A case of Sjögren's syndrome in which diffuse cystic lung lesions led to an accurate diagnosis.

Key Clinical Message: Even in the absence of other symptoms or other pulmonary manifestations suggesting Sjögren's syndrome (SS), it is necessary to include SS in the differential diagnosis of diffuse cystic lung disease (CLD). Abstract: A case of SS that presented initially with diffuse CLD is reported. This case is considered rare because diffuse pulmonary cysts were observed in the early stage with few symptoms, only cysts were observed without other lung lesions on imaging, cyst formation was histologically considered to be alveolar loss, and airway lesions not observed on imaging were suspected based on lung function testing. The details of this case provide extremely important information to consider for the diagnosis and management of CLD and SS.

CT Findings and Treatment Outcomes of Ground-Glass Opacity Predominant Lung Cancer After Stereotactic Body Radiotherapy.

BACKGROUND: Stereotactic body radiotherapy (SBRT) has been rapidly evolving and increasingly performed in patients with ground-glass opacity (GGO) predominant lung cancer (GGOp-LC). PURPOSE: To evaluate early-phase CT findings of GGOp-LC after SBRT. MATERIALS AND METHODS: Patients with GGOp-LC staged as cTis-2bN0M0 treated with SBRT were retrospectively identified. The CT images were analyzed using radiologists' interpretation and CT-density histograms. Long-term treatment outcomes were also assessed. RESULTS: This study evaluated 126 patients with 133 cases of GGOp-LC, comprising GGOp-LC with pure GGO (pureGGO-LC) (n = 31) and part-solid tumors (partsolid-LC) (n = 102). The median follow-up duration was 64.3 months (range, 10.8-178.9 months). Most GGOp-LC cases were interpreted as stable disease at 1 and 3 months after SBRT (96% [125/130] and 85% [62/73], respectively). However, the solid component was often interpreted as progressive disease (42% [34/82] and 60% [29/48], respectively). The GGO component was interpreted as denser in 47% (61/130) and 86% (63/73) of cases, respectively. For 25 evaluable pureGGO-LC cases at 3 months, the median tumor density values increased over time (P < .001). For 48 evaluable partsolid-LC cases at 3 months, the median areas of CT-density ≥ -160 HU increased over time (P < .001). The 5-year overall survival for GGOp-LC patients was 78.0%. No local or regional recurrence were observed. CONCLUSION: Clinical outcomes of SBRT for GGOp-LC were excellent, without local or regional recurrence. In the interpretation of early-phase follow-up CT scans of GGOp-LC after SBRT, it should be noted that most GGOp-LC remains stable disease, solid component increases in size, and GGO component is denser.

SMARCA4-deficient lung tumour that presented with haemoptysis and progressed rapidly.

The case of a heavy ex-smoking man in his early 70s who presented with haemoptysis and died following rapid progression is presented. The tumour excised by surgery was mostly composed of monotonous large rhabdoid cells showing prominent nucleoli and eosinophilic cytoplasm. On immunohistochemistry with SMARCA4 (BRG-1), the tumour cells showed significant loss of expression. The tumour was diagnosed as a SMARCA4-deficient thoracic sarcoma. This is a disease that progresses rapidly and has a poor prognosis. However, the search for specific treatments using synthetic lethality is underway. Clinical and pathological characteristics can be identified with examination of more cases, and when the tumour is suspected, it is necessary to actively perform immunohistochemical examination.

Stereotactic body radiotherapy for lung cancer patients with idiopathic interstitial pneumonias.

PURPOSE: To compare toxicity and survival after stereotactic body radiotherapy (SBRT) between lung cancer patients with or without idiopathic interstitial pneumonias (IIPs), and to investigate the potential value of SBRT for the patients. METHODS: Among lung cancer patients receiving SBRT between 2005 and 2016, we evaluated those treated with a total dose of 40-60Gy in five fractions with curative intent who either were staged as cT1-4N0M0 or experienced postoperative isolated local recurrence. We analyzed the incidence of radiation pneumonitis (RP) in all patients and local recurrence and overall survival (OS) in T1a-2a patients. RESULTS: A total of 508 patients were eligible, including 42 with IIPs. The median follow-up was 32.3 (6.0-120.9) months. Significantly more patients with IIPs had grade ≥3RP than did those without IIPs (12% vs. 3%, p=0.009). The 2-year local recurrence rate was low in both groups (3.4% vs. 5.6%, p=0.38). The 2-year OS rate was significantly lower in the patients with IIPs (42.2% vs. 80.9%, p<0.001), although death from lung cancer was comparable (p=0.74). CONCLUSION: SBRT achieved excellent local control with acceptable pulmonary toxicity in lung cancer patients with IIPs. SBRT can be a reasonable option for early lung cancer patients with IIPs.

Effects of extrathoracic mechanical ventilation on pulmonary hypertension secondary to lung disease.

PURPOSE: Biphasic cuirass ventilation (BCV) is a form of non-invasive extrathoracic positive and negative pressure mechanical ventilation. The present study was conducted to quantify our positive experience using BCV to dramatically improve gas exchange and cardiac function in patients with acute exacerbation of chronic respiratory failure and secondary pulmonary hypertension (PH). METHODS: BCV was applied for 2 weeks in 17 patients with PH caused by lung disease. Ventilation sessions were limited to 1 h per day to prevent exhaustion. To assess respiratory and circulatory effects, percutaneous arterial oxygen saturation (SpO2) was measured before and after each daily BCV session, and right heart catheter test [mean pulmonary artery pressure (mPAP), right atrium pressure (RAP), pulmonary artery occlusion pressure (PAOP) and cardiac index (CI)] and serum N-terminal pro-brain natriuretic peptide (NT-proBNP) were measured before and after a series of BCV sessions. RESULTS: SpO2 transiently improved after each BCV session. After a series of BCV, mPAP decreased from 27.2 to 22.4 mmHg (p = 0.0007). PAOP, CI and serum NT-proBNP levels decreased compared with baseline. No patients were treated with epoprostenol, iloprost, bosentan or sildenafil for PH. CONCLUSION: BCV may improve circulatory function in patients with PH caused by lung disease.

Stereotactic body radiotherapy for T3 and T4N0M0 non-small cell lung cancer.

To evaluate the outcomes and feasibility of stereotactic body radiotherapy (SBRT) for cT3 and cT4N0M0 non-small cell lung cancer (NSCLC), 25 patients with localized primary NSCLC diagnosed as cT3 or cT4N0M0, given SBRT between May 2005 and July 2013, were analyzed. All patients had inoperable tumors. The major reasons for tumors being unresectable were insufficient respiratory function for curative resection, advanced age (>80 years old) or technically inoperable due to invasion into critical organs. The median patient age was 79 years (range; 60-86). The median follow-up duration was 25 months (range: 5-100 months). The 2-year overall survival rates for T3 and T4 were 57% and 69%, respectively. The 2-year local control rates for T3 and T4 were 91% and 68%, respectively. As for toxicities, Grade 0-1, Grade 2 and Grade 3 radiation pneumonitis occurred in 23, 1 and 1 patient, respectively. No other acute or symptomatic late toxicities were reported. Thirteen patients who had no local, mediastinal or intrapulmonary progression at one year after SBRT underwent pulmonary function testing. The median variation in pre-SBRT and post-SBRT forced expiratory volume in 1 s (FEV1) values was -0.1 (-0.8-0.8). This variation was not statistically significant (P = 0.56). Forced vital capacity (FVC), vital capacity (VC), %VC and %FEV1 also showed no significant differences. SBRT for cT3 and cT4N0M0 NSCLC was both effective and feasible. Considering the favorable survival and low morbidity rate, SBRT is a potential treatment option for cT3 and cT4N0M0 NSCLC.

A Surgical Case of Bronchial Artery Aneurysm Directory Connecting with Pulmonary Artery.

We present a surgical case of bronchial artery aneurysm (BAA) connecting pulmonary artery accompanied with racemose hemangioma. This is a third surgical case report of BAA directly connecting pulmonary artery in the English literature. A 63-year-old female was found a BAA, 2 cm in diameter, connecting right A4 pulmonary artery. The patient underwent two attempts for embolization. However, due to extensive collaterals, there was persistent flow in the aneurysm. Standard lateral thoracotomy was performed. A BAA was located between A4 and A5 PA. A small branch of A4 PA was separated, and the small vessel connecting to the BAA could be ligated. A5 PA was separated similarly, however BAA was ruptured not to identify the other small vessel connecting to the BAA. After a clamp of the BAA, middle lobe lobectomy was performed. We removed the aneurysm with dilated bronchial artery connecting to the aneurysm. The postoperative course was uneventful.