Pleural Involvement in IgG4-Related Disease: Case Report and Review of the Literature

Diagnostic work-up of IgG4-related disease (IgG4-RD) pleural involvement is a complex task, as there is a broad spectrum of differential diagnoses to consider. We report the case of a patient presenting with relapsing pleural effusion, discussing the main challenges for achievement of a definite diagnosis. A 63-year-old man was admitted for pleural effusion prevalent on the ride side, initially labeled as idiopathic non-specific pleuritis, based on tissue evaluation after a medical thoracoscopy. He was started on steroids with initial improvement, but a later CT scan showed a relapse of pleural effusion associated with diffuse pleural thickening; a subsequent surgical pleural biopsy revealed features suggestive for IgG4-RD, with a marked increase of IgG4 positive plasma cells. High IgG4 serum levels were also found. The present case underlines the importance of increasing awareness of this potential condition among physicians in order to properly guide the diagnostic work-up, as it is likely that IgG4-RD accounts for a proportion of patients with pleural effusions, labeled as idiopathic. In particular, in patients with unexplained pleural effusion, IgG4-RD should be included among differential diagnoses when lymphoplasmacytic infiltration is observed, and a multidisciplinary interaction between clinicians and pathologists appears crucial for an accurate diagnosis and an appropriate management.

Unusual case of chronic cavitary pulmonary aspergillosis presenting as spontaneous pneumothorax in an immunocompromised man

Chronic cavitary pulmonary aspergillosis (CCPA) is a slow destructive type of chronic pulmonary aspergillosis, characterised by multiple pulmonary cavities that develop and expand over several months or years. Pleural involvement in the form of pneumothorax has been rarely reported in CCPA. We report such an unusual case of an immunocompromised male, with a history of chronic cough and fever, presenting with acute onset of shortness of breath. Chest imaging showed bilateral multiple cavitating nodules, ground glass opacities and dense right middle lobe consolidations and right-sided pneumothorax. Bronchoalveolar lavage (BAL) showed septate hyaline hyphae on KOH (potassium hydroxide) staining and fungal culture grew Aspergillus fumigatus. BAL and serum galactomanan were positive and serum IgG for A. fumigatus was 58 MgA/L (0–40MgA/L) confirming the cause of spontaneous secondary pneumothorax in our patient as CCPA.

Growth rate and site of pulmonary metastasis to predict lung relapse and overall survival in patients affected by bone and soft tissue sarcomas (B-STS).

11571 Background: Despite surgically resectable pulmonary metastases may lead to cure patients with B-STS (Chudgar NP 2017), a substantial proportion of patients will eventually relapse. Presently, patient selection is based on unique organ involvement, number of metastases, interval between previous surgery and pulmonary progression or relapse. We assessed the impact of anatomical site of metastasis into the lung (as if the pleural site might ease further tumor spreading) and nodule growth rate as additional predictive/prognostic factors of lung progression-free survival (L-PFS) and overall survival (OS). Methods: In our prospectively collected database, we retrospectively evaluated patients operated for B-STS pulmonary progression at 3 different centers from 2005 to 2019. Beyond patients’ clinical features at both baseline and disease progression in the lungs, we focused on whether the relapse occurred into the parenchyma or nearby the pleura (Welter S 2012); secondly, we estimated lung metastasis growth rate, defined as tumor doubling time (TDT) (Nakamura T 2011). Statistical analyses were carried out with IBM SPSS (v. 20.0). Survival outcomes were estimated by Kaplan-Meier method. Hazard ratios (HR) were estimated by Cox regression. Multivariate analysis was performed for both L-PFS and OS according to Cox proportional hazard model. All tests were 2-sided with their corresponding 95% confidence intervals (CI95%). Results: We identified 138 patients who underwent lung metastasectomy [(F=66 (48%); median age at surgery 50 (14-78)]. Median PFS and L-PFS were 8.7 months (CI95% 6.6-10.9) and 8.6 months (CI95% 6.2-11.0), respectively. Median OS was 40.6 months (CI 95% 32.8-48.5). Univariate analysis showed a statistically significant impact of the following variables for both L-PFS and OS: ECOG 0, nodule number <3, being disease-free after first-line treatment, no pleural involvement, and TDT >40 days. Disease-free interval ≤ 24 months and absence of metastases at diagnosis showed significant correlation with L-PFS and OS, respectively. At multivariate analyses the following variables retained statistical significance for L-PFS: TDT >40 days (HR 0.53, CI95% 0.31-0.93, p=0.028); nodule number <3 (HR 0.54, 95%CI 0.29-0.99, p=0.048), no pleural involvement (HR 0.39, CI95% 0.22-0.70, p=0.001); and for OS: TDT >40 days (HR 0.36, CI95% 0.18-0.72, p=0.004), nodule number <3 (HR 0.35, 95%CI 0.18-0.71, p=0.004), no pleural involvement (HR 0.49, CI95% 0.24-0.98, p=0.045), and ECOG 0 (HR 0.29, 95%CI 0.14-0.59, p=0.001). Conclusions: Acknowledging its retrospective nature and the need for an external validation, our series highlights the key-role of the anatomical site of relapse within the lung and the impact of tumor growth rate. If confirmed, these two clinical parameters should be factored in the decision making on performing pulmonary metastasectomy.

Pleural Involvement in Pulmonary Sarcoidosis: A Case Report and Review of the Literature

Background: Sarcoidosis is a chronic, multisystemic granulomatosis of unknown origin that can affect multiple organs throughout the body with variable progression and prognosis. Although the lungs and mediastinal lymph nodes are almost always affected in sarcoidosis, involvement of the pleura remains uncommon.Case presentation: A 69-year-old female presented with dry cough, dyspnea on exertion and fatigue. She was diagnosed with pleural sarcoidosis based on the histological examination of the pleural biopsy and was treated with oral prednisone. Her complaints regressed two weeks later and the left pleural effusion almost disappeared four weeks later.Conclusions: Pleural involvements in sarcoidosis should be considered in the differential diagnosis of pleural effusion and pleural nodules.

Pulmonary Lymphangitic Spread of Multiple Myeloma as Early Relapse after Autologous Stem Cell Transplantation

Clinical relapses early after autologous stem cell transplantation portrays an inferior clinical outcome. Early relapse in this setting with extramedullary disease (EMD) of lung involvement in multiple myeloma is rare. To our knowledge, this is the first reported case of lymphangitic spread of myeloma with pulmonary parenchymal and pleural involvement occurring at first relapse.

Novel Use of Bromelain and Acetylcysteine (BromAc®) for Pleural Involvement in Pseudomyxoma Peritonei

Pseudomyxoma peritonei (PMP) is a rare mucinous disease most commonly arising from the appendix. Pleural involvement arising from established PMP is seen in a small number of cases. Combined cytoreductive surgery and hyperthermic intrathoracic chemotherapy is the treatment of choice when managing intra-thoracic PMP. In cases of recurrence, surgical intervention may be technically challenging and carry higher rates of complications, morbidity, and mortality. Bromelain and acetylcysteine (BromAc^®) is a novel treatment modality that has demonstrated mucolytic properties. When injected directly into mucinous disease, it facilitates tumour dissolution and allows it to be aspirated. It has recently been tested in the treatment of inoperable peritoneal mucinous disease, with an acceptable safety profile and positive objective response. Here we describe the first two cases of BromAc^® administered directly into pleural adenomucinosis, with striking differences in response between the two patients likely due to differences in tumour hardness.

Secondary spontaneous pneumothorax in a patient of allergic bronchopulmonary aspergillosis: an unusual presentation

Allergic bronchopulmonary aspergillosis (ABPA) is a complex immunologic pulmonary disorder caused by hypersensitivity to fungus, Aspergillus fumigates. It clinically manifests with non-specific respiratory and systemic symptoms. ABPA is typically seen in patients with long-standing asthma or cystic fibrosis. Pleural involvement in ABPA is uncommon and secondary spontaneous pneumothorax is very rare. Herein, we report a case of 33 years old male patient presented with dyspnoea, low grade fever and productive cough. High Resolution Computed tomography (HRCT) scan of thorax was suggestive of ABPA with secondary pneumothorax.