chronic granulomatous disease (cgd) is a rare primary immunodeficiency disease. although the most affected patients are diagnosed in childhood, there are several reports of the disease presenting in adult patients. here we present a 40 years old man who was admitted in hospital due to respiratory symptoms and ground glass pattern in high resolution computed tomography of lung. open lung biopsy ...

Pulmonary complications occur in up to 60% of patients after hematopoietic stem cell transplantation (HSCT), causing significant morbidity and mortality. Among them, non-infectious bronchiolitis is considered a late complication in the form of bronchiolitis obliterans. We report a patient who developed non-infectious bronchiolitis within four weeks after undergoing HSCT for biphenotypic leukemi...

Bronchiolitis obliterans is a complication after allogeneic haematopoietic stem cell transplantation (HSCT). Management of bronchiolitis obliterans comprises intensive immunosuppression, but treatment response is poor. We investigated the effect of cyclosporine A (CsA), tacrolimus (FK506), methylprednisolone (mPRED), mycophenolate mofetil (MMF) and everolimus on the proliferation of primary lun...

David B. Ettensohn, M. D., F.C.C. ;j Kenneth H. Mayer, M. D.;t Noubar Kessimian, M. D.;t and Peter S. Smith, M. D. § Patients with the acquired immune deficiency syndrome (AIDS) frequently develop interstitial lung disease. This is due most commonly to opportunistic infections, but malignancy and lymphocytic interstitial pneumonitis have also been associated with the syndrome. In contrast, ther...

BACKGROUND The role of nitric oxide (NO) in the pathophysiology of graft dysfunction following lung transplantation remains unclear. To determine whether measurement of NO in the exhaled breath of lung transplant recipients provides useful information about graft pathology, a cross sectional study was performed on a cohort of recipients as they attended for review. METHODS One hundred and fou...

A 44-year-old Japanese man consulted to our hospital because of cough and sputum. Chest-XP and CT revealed diffuse reticular opacities in both lungs.A transbronchial lung biopsy (TBLB) showed a moderate infiltration of lymphocytes in the alveolar septae. He was diagnosed as interstitial pneumonia, and treated by drugs. One year later, his condition deteriolated, and a large open biopsy was perf...

Common variable immunodeficiency is one of the most frequent immunity alterations. The most common clinical presentation occurs with recurrent respiratory infections, from pneumonia to otitis, and may be associated with other diseases such as bronchiectasis or interstitial lung diseases. We report the case of a 28-year-old patient with frequent respiratory infections and nodular pulmonary infil...

Chronic rejection after lung transplantation is manifested as obliterative bronchiolitis (OB). The development of de novo lymphoid tissue (lymphoid neogenesis) may contribute to local immune responses in small airways. Compared with normal lungs, the lung tissue of 13 lung transplant recipients who developed OB demonstrated a significantly larger number of small, airway-associated, peripheral n...

Lung transplantation has evolved into a life-saving therapy for select patients with end-stage lung diseases. However, long-term survival remains limited because of bronchiolitis obliterans syndrome (BOS). Soluble HLA-G, a mediator of adaptive immunity that modulates regulatory T cells and certain classes of effector T cells, may be a useful marker of survival free of BOS. We conducted a retros...

Azithromycin reduces airway inflammation and improves forced expiratory volume in 1 s (FEV₁) in chronic rejection or bronchiolitis obliterans syndrome (BOS) after lung transplantation (LTx). Azithromycin prophylaxis might prevent BOS. A double-blind randomised controlled trial of azithromycin (n = 40) or placebo (n = 43), initiated at discharge and administered three times a week for 2 yrs, was...