Diagnosis and management of idiopathic pulmonary fibrosis: Thoracic Society of Australia and New Zealand and Lung Foundation Australia position statements summary.

INTRODUCTION: Idiopathic pulmonary fibrosis (IPF) is a fibrosing interstitial lung disease associated with debilitating symptoms of dyspnoea and cough, resulting in respiratory failure, impaired quality of life and ultimately death. Diagnosing IPF can be challenging, as it often shares many features with other interstitial lung diseases. In this article, we summarise recent joint position statements on the diagnosis and management of IPF from the Thoracic Society of Australia and New Zealand and Lung Foundation Australia, specifically tailored for physicians across Australia and New Zealand. Main suggestions: A comprehensive multidisciplinary team meeting is suggested to establish a prompt and precise IPF diagnosis. Antifibrotic therapies should be considered to slow disease progression. However, enthusiasm should be tempered by the lack of evidence in many IPF subgroups, particularly the broader disease severity spectrum. Non-pharmacological interventions including pulmonary rehabilitation, supplemental oxygen, appropriate treatment of comorbidities and disease-related symptoms remain crucial to optimal management. Despite recent advances, IPF remains a fatal disease and suitable patients should be referred for lung transplantation assessment.

Management of refractory chylothorax in pulmonary lymphangioleiomyomatosis.

This case reports the successful management of chylothorax in a non-transplanted patient with pulmonary lymphangioleiomyomatosis (pLAM). Prolonged initial therapy failed, including total parenteral nutrition, pleural drainage, surgical pleurodesis, and pleurectomy. Commencement of sirolimus 2 mg daily (2 mg alternating days had failed) led to resolution of chylothorax after 20 days. Discontinuation of sirolimus for abdominal surgery led to recurrence of the chylothorax. Reinstitution of sirolimus led to rapid resolution of the effusion, stabilization of lung function, and there has been no recurrence in the ensuing 4 years. We conclude that sirolimus should be considered in the management of pLAM-related chylothorax, perhaps before surgical intervention.

Autoimmune disease leading to pulmonary AL amyloidosis and pulmonary hypertension.

A 33-year-old woman with past history of Sjögren's syndrome and systemic lupus erythematosus presented with dyspnea and syncope secondary to pulmonary hypertension. After progressive symptoms over 4 years, she received bilateral lung transplantation. Histopathology of the explanted lungs showed isolated pulmonary amyloid light-chain amyloidosis and pulmonary cysts. No evidence of systemic amyloidosis was found at the time of transplantation. Seven years post lung transplantation, she remains well with no evidence of systemic amyloidosis recurrence.

Proliferating bronchial webs after lung transplantation.

This case details the decline in lung function due to bronchial webs in a lung transplant recipient. The decline occurred 2 years after transplantation and, despite therapy, the webs, which had an inflammatory component, became treatment resistant. We outline the pathological findings and management strategies used, discuss the evidence in the literature, and offer possible causes for these unusual clinical findings.

Surveillance bronchoscopy in lung transplant recipients: risk versus benefit.

BACKGROUND: Long-term survival of lung transplant (LT) recipients is limited by the development of the bronchiolitis obliterans syndrome (BOS). A number of risk factors for BOS have been identified, which can be detected using bronchoscopy with transbronchial biopsy (TBB). Many LT units perform routine surveillance bronchoscopy (SB) to detect problems such as: acute rejection (AR); infection, particularly with cytomegalovirus (CMV); and lymphocytic bronchiolitis. This study aimed to assess the safety and efficacy of surveillance bronchoscopy in lung transplant recipients (LTRs), including TBB and bronchoalveolar lavage (BAL). METHODS: All bronchoscopy procedures, including SB and clinically indicated (CB) procedures performed on LTRs in one calendar year, were audited prospectively. Complications and clinical utility were recorded to determine the clinical utility both early (3 months and 3 to 12 months) and late (>12 months) post-LT. RESULTS: In one calendar year, 353 procedures (232 SBs and 121 CBs) were performed on 124 LTRs, with 246 performed <1 year post-LT. The complication rates were similar to those reported previously, except for an increased rate of sedation-related complications, particularly up to 3 months post-LT. SBs showed high rates of acute rejection, particularly in the first year post-LT (p = 0.01). The rate of asymptomatic infection diagnosed on BAL remained high regardless of time post-transplant. CONCLUSIONS: This study confirms that SB can frequently detect clinically significant infection and rejection with very low complication rates. The data support SB with TBB up to 12 months post-LT, and ongoing use of SB with BAL (only) to detect clinically silent infection beyond 1 year post-LT.

Regression of pulmonary lymphangioleiomyomatosis (PLAM)-associated retroperitoneal angiomyolipoma post-lung transplantation with rapamycin treatment.

Pulmonary lymphangioleiomyomatosis (PLAM) is an indication for lung transplantation (LTx). Angiomyolipomas occur in approximately 50% to 60% of patients with PLAM. We describe a patient presenting with hemoptysis post-LTx for PLAM. Computed tomography (CT) scan demonstrated no pulmonary abnormality, but identified a retroperitoneal mass confirmed as angiomyolipoma by CT-guided core biopsy. Based on experimental work that rapamycin may inhibit angiomyolipoma cells, we commenced the patient on low-dose rapamycin. She had no adverse reactions and follow-up CT scan after 7 months demonstrated almost complete resolution of the tumor. This suggests a role for rapamycin in routine post-LTx immunosuppression for PLAM.

Everolimus alters the bronchoalveolar lavage and endobronchial biopsy immunologic profile post-human lung transplantation.

Everolimus has recently shown promise in terms of short- and long-term clinical lung transplant outcomes. This study aims to determine the altered lung allograft cellular and cytokine mileau when everolimus is substituted for azathioprine (AZA). Twenty-three stable lung transplantation (LTx) recipients were randomized in a double-blinded study to receive everolimus (13) or AZA (10) plus standard cyclosporine/prednisolone. Bronchoalveolar lavage (BAL) and endobronchial biopsies (EBB) were performed on three occasions (T(0)-T(2)) to elucidate cellular and cytokine profiles via immunocytochemistry, immunohistology and enzyme-linked immunosorbent assay (ELISA) techniques. There were no group differences for demographics or clinical events throughout the study nor baseline cellular/cytokine differences. BAL lymphocyte percentage fell in the AZA group by T(2) (p = 0.05). BAL and EBB CD4 measures significantly declined in the everolimus group by T(2) (p < 0.05). EBB neutrophils rose significantly in the AZA group, with a fall in the everolimus group resulting in a significant difference at T(2) (p = 0.01). In conclusion, everolimus has contributed to potentially important differences in BAL and EBB cellular profiles.