Familial interstitial lung disease in children: Response to chloroquine treatment in one sibling with desquamative interstitial pneumonitis

We describe a male infant with biopsy-confirmed interstitial lung disease (ILD) who responded to chloroquine, after he failed to improve on oral corticosteroids or cyclophosphamide. The infant presented at 8 days of age with respiratory distress and cyanosis. Lung biopsy at 8 weeks of age was consistent with desquamative interstitial pneumonitis (DIP). He was treated with corticosteroids at 2 weeks of age because of a family history of two siblings who died during infancy and who had DIP on postmortem examination. At 8.5 months, our patient was treated with cyclophosphamide because of lack of response to corticosteroids therapy. At 14 months of age, he began treatment with chloroquine in addition to corticosteroids and had a dramatic response within 3 weeks. The patient has been maintained successfully on continuous treatment with chloroquine alone for more than 9 years since this treatment was started. Pediatr. Pulmonol. 1997; 23:55–61. © 1997 Wiley-Liss, Inc.     

Induction of interstitial pneumonitis during interferon treatment for chronic hepatitis C

A 48-year-old woman developed interstitial pneumonitis while receiving interferon treatment for chronic hepatitis C. Laboratory studies prior to treatment showed elevated serum alanine aminotransferase levels, but chest X-rays and physical examination revealed no abnormalities suggestive of interstitial pneumonitis. At the 9th week of interferon treatment (total dose, 380MU of recombinant interferon-α, without other medications), the patient began to complain of cough and exertional dyspnea. A chest X-ray film revealed diffuse reticulo-nodular shadows in bilateral lung fields, suggesting a diagnosis of interstitial pneumonitis. A marked increase in lymphocyte count was observed in bronchoalveolar lavage fluid and a diagnosis of interstitial pneumonitis was made as a result of transbronchial lung biopsy. Her clinical symptoms and abnormal lung shadows were reversible, improving within a month of the discontinuation of interferon, and disappearing promptly after exogenous corticosteroid was instituted. This may be the first case of drug-induced interstitial pneumonitis occurring during the course of treatment with interferon alone. We should be aware of the possibility of interstitial pneumonitis developing during treatment of chronic hepatitis C with interferon.     

Cyclosporine treatment of advanced interstitial pneumonitis associated with systemic sclerosis

Abstract

A patient who had systemic sclerosis (SSc) with interstitial pneumonitis (IP) was being treated with prednisolone,d-penicillamine (D-P) and colchicine but developed progressive respiratory insufficiency. His ventilatory function showed the progression of restrictive disturbance without an obstructive one. We thought that this worsening was due to the developing IP but not bronchiolitis obliterans induced by D-P and started cyclosporine (CSA) therapy at 3 mg/kg/day. His symptoms improved after 3 months, and pulmonary function tests and blood gas analysis showed the best results after 1 year. There were no life-threatening side effects. CSA is an acceptable agent for advanced interstitial pneumonitis associated with SSc.     

Cyclosporine treatment of advanced interstitial pneumonitis associated with systemic sclerosis

A patient who had systemic sclerosis (SSc) with interstitial pneumonitis (IP) was being treated with prednisolone,d-penicillamine (D-P) and colchicine but developed progressive respiratory insufficiency. His ventilatory function showed the progression of restrictive disturbance without an obstructive one. We thought that this worsening was due to the developing IP but not bronchiolitis obliterans induced by D-P and started cyclosporine (CSA) therapy at 3 mg/kg/day. His symptoms improved after 3 months, and pulmonary function tests and blood gas analysis showed the best results after 1 year. There were no life-threatening side effects. CSA is an acceptable agent for advanced interstitial pneumonitis associated with SSc.     

Hydroxyurea-induced pneumonitis in a patient with chronic idiopathic myelofibrosis after prolonged drug exposure

We describe a 63-yr-old patient diagnosed with chronic idiopathic myelofibrosis whose disease was treated with hydroxyurea for 2 yr. He developed respiratory symptoms that were extensively investigated including lung biopsy. Clinical, radiological and histological features were compatible with a diagnosis of hydroxyurea-induced pneumonitis. Following drug withdrawal there was remarkable improvement in clinical and radiological findings.     

Trastuzumab (Herceptin)-associated lung injury

We report a case of trastuzumab pneumonitis in a patient with metastatic breast cancer. Bronchoalveolar lavage showed marked neutrophilia. A CT scan of the chest showed diffuse ground-glass opacities. The patient was treated with corticosteroids with a partial response. Trastuzumab-associated pneumonitis is not well described, and data in the literature is sparse. We describe the clinical and radiographical findings of trastuzumab-associated pneumonitis.     

Leflunomide-induced lung injury that developed after its withdrawal, coinciding with peripheral blood lymphocyte count decrease

A 60-year-old rheumatoid arthritis (RA) female with lung fibrosis was treated with leflunomide (LEF) for only 12 days, and responded well. Twenty-five days after the withdrawal of the drug, she had fever, dyspnea, and an elevated serum C-reactive protein level. Chest CT revealed ground-glass opacities (GGOs) and consolidations forming a mosaic pattern, in lung fields including the upper, anterior and central areas, and honeycomb patterns in the lung bases and backs. The level of plasma A771726, an active metabolite of LEF, was still as high as that usually noted under LEF therapy. After pulsed steroid and cholestyramine administration, A771726 was depleted and she recovered. The peripheral blood lymphocyte count that had been approximately 1,000/μL, decreased to 220/μL just at the onset of lung injury, and rapidly and steadily returned to the preinjury level preceding recovery from the injury. Serum albumin level decreased in association with lung injury, and gradually returned to the preinjury level. Special caution is necessary when prescribing leflunomide to elderly patients with preexisting interstitial lung disease, and remains necessary until at least 1 month after its withdrawal.     

Accelerated usual interstitial pneumonitis,anti-DNA antibodies and hypocomplementemia

A healthy 60-year-old patient presented with progressive dyspnoea. Clinical, radiographic and pathological features of interstitial lung disease were found and an open lung biopsy established the diagnosis of usual interstitial pneumonitis (UIP) (idiopathic pulmonary fibrosis). Despite treatment, the patient died 4 months later in respiratory failure. Although the patient had no extra-thoracic involvement at autopsy, his illness was associated with a very high titre of anti-double-stranded DNA antibodies, hypocomplementemia, hypergammaglobulinaemia and lymphoid hyperplasia. These features and a literature review, suggest immune-mediated lung damage in a subset of patients with UIP.     

类风湿性关节炎相关间质性肺疾病的研究进展

间质性肺疾病(interstitial lung disease,ILD)是类风湿性关节炎(rheumatoid arthritis,RA)常见的关节外病变和主要的死亡原因之一,早期诊治类风湿性关节炎相关间质性肺疾病(RA-ILD)直接影响RA患者预后。本文对近年RA-ILD的研究进展作简要概述,以期提高对该病的认识。     

Idiopathic interstitial pneumonitis in children: a national survey in the United Kingdom and Ireland

Interstitial lung disease (ILD) of unknown etiology in immunocompetent patients is rare in children. A national survey was carried out in the United Kingdom and Ireland over a 3-year period in order to identify prevalence, age distribution, histopathology, natural history of the illness, and response to current treatment.Forty-six cases were identified, including 29 males and 17 females. Seventy-six percent presented in the first year of life. Nine (16%) occurred within four families. Conventional treatment with pulsed methylprednisolone, prednisolone, or hydroxychloroquine, singly or in combination, resulted in an excellent response in 65% of cases. Seven children died (15%). The recurrence risk for further children within the same family to develop ILD is estimated to be approximately 10%. The prevalence rate of this condition in the United Kingdom and Ireland during the period of study for children aged 0-16 years is estimated to be 3.6 cases/million.     

Candida pneumonia in a case of polymyositis complicated with interstitial pneumonitis

Abstract

We report a polymyositis patient who developed Candida pneumonia, whose clinical manifestations such as dry cough, dyspnea, fever and elevated LDH looked like a recurrence of interstitial pneumonitis (IP) which she had had at admission. The distribution of the lesions in high resolution computed tomography scan, the high serum level of β-d-glucan and positive Candida antigen test led to make a diagnosis of Candida pneumonia rather than IP, and fluconazole therapy was successful.     

Features of transbronchial lung cryobiopsy‐diagnosed fibrotic hypersensitivity pneumonitis

BackgroundHypersensitivity pneumonitis (HP) is a common type among all the interstitial lung diseases, and transbronchial lung cryobiopsy is an alternative diagnostic technique for interstitial lung diseases. In this study, we describe the clinical and pathological features of fibrotic hypersensitivity pneumonitis diagnosed with transbronchial lung cryobiopsy (TBLC).MethodsA total of 46 diffused parenchyma lung disease (DPLD) patients received TBLC were included in this study. Medical records including medical history spirometry examinations, 6‐min walk test (6MWT) results, high resolution computed tomographic (HRCT) scans, BAL, and histopathology were collected. Results of HRCT and histopathology were compared and classified, especially.ResultsSixteen patients were diagnosed with fibrotic HP, the mean age of whom was 56.3 ± 12.1 years, and 62.5% of them were male. Three of the 16 patients had been misdiagnosed as tuberculosis and received antituberculosis medications, five patients had been diagnosed as unclassifiable pulmonary fibrosis, and five patients had been diagnosed as idiopathic pulmonary fibrosis (IPF). Thirteen (81.3%) patients had a normal lymphocyte count in BAL. The pathological features of usual interstitial pneumonia (UIP) were detected in 11 (68.8%) of the cases, poor defined granulomatous was detected in nine (56.3%) of the cases, and bronchiolocentric fibrosis was detected in two (12.5%) of the 16 cases.ConclusionsFibrotic hypersensitivity pneumonitis should be included in differential diagnosis of pulmonary fibrosis. Pathological characteristics of fibrotic hypersensitivity pneumonitis could be demonstrated from cryobiopsy lung tissue. TBLC is recommended as an alternative diagnostic technique, which may improve the specificity of hypersensitivity pneumonia detection, and UIP is the most frequent pathological finding.     

Exacerbation of pulmonary fibrosis following single lung transplantation

Acute exacerbations of interstitial lung disease present as clinical deteriorations, with progressive hypoxemia and parenchymal consolidation not related to infection, heart failure or thromboembolic disease. Following single lung transplantation, patients receive maintenance immunosuppression, which could mitigate the development of acute exacerbations in the native lung. A 66-year-old man with fibrotic, nonspecific interstitial pneumonitis presented with fever, hypoxemia and parenchymal consolidation limited to the native lung four years after single lung transplantation. Investigations were negative for infection, heart failure and thromboembolic disease. The patient worsened over the course of one week despite broad-spectrum antimicrobial therapy, but subsequently improved promptly with augmentation of prednisone dosed to 50 mg daily and addition of N-acetylcysteine. Hence, the patient fulfilled the criteria for a diagnosis of an acute exacerbation of pulmonary fibrosis in his native lung. Clinicians should consider acute exacerbation of parenchymal lung disease of the native lung in the differential diagnosis of progressive respiratory deterioration following single lung transplantation for pulmonary fibrosis.