Raising awareness about chronic intestinal pseudo-obstruction (CIPO): a case report showing CIPO as initial manifestation of atypical seronegative systemic sclerosis.

Only few case studies address pseudo-obstruction, a disorder - which often frustrates clinicians and patients due to an unclear diagnosis and limited therapeutic options. Thus, the aim of this paper is to investigate a relevant case concerning a patient presenting with symptoms of acquired chronic intestinal pseudo-obstruction (CIPO). After one year of extensive diagnostic tests and unsuccessful treatment with prokinetics, the patient underwent a subtotal ileocolectomy. The histology of the intestinal specimen revealed continuous atrophy and fibrosis mainly within the circular, inner muscle layer of muscularis propria of the ileum and colon. Even though serum markers were lacking, a subsequent skin biopsy showed signs of scleroderma supporting an initial diagnosis of intestinal involvement in systemic sclerosis. Despite treatment with steroids and methotrexate, the increasingly emaciated patient died. In conclusion, there is a bias against the publishing of pseudo-obstruction studies, in particular, due to the obscure underlying causes. To raise awareness of this problem, we call for clinicians to systematically generate comprehensive data about patients presenting these symptoms.

[Pulmonary necrotizing sarcoid granulomatosis in a patient with primary biliary cirrhosis]. / Nekrotisierende sarkoidale Granulomatose der Lunge bei einer Patientin mit primärer biliärer Leberzirrhose.

HISTORY AND ADMISSION FINDINGS: A 53-year-old woman presented with recurrent episodes of cough and non-specific pulmonary symptoms. For many years she had been known to have primary biliary cirrhosis. INVESTIGATIONS: The chest X-ray showed multiple pulmonary nodules. Microbiological examination did not detect any pathogen and transbronchial biopsy of the pulmonary nodules failed to provide a diagnosis. Histology of a surgical lung biopsy showed interstitial inflammation, vasculitis and non-caseating granulomas. TREATMENT AND COURSE: The findings indicated necrotizing sarcoid granulomatosis. During oral corticoid therapy the pulmonary nodules regressed within a few weeks. The patient has remained free of pulmonary symptoms. CONCLUSION: Pulmonary necrotizing sarcoid granulomatosis is a rare condition to consider in the differential diagnosis of pulmonary nodules. Because of the histological findings and its benign course it resembles sarcoidosis.