Objective To analyze the data from patients with pathologically proved granulomatous lung disease, including etiology, clinical, radiological features and laboratory results. Methods 36 patients with granulomatous lung disease confirmed by lung biopsy in Shanghai First People’s Hospital of Shanghai Jiao Tong University from January 2008 to June 2012 were retrospectively reviewed. The clinical presentation, radiological features and laboratory results were collected and statistically analyzed.Results After haematoxylin and eosin stain combined with special stain, the diagnoses were comfirmed, ie.13 cases of mycobacterial infection, 5 cases of aspergillar infection, 4 cases of cryptococcal infection, 6 cases of sarcoidosis, 4 cases of Wegener’s granulomatosis, 4 cases of unknown causes. Cough was the most common clinical symptom, followed by expectoration. Some patients also developed fever, chest tightness and weight loss. The lesions were widely distributed, of which the right upper lung was the common lesion of mycobacterial infection, inferior lobe of right lung was the common lesion of aspergillar infection. The common lesion of cryptococcal infection was uncertain. The common lesions of sarcoidosis and Wegener ’s granulomatosis were in left upper lung. Small nodule was the most common shapes of lesion, while mass and consolidation were present sometimes. Cavity, air bronchogram, pleural effusion, hilar and mediastinal lymph node enlargement could be found in the chest CT. Interferon gamma release assay, galactomannan antigen assay and latex agglutination test were helpful in the diagnosis of mycobacterial infection, aspergillar infection and cryptococcal infection induced granuloma. Conclusions The clinical presentations and radiological features of granulomatous lung disease are nonspecific. Histopathology obtained through biopsy is the key for the diagnosis. Immunological examination, test of new antigens to microorganism and clinical microorganism detection are valuble in the diagnosis and differential diagnosis of granulomatous lung disease.
ObjectiveTo improve the understanding of pulmonary alveolar microlithiasis (PAM).MethodsA male patient and his brother were considered PAM by chest image and further examined by gene sequencing. The patient was confirmed through percutaneous lung biopsy. The clinical data of 172 patients from 108 pieces of literature were collected and reviewed from PubMed. The clinical presentation, radiological character, pathological finding, diagnosis and differential diagnosis, treatment and prognosis of the disease were analyzed and summarized.ResultsThe results of gene sequencing revealed the homozygous mutation of c.910A>T in exon 8 of SLC34A2 gene. The genetic mutation encoding the sodium phosphate co-transporter Npt2b (SLC34A2) was considered as the major pathogenesis. Mutations appeared to cluster in exon 8, c.910A>T was the most common mutation observed in Chinese cases. The most obvious symptoms were dyspnea, followed by cough, chest pain, fever, fatigue, and hemoptysis. The clinical signs consisted of cyanosis, clubbed fingers, moist rales and velcro crackles in the lungs. The typical presentation of PAM on a chest X ray was a ‘sandstorm’ appearance. The most frequent high-resolution CT findings of PAM were diffuse ground-glass attenuation and subpleural linear calcifications. Lung biopsy showed lamellar microliths deposited in alveolar spaces and the pleura. Etidronate had an imprecise role in the treatment of PAM. Lung transplantation was the ultimate effective treatment option.ConclusionsPAM is a rare autosomal recessive inherited lung disease. The characteristics of the disease should be fully understood in order to achieve early diagnosis and early intervention. Lung transplantation is recommended for patients of end stage.