BK virus-associated nephropathy in a lung transplant patient: case report and literature review.

BACKGROUND: BK virus-associated nephropathy (BKVAN) is a relatively common cause of renal dysfunction in the first six months after renal transplantation. It arises from reactivation of the latent and usually harmless BK virus (BK virus) due to immunosuppression and other factors including some that are unique to renal transplantation such as allograft injury. BKVAN is much rarer in non-renal solid organ transplantation, where data regarding diagnosis and management are extremely limited. CASE PRESENTATION: We report a case of a 58-year-old man found to have worsening renal dysfunction nine months after bilateral sequential lung transplantation for chronic obstructive pulmonary disease (COPD). He had required methylprednisolone for acute allograft rejection but achieved good graft function. Urine microscopy and culture and renal ultrasound were normal. BK virus PCR was positive at high levels in urine and blood. Renal biopsy subsequently confirmed BKVAN. The patient progressed to end-stage renal failure requiring haemodialysis despite reduction in immunosuppression, including switching mycophenolate for everolimus, and the administration of intravenous immunoglobulin (IVIG). CONCLUSIONS: This very rare case highlights the challenges presented by BK virus in the non-renal solid organ transplant population. Diagnosis can be difficult, especially given the heterogeneity with which BKV disease has been reported to present in such patients, and the optimal approach to management is unknown. Balancing reduction in immunosuppression against prevention of allograft rejection is delicate. Improved therapeutic options are clearly required.

Progressive multifocal leukoencephalopathy in a lung transplant recipient presenting with memory impairment: Case report and literature review.

BACKGROUND: Progressive multifocal leukoencephalopathy (PML) is a rare demyelinating disease of the central nervous system caused by JC virus (JCV). The disease occurs in the setting of significant immunocompromise and has now been reported in many different settings, although only very rarely after lung transplantation. The mortality rate is high and therapeutic options are limited. CASE PRESENTATION: We report a case of a 66-year-old man who presented with non-specific memory disturbance at 19 months after lung transplantation for chronic hypersensitivity pneumonitis. He had required methylprednisolone for acute allograft rejection but achieved good graft function. Physical examination was unremarkable. CT revealed hypodensity in the left frontal lobe. MR demonstrated significant hyperintense white-matter abnormalities on T2-weighted and fluid-attenuated inversion recovery (FLAIR) sequences, mainly focused on the periventricular region adjacent the frontal horn of the left lateral ventricle. Brain biopsy confirmed PML. The patient had his immunosuppression reduced but then developed antibody-mediated rejection four months later. Despite re-escalation of immunosuppression, he remains neurologically stable on mirtazapine at eight months post-diagnosis. CONCLUSIONS: This very rare case highlights the challenges presented by PML, especially in the lung transplant population. It reveals the difficult balance between reducing immunosuppression to protect the brain versus prevention of lung allograft rejection. It clearly highlights the need for improved therapeutic modalities.

Case report of severe bronchial web-like stenoses after 'surviving the unsurvivable'.

BACKGROUND: There are few cases of multiple bronchial stenoses reported in the literature and none of the severity described here. The case is relevant due to its rareness, the pathophysiological insights derived, the successful interventional pulmonology strategies demonstrated, and as an example of a rare indication for high-risk lung transplantation. CASE PRESENTATION: A 47-year-old man developed multiple recurrent bronchial web-like stenoses five weeks after an episode of severe tracheo-bronchitis presumed secondary to a chemical inhalation injury which initially caused complete bilateral lung collapse necessitating veno-venous extracorporeal membrane oxygenation. The stenoses completely effaced bronchi in many locations causing severe type II respiratory failure requiring mechanical ventilation and bronchoscopic puncture / dilatation then ultimately bilateral lung transplantation. CONCLUSION: This very rare case highlights the morbid sequelae that can arise after catastrophic tracheobronchitis which now, in the era of extracorporeal membrane oxygenation, may be survivable in the short-term.

Impulse Oscillometry Versus Spirometry to Detect Bronchiolitis Obliterans Syndrome in Bilateral Lung Transplant Recipients: A Prospective Diagnostic Study.

BACKGROUND: Chronic lung allograft dysfunction (CLAD), and especially bronchiolitis obliterans syndrome (BOS), remain dominant causes of morbidity and mortality after lung transplantation. Interest is growing in the forced oscillation technique, of which impulse oscillometry (IOS) is a form, as a tool to improve our understanding of these disorders. However, data remain limited and no longitudinal studies have been published, meaning there is no information regarding any capacity IOS may have for the early detection of CLAD. METHODS: We conducted a prospective longitudinal study enrolling a consecutive sample of adult bilateral lung transplant recipients with healthy lung allografts or CLAD and performed ongoing paired IOS and spirometry tests on a clinically determined basis. We assessed for correlations between IOS and spirometry and examined any predictive value either modality may hold for the early detection of BOS. RESULTS: We enrolled 91 patients and conducted testing for 43 mo, collecting 558 analyzable paired IOS and spirometry tests, with a median of 9 tests per subject (interquartile range, 5-12) and a median testing interval of 92 d (interquartile range, 62-161). Statistically significant moderate-to-strong correlations were demonstrated between all IOS parameters and spirometry, except resistance at 20 Hz, which is a proximal airway measure. No predictive value for the early detection of BOS was found for IOS or spirometry. CONCLUSIONS: This study presents the first longitudinal data from IOS after lung transplantation and adds considerably to the growing literature, showing unequivocal correlations with spirometry but failing to demonstrate a predictive value for BOS.

Hepatic artery dissection in a 65-year-old woman with acute pancreatitis.

We describe a case of hepatic artery dissection in a 65-year-old woman. This is the 22nd reported case of hepatic artery dissection, and the first associated with mild acute pancreatitis. The incidence of this condition may be increasing with rising rates of intervention affecting the biliary system. Recognition may also be increasing with growing use of high-quality radiography. Treatment should prevent rupture and ameliorate cardiovascular risk. Surgery should be considered in patients with complications or those likely to have them in future. There is a paucity of data regarding hepatic artery dissection, limiting evidence for guiding management.

Management of Multidrug Resistant Infections in Lung Transplant Recipients with Cystic Fibrosis.

Cystic fibrosis (CF) is an inherited multisystem disease characterised by bronchiectasis and chronic respiratory infections which eventually cause end stage lung disease. Lung transplantation (LTx) is a well-established treatment option for patients with CF-associated lung disease, improving survival and quality of life. Navigating recurrent infections in the setting of LTx is often difficult, where immune suppression must be balanced against the constant threat of infection. Sepsis/infections are one of the major contributors to post-LTx mortality and multiresistant organisms (eg, Burkholderia cepacia complex, Mycobacterium abscessus complex, Scedosporium spp. and Lomentospora spp.) pose a significant threat to survival. This review will summarize current and novel therapies to assist with the management of multiresistant bacterial, mycobacterial, viral and fungal infections which threaten the CF LTx cohort.

Paraneoplastic acute fibrinous and organizing pneumonia from lymphoma completely responding to bendamustine-rituximab.

Acute fibrinous and organizing pneumonia (AFOP) is a rare histopathological pattern of lung injury characterized by prominent fibrin deposition in alveolar spaces. It may be idiopathic or associated with medications, connective tissue disease, infection, environmental exposures, transplantation, and malignancy. There is no proven treatment but multiple reports describe response to corticosteroids. We report the case of a 65-year-old male never-smoker with a 15-month history of dry cough, dyspnoea, anorexia, and night sweats only partially responsive to doxycycline and oral prednisolone. Computed tomography chest demonstrated adenopathy on both sides of the diaphragm and patchy consolidation in a peribronchovascular and subpleural distribution with lower zone predominance. Axillary node biopsy revealed low-grade non-Hodgkin's lymphoma. Lung biopsy showed AFOP but no lymphoma. Complete pulmonary and neoplastic responses were achieved with bendamustine-rituximab. We report a compelling instance of paraneoplastic AFOP responding to chemotherapy for lymphoma with very limited use of corticosteroids.

A challenging diagnosis of malignant mesothelioma with osteosarcomatous differentiation metastasizing to bone.

Malignant pleural mesothelioma (MPM) is an insidious primary neoplasm of the pleura that can be challenging to diagnose and is commonly considered to be only locally invasive. We present the case of a 74-year-old male who presented with clinical features of MPM but from whom pleural fluid and biopsies initially suggested benign pathology. He later developed diffuse bony metastases and re-examination of pleural biopsies using modern immunohistochemistry and molecular testing revealed a diagnosis of sarcomatoid and desmoplastic MPM with heterologous osteosarcomatous differentiation. This case not only demonstrates the rare potential of skeletal metastasis of MPM, but also highlights the importance of recognizing the utility of modern diagnostic tests and their potential to prevent the need for unnecessary invasive procedures. To our knowledge this is the first description of this rare histological sub-type presenting with skeletal metastases.

The utility of fluorine-18-fluorodeoxyglucose positron emission tomography in the diagnosis and monitoring of large vessel vasculitis: A South Australian retrospective audit.

AIMS: To investigate the utility of fluorine-18-labelled deoxyglucose positron emission tomography (FDG-PET) in routine clinical practice to diagnose and monitor disease activity and treatment response in large vessel vasculitis in a South Australian cohort. METHODS: We performed a retrospective clinical audit of adult patients who received a FDG-PET at a tertiary referral center between August 2010 and August 2015, where the term "vasculitis" appeared in either the request or report. RESULTS: A total of 45 patients met the inclusion criteria. Nine patients (20%) had a positive FDG-PET for large vessel vasculitis. FDG-PET was positive in 3/6 (50%) patients who met the American College of Rheumatology (ACR) criteria for giant cell arteritis or Takayasu's arteritis (TA) on retrospective review. A positive FDG-PET for large vessel inflammation assisted the primary clinician in making the diagnosis of unclassified large vessel vasculitis in six patients. Four of the seven patients who had more than one scan for large vessel vasculitis demonstrated normalized FDG uptake on subsequent scans after a period on glucocorticoid treatment. The remaining three patients persisted in having increased FDG uptake on FDG-PET imaging while on active treatment. CONCLUSION: Fluorine-18-labelled deoxyglucose positron emission tomography has a role in the diagnosis of large vessel vasculitis, particularly in patients with a high suspicion of active large vessel vasculitis who do not meet the ACR criteria. FDG-PET may have a role in monitoring disease activity in selected patients with large vessel vasculitis especially in identifying occult sites of large vessel inflammation or to titrate prednisolone therapy.

De novo Propionibacterium acnes septic arthritis.

We report the third identifiable case of septic arthritis due to Propionibacterium acnes arising in the absence of prior surgical intervention. This anaerobic Gram-positive bacillus is now recognised as an important cause of postoperative infections, typically presenting in an indolent fashion some months after surgery. Reports of de novo septic arthritis due to P. acnes are exceedingly rare. Our case adds to the literature and significantly broadens the reported pathogenic potential of the organism, which in this instance has caused rapid and serious joint destruction.

Sensitivity and specificity of three-point compression ultrasonography performed by emergency physicians for proximal lower extremity deep venous thrombosis.

OBJECTIVE: The present study aims to quantify the sensitivity and specificity of three-point compression ultrasonography for diagnosing proximal lower extremity deep venous thrombosis when performed by Australian consultant emergency physicians with limited specific training. Secondary aims included quantifying rapidity, technical adequacy, predictability of equivocal results and relationships between emergency physician experience and proficiency. METHODS: This prospective diagnostic study enrolled a convenience sample of adult patients presenting to a major ED with suspected lower extremity deep venous thrombosis. The index test was abbreviated compression ultrasonography examining three points: common femoral, proximal great saphenous and popliteal veins. Emergency physicians received specific training. The reference test was full-leg duplex ultrasonography in the Radiology Department. RESULTS: A total of 15 emergency physicians participated, enrolling 178 subjects. Sensitivity of the index test was 77.8% (95% confidence interval: 54.8-91.0%), specificity was 91.4% (95% confidence interval: 84.9%-95.3%) and accuracy was 89.6% (95% confidence interval: 83.1-94.2%). Median duration of the index test was 10 min 34 s (interquartile range: 6 min 31 s) and ED diagnosis occurred significantly before Radiology Department diagnosis. The only statistically significant relationship between emergency physician experience and proficiency related to rapidity, which increased from the 36th scan. Equivocal index tests occurred in 9.2% of examinations and emergency physicians predicted equivocal assessments with specificity of 86.1% (95% confidence interval: 78.8-91.1%). CONCLUSIONS: Abbreviated ultrasonography performed by emergency physicians for proximal lower extremity deep venous thrombosis could be valuable. However, more precise estimates for sensitivity and greater understanding of relationships between emergency physician experience and proficiency are required.