Mycophenolate in scleroderma-associated interstitial lung disease: Real-world data from rheumatology and pulmonology clinics in South Asia.

Introduction: There is a paucity of real-world data on mycophenolate mofetil/mycophenolate sodium in systemic sclerosis-related interstitial lung disease. Aim: To study the efficacy of mycophenolate mofetil/ mycophenolate sodium in systemic sclerosis-related interstitial lung disease. Methods: In this single-centre study, clinical, laboratory and imaging details of consecutive patients with systemic sclerosis-related interstitial lung disease receiving mycophenolate mofetil/mycophenolate sodium from rheumatology and pulmonology clinics between January 2008 and March 2017 were retrospectively retrieved. The change in percentage of predicted normal forced vital capacity at last follow-up visit as compared with baseline was studied. In addition, high-resolution computed tomography scans at baseline and 2-year follow-up visit were scored as either stable/improved or worsened by experienced thoracic radiologists blinded to the clinical details of patients. Results: Altogether, 88 patients (85.2% females) with mean age (SD) of 33.8 years (± 11.3) and median (interquartile range) duration of disease since non-Raynaud's symptoms of 36 months (13.5-60) were studied. Diffuse systemic sclerosis comprised 85.2% of them. The mean baseline forced vital capacity was 61.2 ± 17.9% and median scores for ground glass opacities and fibrosis in high-resolution computed tomography were 0.5 (0-1.3) and 1 (0-1.3), respectively. At a median follow-up duration of 30 months (interquartile range = 16.5-49), the percentage of forced vital capacity improved by 1.8% (-3.82 to 9.07) as compared with baseline visit (p = 0.02). In the 2-year follow-up, the ground glass opacity and fibrosis scores in high-resolution computed tomography improved in 17.3% and 7.7% of patients and stabilized in 63.5% and 78.8% patients, respectively. Conclusion: Mycophenolate mofetil/mycophenolate sodium was efficacious in improving /stabilizing forced vital capacity irrespective of the baseline high-resolution computed tomography lung scores in our patients with systemic sclerosis-related interstitial lung disease during the ⩾ 2-year follow-up period.

Inflammatory myofibroblastic tumours of the thorax: Radiologic and clinicopathological correlation.

CONTEXT AND AIMS: Inflammatory myofibroblastic tumour (IMT) is a rare mesenchymal neoplasm with intermediate malignant potential. The aim of this study is to describe and compare the clinical presentation, computed tomography (CT) findings and anaplastic lymphoma kinase -1 (ALK-1) expression of IMT of the thorax in children and adults. We also sought to study the tumour behaviour after treatment on the follow-up imaging. MATERIALS AND METHOD: This is a retrospective observational study of 22 histopathologically proven cases of IMT in the thorax. The clinical parameters, CT findings, biopsy results, treatment received and follow-up were recorded. Statistical analysis was performed using Fisher's exact test. RESULTS: IMT of the thorax had diverse imaging appearances, presenting either as large invasive lung masses with or without calcifications or as smaller endobronchial lesions. Children commonly presented with long duration fever (P = 0.02) and large invasive lung masses (P = 0.026), whereas adults presented with long duration haemoptysis (P = 0.001) and endobronchial lesions or smaller lung parenchymal lesions. Calcifications were more common in children (P = 0.007). ALK-1 was positive in 40% of children and 18.2% of adults (P = 0.547). Endobronchial lesions showed a trend for ALK-1 negativity. Patients with bronchoscopic excision had local recurrence and patients with surgical wedge resection had metastatic brain lesions as compared to those with lobectomy and pneumonectomy (P = 0.0152). A patient with unresectable lung mass had malignant transformation to spindle cell sarcoma after 9.5 years. CONCLUSIONS: Thoracic IMT presents with some distinct clinical and CT findings in adults and children. The CT findings and management options have implications for prognosis. If resectable, lobectomy is a better option than wedge resection or bronchoscopic excision for preventing local recurrence and metastasis. IMT can undergo malignant transformation.

Utility of cine MRI in evaluation of cardiovascular invasion by mediastinal masses.

BACKGROUND: Accurate imaging assessment of cardiovascular invasion by mediastinal masses is essential for determining surgical feasibility. This can sometimes be difficult on CT owing to limited space available in the mediastinum, resulting in mediastinal masses abutting and indenting adjacent cardiovascular structures. Cine MRI may aid in such situations by demonstrating differential mobility. AIMS AND OBJECTIVES: To evaluate the role of cine MRI in assessing cardiovascular invasion by mediastinal masses, by evaluating sliding motion and the presence of chemical shift artifact between the mediastinal mass and apposing structures. MATERIAL AND METHODS: Retrospective study of 44 patients with mediastinal masses, with equivocal involvement of 162 cardiovascular structures on CT scan, in whom cine MRI was done. Involvement on CT was considered equivocal when there was a loss of intervening fat plane and broad surface (>3 cm) or angle (>90°) of contact between the mediastinal mass and cardiovascular structure. The presence of either sliding movement or type 2 chemical shift artifact or both between mass and the cardiovascular structure was considered as no adherence or invasion. The absence of both the parameters was considered as the presence of invasion or adhesion. Imaging findings were correlated with intraoperative findings. RESULTS: After excluding 25 cardiovascular structures in 7 patients, 137 cardiovascular structures whose involvement was suspected on CT were evaluated in 37 patients with mediastinal masses. In all, 31 cardiovascular structures showed invasion on MRI out of which 28 structures were invaded or adhered intraoperatively and 106 cardiovascular structures showed no invasion on MRI out of which 97 structures were intraoperatively not invaded/adhered. The sensitivity, specificity and accuracy of our study are 75.7%, 97% and 91.2%, respectively. CONCLUSION: Cine MRI can be used as an effective tool in patients with equivocal cardiovascular invasion by mediastinal masses on CT scans.

Myocardial nulling pattern in cardiac amyloidosis on time of inversion scout magnetic resonance imaging sequence - A new observation of temporal variability.

CONTEXT: The pattern of myocardial nulling in the inversion scout sequence [time of inversion scout (TIS)] of cardiac magnetic resonance imaging (MRI) is an accurate tool to detect cardiac amyloidosis. The pattern of nulling of myocardium and blood at varying times post gadolinium injection and its relationship with left ventricular mass (LVM) in amyloidosis have not been described previously. AIMS: The aim is to study the nulling pattern of myocardium and blood at varying times in TIS and assess its relationship with LVM and late gadolinium enhancement (LGE) in amyloidosis. MATERIALS AND METHODS: This was a retrospective study of 109 patients with clinical suspicion of cardiac amyloidosis who underwent MRI. Of these, 30 had MRI features of amyloidosis. The nulling pattern was assessed at 5 (TIS5min) and 10 (TIS10min) minutes (min) post contrast injection. Nulling pattern was also assessed at 3min (TIS3min) in four patients and 7min (TIS7min) in five patients. Myocardial mass index was calculated. Mann-Whitney U test was done to assess statistical difference in the myocardial mass index between patients with and without reversed nulling pattern (RNP) at TIS5min. RESULTS: RNP was observed in 58% at TIS5min and 89.6% at TIS10min. Myocardial mass index was significantly higher in patients with RNP at TIS5min[mean = 94.87 g/m2; standard deviation (SD) =17.63) when compared with patients with normal pattern (mean = 77.61 g/m2; SD = 17.21) (U = 18; P = 0.0351). CONCLUSION: In cardiac amyloidosis, TIS sequence shows temporal variability in nulling pattern. Earlier onset of reverse nulling pattern shows a trend toward more LVM and possibly more severe amyloid load.

Spectrum of pulmonary valve morphology and its relationship to pulmonary trunk in tetralogy of Fallot.

BACKGROUND: Tetralogy of Fallot (TOF) is a complex congenital heart disease with anatomic variations. Although the pulmonary valve in TOF is abnormal, it has not been studied well, especially on newer imaging modalities such as multidetector computed tomography (CT), which gives excellent anatomic detail. AIMS: The aim of this study was to assess the morphology of pulmonary valve in TOF on CT and evaluate its association with the degree of hypoplasia of infundibulum and pulmonary trunk. MATERIALS AND METHODS: The cardiac CT scans of 30 patients with TOF were reviewed to evaluate the morphology of the pulmonary valve, infundibulum, and pulmonary arteries. Fisher's exact test was performed to examine the association between pulmonary valve morphology and degree of hypoplasia of the infundibulum and pulmonary trunk. RESULTS: 16.7% of patients with TOF had pulmonary atresia. The prevalence of tricuspid, bicuspid, and absent valves were 10%, 53.3% and 6.7%, respectively. In another 13.3% of patients, although valve tissue was present, exact morphology could not be determined on CT. The commissures of 62.5% of the bicuspid valves were at 12 o'clock and 6 o'clock or slightly off the midline. There was statistically significant association between valve morphology and degree of infundibular hypoplasia (P < 0.001) and calibre of pulmonary trunk (P < 0.001). CONCLUSION: Morphological abnormality of the pulmonary valve is common in TOF. The most common type of pulmonary valve in TOF patients is bicuspid valve with commissures at 12 o'clock and 6 o'clock or slightly off the midline. Fewer cusps of the pulmonary valve are associated with a more severe degree of pulmonary artery hypoplasia.