A Unique Case of Combined Nodular and Tracheobronchial Amyloidosis.

Amyloidosis is a heterogeneous group of diseases characterized by the extracellular deposition of misfolded proteins that can affect either systemically or locally confined to one system. Pulmonary amyloidosis is rare and can be classified into three forms according to the anatomic site of involvement: nodular pulmonary amyloidosis, tracheobronchial amyloidosis and diffuse alveolar-septal amyloidosis. The former two usually represent localized amyloid disease and the latter represents systemic disease.

A case of hypereosinophilic syndrome with STAT5b N642H mutation.

Hypereosinophilia is defined as persistent eosinophilia (>1.5 × 10/L). Hypereosinophilic syndrome (HES) is a term used to describe a group of disorders characterized by sustained hypereosinophilia associated with end-organ damage.

Vaping-related pulmonary granulomatous disease.

The use of electronic cigarettes, or "vaping," has garnered significant popularity and attention in recent years. Its pulmonary and systemic effects have yet to be fully studied and quantified, and recent reports of vaping-related illnesses and deaths have brought the clinical consequences of vaping into the public spotlight. This report describes the case of a 34 year old woman who presented to clinic with new-onset cough and dyspnea, shortly after beginning to use electronic cigarettes.

e-Cigarette or Vaping Product Use-Associated Lung Injury: Clinical, Radiologic, and Pathologic Findings of 15 Cases.

Since mid-2019, > 2,000 cases of e-cigarette or vaping product use-associated lung injury (EVALI) have been reported. Although initial reports suggested that this entity may be a form of inhalation-related lipoid pneumonia, subsequent studies indicate that EVALI represents various patterns of acute lung injury. Cases of EVALI continue to be reported, and public awareness of the epidemic is increasingly high.

Mysterious quad of constrictive pericarditis, recurrent pleural effusions, bone involvement and interstitial lung disease.

Erdheim-Chester disease (ECD) is a rare multisystemic non-Langerhans cell histiocytic neoplasm. The rarity of the disease and heterogenous clinical presentations often leads to delayed diagnosis. Historically, ECD lacked effective treatment and the prognosis was poor.

A unique case of ectopic Cushing's syndrome from a thymic neuroendocrine carcinoma.

Ectopic adrenocorticotropic hormone (ACTH) production leading to ectopic ACTH syndrome accounts for a small proportion of all Cushing's syndrome (CS) cases. Thymic neuroendocrine tumors are rare neoplasms that may secrete ACTH leading to rapid development of hypercortisolism causing electrolyte and metabolic abnormalities, uncontrolled hypertension and an increased risk for opportunistic infections. We present a unique case of a patient who presented with a mediastinal mass, revealed to be an ACTH-secreting thymic neuroendocrine tumor (NET) causing ectopic CS.

Banff study of pathologic changes in lung allograft biopsy specimens with donor-specific antibodies.

Background: The diagnosis of antibody-mediated rejection (AMR) in the lung transplant is still an area under investigation. We performed a blinded multicenter study to determine if any statistically significant histologic findings in transbronchial biopsy specimens from lung transplant patients correlate with the presence of donor-specific antibodies (DSAs).

Methods: We asked 9 pathologists with experience in lung transplantation to evaluate 161 lung transplant biopsy specimens for various histologic parameters.

Tracheobronchial smooth muscle atrophy and separation.

We report a case series involving 4 patients with chronic obstructive pulmonary disease who were on an appropriate medical regimen including a high dose of inhaled corticosteroids (ICS). During bronchoscopy, patients were found to have an excessive dynamic collapse of the posterior wall and its separation from the ends of the adjacent cartilaginous rings. This was causing a near-total occlusion of the tracheal and bronchial lumen during exhalation, thereby presenting with an obstructive pattern on the pulmonary functions.

A retrospective review of clinical features and treatment outcomes in steroid-resistant interstitial lung disease from polymyositis/dermatomyositis.

Introduction: We reviewed our experience with immunosuppressive agents in patients with steroid-resistant Interstitial Lung Disease in the setting of Polymyositis/Dermatomyositis (PM/DM-ILD) to determine whether there were major differences in outcomes.

Methods: We identified all patients treated for PM/DM-ILD and assessed cyclophosphamide (CYC), azathioprine (AZA) and mycophenolate (MMF) when used as first-line steroid sparing therapy for effects on pulmonary function variables, dyspnea and tolerance at six and twelve months.

Results: Among 46 patients meeting the inclusion criteria, 24 were treated with CYC, 13 with AZA and 9 with MMF.

ALK status testing in non-small cell lung carcinoma: correlation between ultrasensitive IHC and FISH.

ALK gene rearrangements in advanced non-small cell lung carcinomas (NSCLC) are an indication for targeted therapy with crizotinib. Fluorescence in situ hybridization (FISH) using a recently approved companion in vitro diagnostic class FISH system commonly assesses ALK status. More accessible IHC is challenged by low expression of ALK-fusion transcripts in NSCLC.