Pulmonary manifestations and outcomes in paediatric ANCA-associated vasculitis: a single-centre experience.

OBJECTIVES: ANCA-associated vasculitis (AAV) usually involves the renal and respiratory systems, but the paediatric literature on pulmonary manifestations and outcomes is limited. We aimed to describe pulmonary manifestations and outcomes after therapy in a cohort of paediatric AAV (pAAV) patients. METHODS: A retrospective chart review of all patients <19 years presenting to our institution with AAV between 1/2008 and 2/2018 was conducted. Patient demographics, clinical presentation, diagnostic testing, therapy and pulmonary outcomes over the first 3 years after presentation were evaluated. RESULTS: A total of 38 patients were included; all had ANCA positivity by immunofluorescence. A total of 23 had microscopic polyangiitis (MPA), 13 had granulomatosis with polyangiitis and 2 had eosinophilic granulomatosis with polyangiitis. A total of 30 (79%) had pulmonary manifestations, with cough (73%) and pulmonary haemorrhage (67%) being the most common. Abnormalities were noted in 82% of chest CT scans reviewed, with nodules and ground-glass opacities being the most common. At 6, 12 and 36 months follow-up, respectively, 61.8%, 39.4% and 29% of patients continued to show pulmonary manifestations. Five MPA patients with re-haemorrhage are described in detail. CONCLUSION: MPA was more common than granulomatosis with polyangiitis, with pulmonary involvement being common in both. MPA patients had more severe pulmonary manifestations. Chest CT revealed abnormal findings in a majority of cases. A subgroup of young MPA patients experienced repeat pulmonary haemorrhage. Treatment modality and response were comparable in different subtypes of AAV, except for this young MPA group. Additional prospective studies are needed to better understand the different phenotypes of pAAV.

External validation of a convolutional neural network artificial intelligence tool to predict malignancy in pulmonary nodules.

BACKGROUND: Estimation of the risk of malignancy in pulmonary nodules detected by CT is central in clinical management. The use of artificial intelligence (AI) offers an opportunity to improve risk prediction. Here we compare the performance of an AI algorithm, the lung cancer prediction convolutional neural network (LCP-CNN), with that of the Brock University model, recommended in UK guidelines. METHODS: A dataset of incidentally detected pulmonary nodules measuring 5-15 mm was collected retrospectively from three UK hospitals for use in a validation study. Ground truth diagnosis for each nodule was based on histology (required for any cancer), resolution, stability or (for pulmonary lymph nodes only) expert opinion. There were 1397 nodules in 1187 patients, of which 234 nodules in 229 (19.3%) patients were cancer. Model discrimination and performance statistics at predefined score thresholds were compared between the Brock model and the LCP-CNN. RESULTS: The area under the curve for LCP-CNN was 89.6% (95% CI 87.6 to 91.5), compared with 86.8% (95% CI 84.3 to 89.1) for the Brock model (p≤0.005). Using the LCP-CNN, we found that 24.5% of nodules scored below the lowest cancer nodule score, compared with 10.9% using the Brock score. Using the predefined thresholds, we found that the LCP-CNN gave one false negative (0.4% of cancers), whereas the Brock model gave six (2.5%), while specificity statistics were similar between the two models. CONCLUSION: The LCP-CNN score has better discrimination and allows a larger proportion of benign nodules to be identified without missing cancers than the Brock model. This has the potential to substantially reduce the proportion of surveillance CT scans required and thus save significant resources.

Management of Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia: Review and a Single Center Experience.

BACKGROUND: Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare pulmonary condition, characterized by diffuse proliferation of neuroendocrine cells in the respiratory epithelium. DIPNECH lesions are less than 5 mm in size and are limited to the basement membrane with no invasion. There is limited information regarding epidemiology, natural history of disease progression, or the management of this rare entity. We present the experience of a center with extensive expertise in neuroendocrine disease. METHODS: A cohort of patients (N = 13) with DIPNECH treated and followed at our institution was identified. We describe the our approach to their care, our disease management and also provide a review of DIPNECH pathophysiology. RESULTS: Our patient cohort consisted of twelve females and one male with a mean age of 63 years at the time of diagnosis. Dyspnea on exertion and dry cough were the most common presenting symptoms. Two patients were under surveillance without treatment; three patients were treated with a short-acting somatostatin analog; three patients were treated with azithromycin alone; four were treated with a combination of long-acting monthly somatostatin analogs and azithromycin; one patient received a combination of long-acting somatostatin analog and everolimus. Five patients had concomitant bronchial carcinoids. CONCLUSIONS: DIPNECH is a rare pathology that can profoundly affect a patient's quality of life. Paroxysmal coughing episodes can be difficult to treat. Our limited single center experience shows encouraging response to use of somatostatin analogs, azithromycin, and everolimus in the management of debilitating DIPNECH associated symptoms.

Benign metastasizing leiomyoma presenting as multiple cystic pulmonary nodules: a case report.

BACKGROUND: Benign metastatic leiomyoma (BML) is an extremely rare disease. Although uterine leiomyomas are benign histologically, they can metastasize to distant sites. While the incidence is very low, the lung is the organ most frequently affected by BML. Pulmonary BML usually presents as numerous well-defined nodules of various sizes, while the cavitary or cystic features in the nodules are rarely observed on radiologic images. CASE PRESENTATION: A 52-year-old woman complained of cough and dyspnea for one month. She had been previously diagnosed with uterine leiomyoma and had undergone total hysterectomy about 14 years prior. High-resolution computed tomography (CT) images showed that there were multiple cystic nodules of various sizes in both lungs. Pathologic examination revealed that the pulmonary nodule had complex branching glandular structures lined by a single layer of simple cuboidal to columnar epithelium that was surrounded by abundant spindle cells. Additional immunohistochemistry data suggested that pulmonary nodule diagnosis was BML-associated uterine leiomyoma. CONCLUSION: In this report, we introduce an interesting case of pulmonary BML that presented as a combination of various kinds of nodules including simple round nodules, simple cysts, and cysts with a solid portion, which are very rare radiologic features of BML in lung. In addition, when the patient is a woman of reproductive age, physicians should meticulously review the gynecological history and suspect BML when there are various cystic pulmonary lesions.

Surgical Management of Multifocal Ground-Glass Opacities of the Lung: Correlation of Clinicopathologic and Radiologic Findings.

Background We evaluated the clinicopathologic characteristics and oncologic outcome in patients who underwent surgical resection for multifocal ground-glass opacities (GGOs) of the lung. Methods We examined 131 patients who underwent surgical resections for multiple clinical-N0 lung cancers. Multifocal GGOs were defined as tumors showing GGO dominance with a consolidation/tumor ratio (CTR) < 0.5 for all, whereas solid lesions were defined as having at least one tumor that showed CTR ≥0.5. Results Multifocal GGOs were found in 53 (40.5%) patients. A significantly large number of GGOs with a median of 3 per patient (range 2-41) was observed in multifocal GGOs (p < 0.0001). A multivariate analysis demonstrated tumor size ≤ 20 mm (p = 0.0407) and multifocal GGOs (p = 0.0345) were significantly associated with the survival. Regarding surgical managements for multifocal GGOs, the 5-year overall survival (OS) of multiple synchronous or staged limited resection only (n = 26) versus anatomical resection with or without additional limited resection (n = 27) was not significantly different (100% and 91.9%, p = 0.2287). The total number of resected multifocal GGOs was 278, most of which revealed adenocarcinoma or precancerous lesions. Unresected or new GGOs developed in 19 (35.8%) patients, all of which remained pure-GGO of < 10 mm in size without any interventions. The 5-year OS of multifocal GGOs and solid lesions were 94.4% and 80.6% (p = 0.0096), with a median follow-up time of 60 months. Conclusion Surgical interventions combined with limited surgery or adequate follow-up management based on the findings on thin-section CT could provide acceptable oncologic outcomes for multifocal GGOs.

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia syndrome.

The term diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) may be used to describe a clinico-pathological syndrome, as well as an incidental finding on histological examination, although there are obvious differences between these two scenarios. According to the World Health Organization, the definition of DIPNECH is purely histological. However, DIPNECH encompasses symptomatic patients with airway disease, as well as asymptomatic patients with neuroendocrine cell hyperplasia associated with multiple tumourlets/carcinoid tumours. DIPNECH is also considered a pre-neoplastic lesion in the spectrum of pulmonary neuroendocrine tumours, because it is commonly found in patients with peripheral carcinoid tumours.In this review, we summarise clinical, physiological, radiological and histological features of DIPNECH and critically discuss recently proposed diagnostic criteria. In addition, we propose that the term "DIPNECH syndrome" be used to indicate a sufficiently distinct patient subgroup characterised by respiratory symptoms, airflow obstruction, mosaic attenuation with air trapping on chest imaging and constrictive obliterative bronchiolitis, often with nodular proliferation of neuroendocrine cells with/without tumourlets/carcinoid tumours on histology. Surgical lung biopsy is the diagnostic gold standard. However, in the appropriate clinical and radiological setting, transbronchial lung biopsy may also allow a confident diagnosis of DIPNECH syndrome.

Lung nodule and functional changes in smokers after smoking cessation short-term treatment.

BACKGROUND: Tobacco smoke causes lung disfunction and inflammation. METHODS: Twenty-two consecutive smokers with undetermined lung nodules were included. All underwent a baseline imaging, exhaled carbon monoxide level evaluation and spirometry, repeated at three months from smoking cessation therapy. RESULTS: A significant reduction in size of the lung nodules was reported (p = .037) as a trend in number reduction (p = .11). A significant increase in blood arterial oxygen pressure (p < .0001), heart rate reduction (p < .01), and FEV1 increase (p < .0001) was recorded. CONCLUSIONS: Smoking cessation reduces number and size of lung nodules and improves in lung functionality.

Diagnosis of pulmonary nodules by DNA methylation analysis in bronchoalveolar lavage fluids.

BACKGROUND: Lung cancer is the leading cause of cancer-related mortality. The alteration of DNA methylation plays a major role in the development of lung cancer. Methylation biomarkers become a possible method for lung cancer diagnosis. RESULTS: We identified eleven lung cancer-specific methylation markers (CDO1, GSHR, HOXA11, HOXB4-1, HOXB4-2, HOXB4-3, HOXB4-4, LHX9, MIR196A1, PTGER4-1, and PTGER4-2), which could differentiate benign and malignant pulmonary nodules. The methylation levels of these markers are significantly higher in malignant tissues. In bronchoalveolar lavage fluid (BALF) samples, the methylation signals maintain the same differential trend as in tissues. An optimal 5-marker model for pulmonary nodule diagnosis (malignant vs. benign) was developed from all possible combinations of the eleven markers. In the test set (57 tissue and 71 BALF samples), the area under curve (AUC) value achieves 0.93, and the overall sensitivity is 82% at the specificity of 91%. In an independent validation set (111 BALF samples), the AUC is 0.82 with a specificity of 82% and a sensitivity of 70%. CONCLUSIONS: This model can differentiate pulmonary adenocarcinoma and squamous carcinoma from benign diseases, especially for infection, inflammation, and tuberculosis. The model's performance is not affected by gender, age, smoking history, or the solid components of nodules.

A novel application of pulmonary transit time to differentiate between benign and malignant pulmonary nodules using myocardial contrast echocardiography.

Malignant pulmonary nodules (PNs) are often accompanied by vascular dilatation and structural abnormalities. Pulmonary transit time (PTT) measurement by contrast echocardiograghy has used to assess the cardiopulmonary function and pulmonary vascular status, such as hepatopulmonary syndrome and pulmonary arteriovenous fistula, but has not yet been attempted in the diagnosis and differential diagnosis of PNs. The aim of this work was to evaluate the feasibility and performance of myocardial contrast echocardiography (MCE) for differentiating malignant PNs from benign ones. The study population consisted of 201 participant: 66 healthy participants, 65 patients with benign PNs and 70 patients with malignant PNs. Their clinical and conventional echocardiographic characteristics were collected. MCE with measurements of PTT were performed. There was no difference in age, sex, heart rate, blood pressure, smoking rate, background lung disease, pulmonary function, ECG, myocardial enzymes, cardiac size and function among the healthy participant, patients with benign and malignant PNs (P > 0.05). PTT did not differ significantly in patients with PNs of different sizes, nor did they differ in patients with PNs of different enhancement patterns (P > 0.05). However, the PTT were far shorter (about one half) in patients with malignant PNs than in patients with benign ones (1.88 ± 0.37 vs. 3.73 ± 0.35, P < 0.001). There was no significantly different between patients with benign PNs and healthy participant (3.73 ± 0.35 vs.3.89 ± 0.36, P > 0.05). The area under the receiver operating characteristics curve (AUC) of PTT was 0.99(0.978-1.009) in discriminating between benign and malignant PNs. The optimal cutoff value was 2.78 s, with a sensitivity of 98.52%, a specificity of 97.34%, and a accuracy of 97.69%. MCE had a powerful performance in differentiating between benign and malignant PNs, and a pulmonary circulation time of < 2.78 s indicated malignant PNs.

Pulmonary Manifestations of GATA2 Deficiency.

BACKGROUND: GATA2 deficiency is a genetic disorder of hematopoiesis, lymphatics, and immunity caused by autosomal dominant or sporadic mutations in GATA2. The disease has a broad phenotype encompassing immunodeficiency, myelodysplasia, leukemia, and vascular or lymphatic dysfunction as well as prominent pulmonary manifestations. RESEARCH QUESTION: What are the pulmonary manifestations of GATA2 deficiency? STUDY DESIGN AND METHODS: A retrospective review was conducted of clinical medical records, diagnostic imaging, pulmonary pathologic specimens, and tests of pulmonary function. RESULTS: Of 124 patients (95 probands and 29 ascertained), the lung was affected in 56%. In addition to chronic infections, pulmonary alveolar proteinosis (11 probands) and pulmonary arterial hypertension (nine probands) were present. Thoracic CT imaging found small nodules in 54% (54 probands and 12 relatives), reticular infiltrates in 40% (45 probands and four relatives), paraseptal emphysema in 25% (30 probands and one relative), ground-glass opacities in 35% (41 probands and two relatives), consolidation in 21% (23 probands and two relatives), and a typical crazy-paving pattern in 7% (eight probands and no relatives). Nontuberculous mycobacteria were the most frequent organisms associated with chronic infection. Allogeneic hematopoietic stem cell transplantation successfully reversed myelodysplasia and immune deficiency and also improved pulmonary hypertension and pulmonary alveolar proteinosis in most patients. INTERPRETATION: GATA2 deficiency has prominent pulmonary manifestations. These clinical observations confirm the essential role of hematopoietic cells in many aspects of pulmonary function, including infections, alveolar proteinosis, and pulmonary hypertension, many of which precede the formal diagnosis, and many of which respond to stem cell transplantation.

A 58-Year-Old Woman With a History of Cardiac Myxoma Presents With Pulmonary Nodules.

CASE PRESENTATION: A 58-year-old woman presented to a pulmonology clinic for evaluation of bilateral pulmonary nodules. Two years previously, she had presented with atrioventricular nodal reentrant tachycardia. During evaluation for her tachyarrhythmia, transthoracic echocardiogram (TTE) revealed a large, homogenous, highly mobile right atrial and ventricular mass. She underwent electrophysiologic ablation, tricuspid valve annular ring replacement, and resection of the mass, which pathology confirmed to be a myxoma. Now, a recent abdomen and pelvis CT study obtained for history of nephrolithiasis incidentally noted bilateral lower lobe pulmonary nodules. Follow-up noncontrast chest CT confirmed bilateral peribronchovascular solid pulmonary nodules up to 8 mm in diameter throughout all lobes. The nodules appeared contiguous with the segmental and subsegmental bronchovascular bundles, and many occurred at branch points. There was no mediastinal or hilar lymphadenopathy. To evaluate the pulmonary nodules, she was referred to a pulmonology clinic. She reported only stable, nonlimiting dyspnea on exertion. She did not have a cough. She denied a history of fevers, weight loss, or night sweats. She had no rash or skin changes, visual changes, joint pain or swelling, or palpitations. She had no history of oropharyngeal or genital ulcerations. Social history was notable for a 40-pack-year smoking history, with quit date 2 years prior. She had no risk factors for TB exposure and no exposures to sandblasting, stone cutting, or other environmental risk factors for silicosis. Family history was negative for autoimmune conditions, sarcoidosis, and lymphoproliferative disorders.

A 58-Year-Old Woman With Lung Nodules and Chronic Cough.

CASE PRESENTATION: A 58-year-old woman was referred to our department with a cough of 1 year duration; her condition was unresponsive to the administration of inhaled steroid and beta-2 agonists. She denied the presence of dyspnea, chest pain, or other extrapulmonary symptoms. She was a never-smoker with a negative medical history and no occupational or domestic exposures. There was no history of cancer, gastroesophageal reflux disease, asthma, allergic rhinitis, or other allergies.

Recurrent Henoch-Schönlein Purpura with bullous rash and pulmonary nodules.

BACKGROUND: Henoch-Schönlein purpura (HSP) is the most common vasculitis of childhood. It has a characteristic rash described as palpable purpura that most frequently affects the distal lower extremities and buttocks. HSP rarely presents with bullous rash nor pulmonary nodules. CASE PRESENTATION: We present a novel case of a 12-years-old female with recurrent pediatric HSP with a combination of the rare manifestations of bullous rash and pulmonary nodules. She initially presented with the bullous rash, chest pain, cough, and abdominal pain. Patient was successfully treated with intravenous pulse corticosteroids followed by a high dose oral corticosteroid taper, with resolution of the bullous rash and pulmonary nodules. CONCLUSION: The rare manifestations of scarring bullous rash and pulmonary nodules can be presenting features of pediatric HSP, the combination of which has not been previously reported. The treatment of intravenous corticosteroid resolved patient's abdominal symptoms, rash and pulmonary nodules.

A 52-Year-Old Woman With an Abdominal Mass, Bilateral Pulmonary Nodules, and Mediastinal and Hilar Lymphadenopathy.

CASE PRESENTATION: A 52-year-old, nonsmoking, African-American woman with a history of obesity, hypertension, and rheumatoid arthritis was referred for workup of multiple bilateral pulmonary nodules. The pulmonary nodules were discovered incidentally while undergoing a CT scan for an abdominal mass that was radiographically diagnosed as a uterine leiomyoma. She was asymptomatic from a pulmonary standpoint without unintentional weight loss, fevers, or night sweats. Her mother and sister had a history of lung cancer. She was diagnosed with rheumatoid arthritis 5 years earlier that was controlled with adalimumab for approximately 3 years when she stopped being seen by her rheumatologist and discontinued adalimumab. During evaluation for the abdominal mass, she re-established care with a rheumatologist and was started on 40 mg prednisone daily with plans to restart adalimumab once the workup for the abdominal mass and pulmonary nodules was completed. She had undergone bariatric surgery with cholecystectomy approximately 5 years earlier, after which she experienced intentional postsurgical weight loss.

Three-dimensional navigation-guided thoracoscopic combined subsegmentectomy for intersegmental pulmonary nodules.

BACKGROUND: Extended or combined segmentectomies are usually adapted for intersegmental pulmonary nodules. This study explored precise combined subsegmentectomy (CSS) under the guidance of three-dimensional computed tomography bronchography and angiography (3D-CTBA). METHODS: The definition of a pulmonary intersegmental nodule was based on a minimum distance between the nodule and the involved intersegmental veins in the preoperative 3D-CTBA being less than the size of the nodule. Centering on the involved intersegmental vein, two adjacent subsegments belonging to the different segments were combined as a resected unit. RESULTS: We retrospectively reviewed the records of 47 patients (mean age 53.6 ± 12.3, range: 26-81 years) who underwent CSS. Thirty-nine (83.0%) nodules were involved in most intersegmental locations of the upper lobes; the remainder in the lower lobes. The mean nodule size was 0.86 ± 0.32 cm; the mean margin width was 2.20 ± 0.38 cm. Pathological stages included: Tis (8 cases), T1mi (16), IA1 (T1aN0M0, 13), and IA2 (T1bN0M0, 5). Pathological diagnoses included: invasive adenocarcinoma (18 cases), minimally invasive adenocarcinoma (16), adenocarcinoma in situ (8), atypical adenomatous hyperplasia (3), and benign (2). The average operative duration was 190.8 ± 54.9 minutes; operative hemorrhage was 42.7 ± 23.0 mL; 5.8 ± 2.8 lymph nodes dissected had not metastasized; the duration of postoperative chest tube drainage was 3.0 ± 1.8 days; and the postoperative hospital stay was 5.3 ± 2.4 days. CONCLUSIONS: Under 3D navigation, thoracoscopic CSS is a safe technique for intersegmental nodules, sparing more pulmonary parenchyma and ensuring safe margins to achieve anatomical resection.

Effect of tomographic operator inaccuracies and respiratory motion on PET/CT lung nodule images smearing.

BACKGROUND: In thoracic PET/computed tomography (CT) imaging, uptake foci usually appear smeared because of postreconstruction smoothing and respiratory motion. OBJECTIVE: The aim of the present study was to assess the respective contributions of the reconstruction process and respiratory motion on PET/CT images. MATERIALS AND METHODS: Thirty-one pulmonary lesions were studied. Free-breathing PET/CT acquisitions were followed by a 10-min respiratory-gated PET/CT acquisition. Four different reconstructions were performed by combining two different tomographic operators (TOs) (i.e. the geometric clinical system matrix or a system matrix including the detector response) and taking account (or not) of respiratory motion using a previously developed 'CT-based' technique. For each reconstruction method, lesion segmentation was performed with an adaptive threshold. Next, we computed the volume differences between each reconstruction. Finally, we applied a multiple linear model to compute the relative contributions of TO-based and CT-based respiratory compensation to lesion volume. RESULTS: The three groups, combining the reconstruction methods and the respiratory compensation (or not), differed significantly in terms of the volume differences. For all lesions, the full linear model yielded a regression coefficient R of 76.10%. The partial R values were 65.58 and 10.52% for the detector response operator and the CT-based method, respectively. For lesions in the upper/middle lobes, blurring was mainly because of TO (partial R=78.53%), whereas, for lower lobe lesions, smearing was mainly because of respiratory motion (partial R=56.76%). CONCLUSION: Our results showed that image reconstruction, by TO accuracy, was the main explanatory factor for lesion smearing when considering the chest as a whole. Respiration had a major impact on the lower lobes.

Thoracoscopic pulmonary wedge resection without post-operative chest drain: an observational study.

OBJECTIVE: Chest drains are used routinely after wedge resection by video-assisted thoracoscopic surgery (VATS), although this practice is based largely on tradition rather than evidence. Chest drains may furthermore cause pain, infections, and prolonged length of stay. The aim of this prospective observational study was to assess the feasibility of avoiding chest drains following VATS wedge resection for pulmonary nodules. METHODS: Between 1 February and 25 August 2015 166 consecutive patients planned for VATS wedge resection of pulmonary nodules were screened for inclusion using the following criteria: Forced expiratory volume in 1 s (FEV1) ≥60 % of expected, FEV1/forced vital capacity ≥70 %, tumour diameter ≤2 cm, distance from tumour to visceral pleura ≤3 cm, ≤2 separate wedges, no air leak on an intraoperative air leakage test and absence of severe adhesions, bullous/emphysematous disease, pleural effusion and coagulopathy. Chest X-rays were done twice on the day of surgery. 30-day complications were compiled from patient records. RESULTS: 49 patients underwent 51 unilateral VATS wedge resections without using a post-operative chest drain. No patient required reinsertion of a chest drain. 30 (59 %) patients had a pneumothorax of mean size 12 ± 12 mm on supine 8-h post-operative X-ray for which the majority resolved spontaneously within 2-week control. There were no complications on 30-day follow-up. Median length of stay was 1 day. CONCLUSIONS: The results support that VATS wedge resection for pulmonary nodules without a post-operative chest drain may be safe in a selected group of patients.

Ten Years of Chronic Cough in a 64-Year-Old Man With Multiple Pulmonary Nodules.

A 64-year-old male former smoker with a history of prostate cancer presented to our pulmonary clinic, complaining of nonproductive cough for 10 years. Prior evaluation included treatment for upper airway cough syndrome and gastroesophageal reflux, stopping angiotensin-converting enzyme inhibitor, and initiation of inhaled ß-agonists. Esophageal pH monitoring indicated silent reflux, and proton pump inhibitor therapy was started. He continued to cough and complain of dyspnea. Physical examination produced unremarkable results, with no evidence of lymphadenopathy. Pulmonary function tests showed a pseudo-restrictive pattern with air trapping, hyperreactivity, and incomplete bronchodilator responsiveness: FEV1, 2.48 L (69% of predicted); FVC, 3.57 L (75% of predicted); FEV1/FVC, 92%; total lung capacity, 7.00 L (100% of predicted); and residual volume, 3.05 L (136% of predicted). Laboratory studies, including a complete metabolic panel, prostate-specific antigen test, and complete blood count, yielded normal results.

Amyloid-associated Cystic Lung Disease.

BACKGROUND: Amyloid-associated cystic lung disease is rare. It can be associated with collagen vascular disease (CVD). We aimed to describe the clinical, radiology, and pathology findings of this entity. METHODS: We reviewed the records of subjects having biopsy-proven pulmonary amyloidosis with cystic lung disease demonstrated at high-resolution computed tomography (HRCT). Demographic characteristics, association with CVD and lymphoproliferative disorders, pulmonary function, and pathology results were reviewed. HRCT appearance was analyzed for number, size, distribution, and morphology of cysts and nodules. RESULTS: Twenty-one subjects (13 female, eight male; median age, 61 years) with cystic pulmonary amyloidosis were identified. The most common pulmonary function patterns were normal (42%) and obstructive (32%). The most common associated CVD was Sjögren syndrome (10 of 12). Nine subjects had no CVD. Cysts tended to be multiple (≥ 10 in 14 of 21, 67%), round (21 of 21, 100%), or lobulated (20 of 21, 95%); thin-walled (< 2 mm in 17 of 21, 81%); and of small (< 1 cm in 21 of 21, 100%) to moderate (1-2 cm in 17 of 21, 81%) size. Peribronchovascular (19 of 21, 90%) and subpleural (19 of 21, 90%) cysts were typically present. Seventeen (81%) subjects had lung nodules, which tended to be numerous (≥ 10 in 10 of 17, 59%; 4-9 in six of 17, 35%). At least one calcified nodule was present in 14 of 17 subjects (82%). Pulmonary mucosa-associated lymphoid tissue lymphoma (MALToma) was diagnosed in seven subjects (33%). CONCLUSIONS: Amyloid-associated cystic lung disease can occur with or without underlying CVD. Cystic lesions in the lung are commonly numerous, often are peribronchovascular or subpleural, and are frequently associated with nodular lesions that are often calcified. MALToma was a relatively frequent association.