1 and 2). There were no external compression or emphysema. Bronchoscopy revealed a generally swollen and inflamed mucosa with visually poorly defined tracheal and bronchial cartilage
in both patients. There was a fixed airway narrowing affecting the trachea and main bronchi, with no apparent dynamic changes throughout the respiratory cycle. After a period of medical treatment, repeated bronchoscopy showed reduced inflammatory changes but was otherwise similar. There was no growth of mycobacteria, other bacteria or vira in lavage fluid from the airways. Bronchial biopsies revealed chronic unspecific inflammation. There were no signs of granulomatous inflammation, vasculitis or amyloidosis in a Congo red staining. Unfortunately there was no cartilage in the bronchial biopsies. Additional biopsies from auricular cartilage, ALK activation conchae and costosternal cartilage were normal. All biopsies Selinexor supplier obtained from the siblings were compared, and deemed identical. Electron microscopic evaluation of the biopsies has not been performed. We have not performed biopsies of subcutaneous fat or rectal mucosa. Positron-emission
tomography of patient no 2 whilst receiving both cyclophosphamide and prednisolon, did not show any activity in trachea, bronchi or lung parenchyma. CT of the sinuses were normal apart from minor edema in the right maxillar sinus of patient no 2. Serologic testing was negative for CMV and Mycoplasma pneumoniae, Bordetella pertussis, ANA, ANCA and RF. Skin testing for aspergillus was negative, and the total IgE was normal. Renal function was normal, and there was no proteinuria or signs of nephritis. Serum-electrophoresis showed non-specific inflammation.
Both patients have received immunosuppressive therapy with prednisolone, C-X-C chemokine receptor type 7 (CXCR-7) and cyclophosphamide was added. Patient no 1 received azithromycin as immunomodulation, and patient no 2 has received azathioprine, without any effect on symptoms. They also received inhalations with budesonide/formoterol, salbutamol and ipratriopium bromide ad libitum, with uncertain effect. Initially the condition deteriorated for both patients with progressive worsening of symptoms, inspiratory stridor and declining spirometric values. The condition of patient no 1 has been stable during the last two years with FVC 2.8 l (63%) and FEV1 0.9 l (26%). Cyclophosphamide has been replaced by azathioprine, and he still receives prednisolone and azathioprine, 5 years after the initial presentation. The rapid deterioration of patient no 2 was initially halted when treatment was instituted. After a time she progressed, though, and eventually received rituximab, without any significant effect. There is, however, still a minor effect of large doses of steroids. She still suffers from considerable obstruction of the central airways, resembling what is seen in patient no 1, her latest spirometry values being FVC 2.7 l (83%), FEV1 0.8 l (29%) (Fig. 3).
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