The Beneficial Effect of 3-Month-Induction Therapy with Corticosteroids and Mycophenolate Mofetil Followed by Maintenance Therapy with Yearly Rituximab Infusions as Sole Maintenance Therapy in Cryptogenic Chronic Hypersensitivity Pneumonitis

Background: The available data on cryptogenic chronic hypersensitivity pneumonitis (ccHP) indicate an inherited predisposition to disease with triggering autoimmune phenomena. Hence, we evaluated prospectively the role of a new autoimmune regimen in treatment of its severe and progressive disease. Patients and Methods: A total of 9 patients were included in the study. They had criteria for ccHP viz. 1) clinical features of cryptogenic progressive restrictive lung disease, 2) high-resolution computed tomographic pulmonary abnormalities, and 3) bronchoalveolar lavage lymphocytosis (>30%). The regimen consisted of an initial induction phase of 3-month Solumedrol 1 g IV daily for 3 days followed by 1 month of Prednisone (P) 60 mg/day to tapered down to discontinuation by 3rd month. They also had received Mycophenolate mofetil (MMF) 1 g twice daily for 3 months. This stage was followed by a maintenance phase of yearly Rituximab infusions (1 g followed by 1 g 2 weeks later). Results: compared to their previous 6 months deterioration;all patients showed significant improvement in their forced vital volume, diffusion capacity for carbon monoxide, 6-minutes-walk after the induction phase (at 3 months) which improved further at 15 months with Rituximab therapy. Conclusion: After 3-month induction therapy with P and MMF;yearly R treatment is a safe, practical and effective long-term therapy for ccHP.

Rare imaging findings of hypersensitivity pneumonitis:A case report

BACKGROUND Hypersensitivity pneumonitis(HP)is an immune-mediated syndrome caused by allergen inhalation.High-resolution computed tomography(HRCT)of HP may show diffuse ground-glass opacity,centrilobular ground-glass nodules,areas of air-trapping,thin-walled cysts,or fibrotic changes.CASE SUMMARY A 47-year-old male patient went to the hospital complaining of cough and gradual aggravation of shortness of breath.HRCT of the lung showed that multiple nodules and ground-glass high-density shadows were present in both lungs.In addition,circular high-density shadows of various sizes were widely distributed in both lungs with relatively normal lung markings inside them.But other tests did not have a positive finding that can clarify the cause.Therefore,the patient underwent a lung biopsy.The pathological results showed that the lesions tended to be HP.After 4 mo of follow-up,the lesions in the patient's lungs were absorbed spontaneously,and the symptoms of cough and shortness of breath have disappeared.The review results suggested that the patient's disease was self-healing,which was consistent with the characteristics of HP.CONCLUSION For some patients with HP,abnormal HRCT findings,such as the lesions in the lungs,can be absorbed on their own,which is an important clue in the diagnosis of the disease.Early diagnosis by lung biopsy is necessary when antigen exposure is unknown.

Occupational fibrotic hypersensitivity pneumonia in a halogen dishes manufacturer:A case report

BACKGROUND Fibrotic hypersensitivity pneumonitis(FHP)is an allergic and diffuse pneumonia caused by repeated inhalation of antigenic substances,and sometimes developed in people working in specific environments.While novel antigens and exposures continued to be described,physicians should maintain a high suspicion of potential exposures.A detailed assessment of the patient's occupational exposures as well as living environment is necessary and complete allergen avoidance is the first and most important step in the management of FHP once the allergens are determined.CASE SUMMARY A 35-year-old female was admitted to the hospital with a cough and breathing difficulties for more than one year.She was a nonsmoker and a manufacturer of halogen dishes,which are characteristic Chinese foods,for 15 years without any protection.High resolution computed tomography of the chest demonstrated an interstitial pneumonia pattern.Pulmonary function examination showed restricted ventilation dysfunction and a significant reduction in dispersion ability.Cell differentiation in bronchoalveolar lavage fluid demonstrated lymphocytosis(70.4%)with an increased lymphocyte CD4/CD8 ratio(0.94).Transbronchial lung biopsy combined with lung puncture pathology showed diffuse uniform alveolar interval thickening,chronic inflammatory cell infiltration,a proliferation of tissue in the bronchial wall fiber and alveolar epithelial follicle degeneration,resulting in fibrosis.CONCLUSION Exposure to spices used for the production of halogen dishes may cause FHP.

Clinical characteristics of hypersensitivity pneumonitis:non-fibrotic and fibrotic subtypes

Background:The presence of fibrosis is a criterion for subtype classification in the newly updated hypersensitivity pneumonitis(HP)guidelines.The present study aimed to summarize differences in clinical characteristics and prognosis of non-fibrotic hypersensitivity pneumonitis(NFHP)and fibrotic hypersensitivity pneumonitis(FHP)and explore factors associated with the presence of fibrosis.Methods:In this prospective cohort study,patients diagnosed with HP through a multidisciplinary discussion were enrolled.Collected data included demographic and clinical characteristics,laboratory findings,and radiologic and histopathological features.Logistic regression analyses were performed to explore factors related to the presence of fibrosis.Results:A total of 202 patients with HP were enrolled,including 87(43.1%)NFHP patients and 115(56.9%)FHP patients.Patients with FHP were older and more frequently presented with dyspnea,crackles,and digital clubbing than patients with NFHP.Serum levels of carcinoembryonic antigen,carbohydrate antigen 125,carbohydrate antigen 153,gastrin-releasing peptide precursor,squamous cell carcinoma antigen,and antigen cytokeratin 21-1,and count of bronchoalveolar lavage(BAL)eosinophils were higher in the FHP group than in the NFHP group.BAL lymphocytosis was present in both groups,but less pronounced in the FHP group.Multivariable regression analyses revealed that older age,<20%of lymphocyte in BAL,and≥1.75%of eosinophil in BAL were risk factors for the development of FHP.Twelve patients developed adverse outcomes,with a median survival time of 12.5 months,all of whom had FHP.Conclusions:Older age,<20%of lymphocyte in BAL,and≥1.75%of eosinophil in BAL were risk factors associated with the development of FHP.Prognosis of patients with NFHP was better than that of patients with FHP.These results may provide insights into the mechanisms of fibrosis in HP.