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In a patient with interstitial lung disease (ILD) of known or unknown etiology other than idiopathic pulmonary fibrosis (IPF), progressive pulmonary fibrosis (PPF) is defined by worsening lung fibrosis on high-resolution computed tomography (HRCT), decline in lung function, and/or deterioration in symptoms. The INBUILD trial involved 663 patients with PPF who were randomized to receive nintedanib or placebo. The median exposure to trial medication was approximately 19 months. The INBUILD trial provided valuable learnings about the course of PPF and the efficacy and safety of nintedanib. The relative effect of nintedanib on reducing the rate of forced vital capacity decline was consistent across subgroups based on ILD diagnosis, HRCT pattern, and disease severity at baseline, and between patients who were and were not taking glucocorticoids or disease-modifying anti-rheumatic drugs and/or glucocorticoids at baseline. The adverse events most frequently associated with nintedanib were gastrointestinal, particularly diarrhea, but nintedanib was discontinued in only a minority of cases. The results of the INBUILD trial highlight the importance of prompt detection and treatment of PPF and the utility of nintedanib as a treatment option.
What did we find out from the INBUILD trial about progressive lung fibrosis?Lung fibrosis is a rare disease in which the lung tissue becomes scarred and hardened. This makes it more difficult for the lungs to inflate and for the lungs to exchange oxygen with the blood. In some patients, lung fibrosis gets worse over time. This is known as progressive lung fibrosis. In the INBUILD trial, researchers looked at the effects of a drug called nintedanib in patients with progressive lung fibrosis. In this trial, 663 patients were randomly allocated to receive either nintedanib or a placebo and then followed for approximately 19 months. The patients and the researchers did not know which patients were taking the active drug (nintedanib) and which patients were taking placebo. The results showed that the criteria used to find patients with progressive lung fibrosis to take part in the trial successfully identified patients whose disease would continue to worsen. These criteria were based on a decline in the volume (size) of the lungs, worsening symptoms such as shortness of breath, and worsening of changes seen on a scan of the chest. The trial results also showed that nintedanib slowed down loss of lung function and had a similar benefit in patients with different severities of disease at the start of the trial. The most common side-effects of nintedanib were gastrointestinal problems, particularly diarrhea, but most patients given nintedanib were able to cope with these side-effects without needing to stop treatment. Large trials like the INBUILD trial are important for helping us understand how diseases progress and in which patients particular drugs should be used.
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Progressão da Doença , Indóis , Fibrose Pulmonar , Tomografia Computadorizada por Raios X , Humanos , Indóis/efeitos adversos , Indóis/uso terapêutico , Indóis/administração & dosagem , Fibrose Pulmonar/tratamento farmacológico , Fibrose Pulmonar/fisiopatologia , Capacidade Vital , Índice de Gravidade de Doença , Resultado do Tratamento , Ensaios Clínicos Controlados Aleatórios como Assunto , Glucocorticoides/administração & dosagem , Glucocorticoides/efeitos adversos , Glucocorticoides/uso terapêutico , Pulmão/efeitos dos fármacos , Pulmão/fisiopatologia , Pulmão/diagnóstico por imagem , Doenças Pulmonares Intersticiais/tratamento farmacológico , Doenças Pulmonares Intersticiais/fisiopatologia , Doenças Pulmonares Intersticiais/diagnóstico , Fibrose Pulmonar Idiopática/tratamento farmacológico , Fibrose Pulmonar Idiopática/fisiopatologia , Fibrose Pulmonar Idiopática/diagnóstico , Masculino , Inibidores de Proteínas Quinases/efeitos adversos , Inibidores de Proteínas Quinases/uso terapêutico , Inibidores de Proteínas Quinases/administração & dosagemRESUMO
INTRODUCTION: Survivors of SARS-CoV-2 pneumonia often develop persistent respiratory symptom and interstitial lung abnormalities (ILAs) after infection. Risk factors for ILA development and duration of ILA persistence after SARS-CoV-2 infection are not well described in immunocompromised hosts, such as cancer patients. METHODS: We conducted a prospective cohort study of 95 patients at a major cancer center and 45 patients at a tertiary referral center. We collected clinical and radiographic data during the index hospitalization for COVID-19 pneumonia and measured pneumonia severity using a semi-quantitative radiographic score, the Radiologic Severity Index (RSI). Patients were evaluated in post-COVID-19 clinics at 3 and 6 months after discharge and underwent comprehensive pulmonary evaluations (symptom assessment, chest computed tomography, pulmonary function tests, 6-min walk test). The association of clinical and radiological factors with ILAs at 3 and 6 months post-discharge was measured using univariable and multivariable logistic regression. RESULTS: Sixty-six (70%) patients of cancer cohort had ILAs at 3 months, of whom 39 had persistent respiratory symptoms. Twenty-four (26%) patients had persistent ILA at 6 months after hospital discharge. In adjusted models, higher peak RSI at admission was associated with ILAs at 3 (OR 1.5 per 5-point increase, 95% CI 1.1-1.9) and 6 months (OR 1.3 per 5-point increase, 95% CI 1.1-1.6) post-discharge. Fibrotic ILAs (reticulation, traction bronchiectasis, and architectural distortion) were more common at 6 months post-discharge. CONCLUSIONS: Post-COVID-19 ILAs are common in cancer patients 3 months after hospital discharge, and peak RSI and older age are strong predictors of persistent ILAs.
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COVID-19 , Neoplasias , Humanos , COVID-19/complicações , Estudos Prospectivos , Assistência ao Convalescente , SARS-CoV-2 , Alta do Paciente , Pulmão/diagnóstico por imagem , Hospitalização , Neoplasias/complicações , Neoplasias/epidemiologiaRESUMO
BACKGROUND/OBJECTIVE: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) may involve the kidney, respiratory tract, skin, or central and peripheral nervous system. Reports of interstitial lung disease (ILD) in AAV (AAV-ILD) have been increasing. METHODS: We reviewed the medical records of all patients with AAV-ILD between January 1, 2007, and December 31, 2017, and compared their pulmonary involvement to patients with idiopathic pulmonary fibrosis (IPF). RESULTS: We identified 24 patients with AAV-ILD: 14 with microscopic polyangiitis, 8 with granulomatosis with polyangiitis, and 2 with eosinophilic granulomatosis with polyangiitis. Perinuclear or myeloperoxidase ANCA was present in 16 cases (66.7%), whereas cytoplasmic or proteinase 3 ANCA was positive in 8 (33.3%). Usual interstitial pneumonia (UIP) was seen in 11 (45.8%), probable UIP in 1 (4.2%), indeterminate UIP in 2 (8.3%), and an alternate diagnosis in 10 (41.7%), and was further characterized as chronic hypersensitivity pneumonitis-like pattern seen in 6 (25%), nonspecific interstitial pneumonia-like pattern in 3 (12.5%), and cryptogenic organizing pneumonia-like pattern in 1 (4.2%). Forced vital capacity and diffusing capacity declined over time in patients with AAV-ILD. When compared with the IPF cohort, patients with AAV-ILD had intermediate survival and speed of lung function decline (3-year survival in AAV-ILD group was 94% vs 69% in IPF). CONCLUSIONS: Antineutrophil cytoplasmic antibody-associated vasculitis ILD is a progressive and potentially fatal condition. Although most cases in the literature are associated with p-ANCA and positive myeloperoxidase, we found that a third of patients had c-ANCA or granulomatosis with polyangiitis. Our cohort adds experience in this rare manifestation of AAV.
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Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos , Síndrome de Churg-Strauss , Granulomatose com Poliangiite , Fibrose Pulmonar Idiopática , Doenças Pulmonares Intersticiais , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/complicações , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/diagnóstico , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/terapia , Anticorpos Anticitoplasma de Neutrófilos , Humanos , Fibrose Pulmonar Idiopática/diagnóstico , Fibrose Pulmonar Idiopática/terapia , Pulmão , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/etiologia , Doenças Pulmonares Intersticiais/terapia , Estudos RetrospectivosRESUMO
Dyspnea, cough, sputum production, and recurrent respiratory infections are frequently encountered clinical concerns leading patients to seek medical care. It is not unusual for a well-defined etiology to remain elusive or for the therapeutics of a presumed etiology to be incompletely effective. Either scenario should prompt consideration of central airway pathology as a contributor to clinical manifestations. Over the past decade, recognition of dynamic central airway collapse during respiration associated with multiple respiratory symptoms has become more commonly appreciated. Expiratory central airway collapse may represent the answer to this diagnostic void. Expiratory central airway collapse is an underdiagnosed disorder that can coexist with and mimic asthma, chronic obstructive pulmonary disease, and bronchiectasis. Awareness of expiratory central airway collapse and its spectrum of symptoms is paramount to its recognition. This review includes clear definitions, diagnostics, and therapeutics for this challenging condition. We performed a narrative review through the PubMed (MEDLINE) database using the following MeSH terms: airway collapse, tracheobronchomalacia, tracheomalacia, and bronchomalacia. We include reports from systematic reviews, narrative reviews, clinical trials, and observational studies from 2005 to 2020. Two reviewers evaluated potential references. No systematic reviews were found. A total of 28 references were included into our review. Included studies report experience in the diagnosis and/or treatment of dynamic central airway collapse; case reports and non-English or non-Spanish studies were excluded. We describe the current diagnostic dilemma, highlighting the role of dynamic bronchoscopy and tracheobronchial stent trial; outline the complex therapeutic options (eg, tracheobronchoplasty); and present future directions and challenges.
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Manuseio das Vias Aéreas/métodos , Broncoscopia , Atelectasia Pulmonar , Traqueobroncomalácia , Broncoscopia/instrumentação , Broncoscopia/métodos , Humanos , Atelectasia Pulmonar/etiologia , Atelectasia Pulmonar/fisiopatologia , Atelectasia Pulmonar/terapia , Stents , Traqueobroncomalácia/complicações , Traqueobroncomalácia/cirurgiaRESUMO
CASE PRESENTATION: A 64-year-old man presented for consideration for lung transplant. He had a history of previous tobacco use, OSA on CPAP therapy, and gastroesophageal reflux disease. He worked as a design engineer. The patient had a 4-year history of dyspnea on exertion, followed with periodic CT scan of the chest. Nine months prior to his evaluation for lung transplant, the patient developed worsening of dyspnea, dry cough, poor appetite, and weight loss. At times, the cough was violent and associated with chest pressure. He was prescribed systemic corticosteroids and antibiotics without improvement. Four months later, the patient noted sudden onset of severe chest pain and worsening dyspnea. A CT scan of the chest demonstrated extensive pneumomediastinum in addition to changes consistent with pulmonary fibrosis. An esophagogram showed thickening of the distal esophagus, but no signs of perforation. He was prescribed supplemental oxygen and advised to stop the use of CPAP. The patient sought a second opinion. A CT scan of the chest showed improvement of the pneumomediastinum and extensive fibrotic lung disease. Pulmonary function tests (PFTs) were consistent with a restrictive pattern, decreased diffusing capacity (Dlco), and a preserved residual volume over total lung capacity ratio. The patient was prescribed systemic corticosteroids with no improvement of his symptoms. Repeat PFTs showed further decline of Dlco, and he was referred for lung transplant evaluation.
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Enfisema Mediastínico/fisiopatologia , Insuficiência Respiratória/fisiopatologia , Humanos , Transplante de Pulmão , Masculino , Enfisema Mediastínico/cirurgia , Pessoa de Meia-Idade , Testes de Função Respiratória , Insuficiência Respiratória/cirurgiaRESUMO
Lung involvement in connective tissue diseases is associated with substantial morbidity and mortality, most commonly in the form of interstitial lung disease, and can occur in any of these disorders. Patterns of interstitial lung disease in patients with connective tissue disease are similar to those seen in idiopathic interstitial pneumonias, such as idiopathic pulmonary fibrosis. It may be difficult to distinguish between the 2 ailments, particularly when interstitial lung disease presents before extrapulmonary manifestations of the underlying connective tissue disease. There are important clinical implications in achieving this distinction. Given the complexities inherent in the management of these patients, a multidisciplinary evaluation is needed to optimize the diagnostic process and management strategies. The aim of this article was to summarize an approach to diagnosis and management based on the opinion of experts on this topic.
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Doenças do Tecido Conjuntivo/complicações , Doenças Pulmonares Intersticiais , Pulmão/diagnóstico por imagem , Fibrose Pulmonar , Biópsia , Doenças do Tecido Conjuntivo/diagnóstico , Saúde Global , Humanos , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/epidemiologia , Doenças Pulmonares Intersticiais/etiologia , Morbidade/tendências , Fibrose Pulmonar/diagnóstico , Fibrose Pulmonar/epidemiologia , Fibrose Pulmonar/etiologia , Testes de Função Respiratória , Taxa de Sobrevida/tendências , Tomografia Computadorizada por Raios XRESUMO
OBJECTIVES: To describe the effect of implementing a contemporary perioperative pulmonary hypertension (PH)-targeted protocol in patients with pulmonary arterial hypertension (PAH) undergoing noncardiac surgery (NCS). METHODS: The data of consecutive patients with PAH diagnosed by right heart catheterization who underwent NCS between January 1, 2006 and February 9, 2016 were reviewed. Patient demographics, etiology of PAH, clinical features, diagnostic data, utilization of PH-specific medications, and trend of perioperative complications rate were recorded during the study period. RESULTS: In the base cohort of 375 patients, 37 had NCS. The mean age at surgery was 62 years. Most patients were women (78%) classified in group 1 PAH. At the time of the surgery, 86% were New York Heart Association functional class III/IV and 97% had American Society of Anesthesiologists classifications 3 and 4. A larger proportion of patients displayed lower PAH risk scores between 2006 and 2011 (P = 0.045). Conversely, a higher percentage of patients exhibited moderately high to very high PAH risk scores between 2012 and 2016 (P = 0.003). Perioperative and anesthetic-related morbidity was 27%, and no difference was observed between either period (P = 0.944). Most of the complications (70%) were related to general anesthesia. Two deaths (5%) occurred in our study group, both during the 2006-2011 period. CONCLUSIONS: The combination of a multidisciplinary perioperative approach, utilization of novel pulmonary vascular disease-targeted therapy, adequate perioperative optimization, and thoughtful selection of anesthetic technique seems to be a potential strategy to at least maintain similar perioperative outcomes among higher- and lower-risk patients with PAH undergoing NCS.
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Hipertensão Pulmonar/terapia , Complicações Intraoperatórias/prevenção & controle , Equipe de Assistência ao Paciente , Assistência Perioperatória/métodos , Complicações Pós-Operatórias/prevenção & controle , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Anti-Hipertensivos/uso terapêutico , Protocolos Clínicos , Terapia Combinada , Quimioterapia Combinada , Feminino , Humanos , Hipertensão Pulmonar/complicações , Hipertensão Pulmonar/diagnóstico , Complicações Intraoperatórias/epidemiologia , Complicações Intraoperatórias/etiologia , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Estudos Retrospectivos , Resultado do Tratamento , Adulto JovemRESUMO
Background: Sarcoidosis is an idiopathic granulomatous disease characterized by variable organ involvement and non-necrotizing granulomas. Objectives: To determine how often non-necrotizing granulomas are not secondary to sarcoidosis. Methods: A retrospective review was conducted to evaluate all biopsies performed at Mayo Clinic in Jacksonville, Florida from January 1, 1996, to December 31, 2013, showing non-necrotizing granulomas. Results: Three hundred and eight biopsies showing non-necrotizing granulomas met inclusion criteria. The average age was 58.2 years, 60.7% were female, and 85% were Caucasian. The most common symptoms were pulmonary (74.6% of cases), and the most common objective finding was lymphadenopathy (33.8%). The organs biopsied included lung parenchyma (65.3%), intrathoracic lymph nodes (25%), other lymph nodes (1.6%), liver (1.3%), airway (1.3%), skin (1.3%), kidney (0.7%), bone marrow (0.7%), gastrointestinal (0.7%), and one each from the brain, heart, bone, bladder, spleen, tendon, and eye. The suspected diagnosis was confirmed in 224 cases (72.7%). From the remaining 84 cases (27.3%), suspected sarcoidosis was refuted in 9, the initial diagnosis was changed to sarcoidosis in 37 (44%), and in 38, it was changed to a different diagnosis. Sarcoidosis was the final diagnosis in 173 (56%). Conclusion: Sarcoidosis was the leading cause of non-necrotizing granulomas, but in 44% of cases, there was an alternate diagnosis. We estimate that more than a quarter of the initial diagnoses will be changed based on biopsy results and clinical course. (Sarcoidosis Vasc Diffuse Lung Dis 2017; 34: 115-121).
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Hydrocephalus is an uncommon presentation of neurosarcoidosis. We discuss the case of a 67-year-old woman with a prior diagnosis of hypothyroidism, systemic hypertension, and lung sarcoidosis who presented with a 1-month history of progressive impairment of consciousness leading to prostration and loss of sphincter control. At admission, patient was febrile with altered speech and without focalization. Laboratory results showed leucocytosis and mild anaemia. Cerebrospinal fluid (CSF) analysis was characterized by mild elevated protein level, increased cell count, normal glucose and adenosine deaminase (ADA), negative cytology, and no bacterial isolations. Electroencephalogram showed toxic-metabolic encephalopathy. Computed tomography (CT) of the brain revealed hydrocephalus without structural damage, and magnetic resonance imaging of the brain demonstrated non-specific diffuse meningeal enhancement and periventricular changes supporting normal pressure hydrocephalus. Chest X-ray showed mediastinal adenopathy and parenchymatous lesions, consistent with stage II lung sarcoidosis. A ventriculoperitoneal shunt was installed, and the patient experienced rapid improvement in her symptoms.
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Pulmonary vein stenosis (PVS) is a known complication after catheter ablation of arrhythmias. Surprisingly, little information is available on its manifestations in the lung. We describe the case of a 39-year-old woman who presented from an outside hospital with worsening shortness of breath after catheter ablation of pulmonary veins for atrial fibrillation. After an initial diagnosis of pneumonia and its nonimprovement with antibiotics, a surgical lung biopsy was done and interpreted as nonspecific interstitial pneumonia (NSIP) with vascular changes consistent with pulmonary arterial hypertension. Later, she was admitted to our institution where a transthoracic echocardiogram (TTE) and subsequent computed tomography (CT) angiogram of the heart showed severe stenosis of all four pulmonary veins. The previous lung biopsy was rereviewed and reinterpreted as severe parenchymal congestion mimicking NSIP. Our case demonstrates that PVS is an underrecognized complication of catheter ablation, and increased awareness among both clinicians and pathologists is necessary to avoid misdiagnosis.
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A 40-year-old woman (a nonsmoker) with history of idiopathic thrombocytopenic purpura and a platelet count > 90,000 cells/µL without specific medication was referred to pulmonary clinic for evaluation of multiple pulmonary nodules. The patient presented to an outside hospital with fatigue, lack of energy, and dyspnea on exertion for 2 years. She denied fever, cough, chest pain, or weight loss. An initial chest radiograph showed bilateral multiple pulmonary nodules. A chest CT scan revealed multiple nodular lesions, varying in size, in all lobes of both lungs. There was no mediastinal lymphadenopathy or pleural effusion. There was no significant hypermetabolic activity on a subsequent fluorodeoxyglucose PET scan/CT scan, and there had been no significant change. She underwent CT scan-guided percutaneous transthoracic biopsy and bronchoscopy with transbronchial biopsies, all of which were inconclusive. An open lung biopsy was considered.
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Granuloma do Sistema Respiratório/diagnóstico , Imagem Multimodal/métodos , Nódulos Pulmonares Múltiplos/diagnóstico , Cirurgia Torácica Vídeoassistida/métodos , Adulto , Biópsia por Agulha , Broncoscopia/métodos , Diagnóstico Diferencial , Feminino , Seguimentos , Granuloma do Sistema Respiratório/cirurgia , Humanos , Hialina/metabolismo , Imuno-Histoquímica , Nódulos Pulmonares Múltiplos/cirurgia , Tomografia por Emissão de Pósitrons/métodos , Radiografia Torácica/métodos , Testes de Função Respiratória , Tomografia Computadorizada por Raios X/métodos , Resultado do TratamentoRESUMO
Primary malignant lymphoma of the trachea is rare. We report a case of a 60-year-old woman with a mucosa-associated lymphoid tissue lymphoma of the trachea and review the existing literature. This case highlights the importance of including intraluminal lesions in the differential diagnosis when a variable degree of obstruction is seen on pulmonary function testing.