. Partial characterisation of the PAB antigen, isolated from pancreatic juice, showed a trypsin sensitive macromolecular protein of more than 10(6) daltons not identical with a panel of defined exocrine pancreatic proteins. By contrast, antibodies against goblet cells (GAB) were found in 13 of 51 patients with ulcerative colitis (29%) and in none of the patients with Crohn's disease or control subjects. PAB were found as a highly specific serological marker for Crohn's disease and GAB for ulcerative colitis, but the relevance of PAB and GAB in the pathogenesis in Crohn's disease remains unclear.

[Prognostic factors in Crohn's disease. Is the probability of a later operation assessable at the initial diagnosis?]. / Prognostische Faktoren bei Morbus Crohn. Ist die Wahrscheinlichkeit einer späteren Operation bei Erstdiagnose abschätzbar?

Data were retrospectively analysed of 492 patients (268 women, 224 men; mean age 27 [9-71] years) to find out what features present at time of first diagnosis (age, sex, site of disease, biochemical findings) will play a role in determining the probability of surgery ultimately becoming necessary. The probability of an operation ten years after first diagnosis was 55%, after 20 years it was 88%, significantly higher if the ileum rather than only the colon was affected. Age and sex had no influence. Patients with a haemoglobin content below 12 g/dl (women) or below 13.5 g/dl (men), or an albumin concentration under 4.0 g/dl, or a blood sedimentation rate over 30 mm in the first hour had a probability of operation after ten years nearly three times higher than those without one of these findings. A prognosis about the likely future course of the disease can be made from its localization and the biochemical values. Thus patients with an early ileocolitis and unfavourable biochemical findings apparently constitute a subgroup in which the disease takes a primarily aggressive course.

Oral manifestations of Crohn's disease. An analysis of 79 cases.

We report four new cases of oral manifestation in Crohn's disease (CD) and evaluate 75 reported cases for morphology and site of oral and intestinal manifestations of CD, clinical manifestation, and treatment. Oral CD was the presenting symptom in 43 of 72 (60%) patients and relapsed in 34 of 60 (57%). Median age at presentation was 22 (range 6-57) years, and males were affected more often (1.85:1, male:female ratio). From a total of 228 oral lesions in 79 patients, lips (57 lesions), gingiva (40 lesions), vestibular sulci (31 lesions), and buccal mucosa (25 lesions) were the sites most frequently affected. Edema (62 lesions), ulcers (57 lesions), and polypoid papulous hyperplastic mucosa (45 lesions) were the most common type of lesions. The rate of granuloma detection was high in oral (67-77%) and intestinal lesions (45-71%). A total of 66 courses of drug therapy in 51 patients were analyzed. Complete remission of oral symptoms was achieved by systemic steroids and/or azathioprine in 13 of 26 (50%) patients, whereas strictly topical treatment with steroids resulted in complete remission of oral symptoms in 7 of 12 (58%). We conclude that oral CD exhibits a characteristic morphologic appearance, as often as not preceding intestinal symptoms in adolescents and young adults. Thus, patients with orofacial granulomatosis CD should be vigorously searched for by complete gastrointestinal endoscopic investigation. Oral CD may cause disabling pain and facial distortion, and results of treatment remain unrewarding. In the absence of data from controlled therapeutic trials, systemic steroids and/or azathioprine are recommended if topical treatment has failed to control symptoms.

[Crohn's disease: disease activity and recurrence following surgery]. / Morbus Crohn: Krankheitsaktivität und Rezidiv nach Operation.

The data from 238 patients (108 men, 130 women, mean age 29 [15-71] years), who had undergone operations for Crohn's disease between 1968 and 1988, were analysed retrospectively with the purpose of ascertaining the significance of an endoscopically demonstrated "early recurrence". In 170 patients postoperative colonoscopy had been performed at least once. In 130 patients the activity of the disease in the years before and after operation was compared in terms of such parameters as haemoglobin, erythrocyte sedimentation rate, serum albumin, body weight and the frequency of acute flare-ups of the disease and admissions to hospital. The probability of an endoscopically demonstrable recurrence was 90% after 5 years, while the corresponding figure for a symptomatic recurrence was 40%. This indicates that the routine performance of postoperative colonoscopies is of no value in assessing the prognosis. The probability of a reoperation was 21% after 5 years; in cases where both the ileum and colon were involved the probability was three times as high as in those with isolated involvement of either the small or the large intestine. Post-operatively, a substantial reduction in disease activity of several years' duration was achieved in the overall majority of cases.

[Meckel's diverticulum and lower gastrointestinal bleeding. Problems of preoperative diagnosis]. / Meckelsches Divertikel und untere Gastrointestinalblutung. Probleme der präoperativen Diagnose.

A hitherto perfectly healthy 28-year-old man suddenly passed stools containing bright red blood, and haemoglobin concentration fell at first to 10.1 g/dl. No bleeding source was found on gastroscopy and coloscopy. Nor was the source found by enteroclysis during a bleeding-free interval. Emergency laparotomy was performed after a recurrence of massive bleeding and revealed, 60 cm oral of the ileocaecal (Bauhin's) valve, a Meckel's diverticulum with ulcerated heterotopic gastric mucosa, 5 x 3 x 2 cm in size, and this was resected. 17 red blood cell concentrates had to be infused pre- and postoperatively, but there were no further complications.