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Histopathologic criteria of usual interstitial pneumonia (UIP)/idiopathic pulmonary fibrosis (IPF) were defined over the years and endorsed by leading organizations decades after Dr. Averill A. Liebow first coined the term UIP in the 1960s as a distinct pathologic pattern of fibrotic interstitial lung disease. Novel technology and recent research on interstitial lung diseases with genetic component shed light on molecular pathogenesis of UIP/IPF. Two antifibrotic agents introduced in the mid-2010s opened a new era of therapeutic approaches to UIP/IPF, albeit contentious issues regarding their efficacy, side effects, and costs. Recently, the concept of progressive pulmonary fibrosis was introduced to acknowledge additional types of progressive fibrosing interstitial lung diseases with the clinical and pathologic phenotypes comparable to those of UIP/IPF. Likewise, some authors have proposed a paradigm shift by considering UIP as a stand-alone diagnostic entity to encompass other fibrosing interstitial lung diseases that manifest a relentless progression as in IPF. These trends signal a pendulum moving toward the tendency of lumping diagnoses, which poses a risk of obscuring potentially important information crucial to both clinical and research purposes. Recent advances in whole slide imaging for digital pathology and artificial intelligence technology could offer an unprecedented opportunity to enhance histopathologic evaluation of interstitial lung diseases. However, current clinical practice trends of moving away from surgical lung biopsies in interstitial lung disease patients may become a limiting factor in this endeavor as it would be difficult to build a large histopathologic database with correlative clinical data required for artificial intelligence models.
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BACKGROUND: Chronic lymphocytic leukemia (CLL) is the most common type of leukemia in Western countries. Although various patterns of lung involvement with CLL have been reported, data on clinicoradiologic presentation are sparse. METHODS: A computer-assisted search was conducted to identify patients encountered at Mayo Clinic from 1998 to 2022 and had leukemic pulmonary infiltrates (LPI) with CLL demonstrated on lung biopsy. Medical records and chest imaging studies were reviewed to identify clinical and radiologic features. RESULTS: Among 13 patients, median age was 77 years (range: 60-88) and included 10 men (77 %). All patients were known to have CLL with a median duration of 96 months (range: 50-408), and none were on treatment. Most common symptoms were dyspnea (62 %), cough (54 %), and fatigue (46 %); 2 patients (15 %) were asymptomatic. Dominant abnormality on CT consisted of single or multiple nodular/mass-like opacities in 10 patients (77 %), while diffuse centrilobular nodules, pleural mass, and diffuse bronchial wall thickening were each seen in one patient, respectively; intrathoracic lymphadenopathy was present in all. After diagnosis of LPI, treatment for CLL was administered to 7 patients (54 %); 6 patients (86 %) exhibited improvement. During follow-up (median 41 months), 8 (62 %) patients died. Causes of death included progressive CLL or treatment-related complications (2 patients), pneumonia (1 patient), unrelated causes (3 patients), and unknown in 2 patients. CONCLUSIONS: LPI in CLL is generally encountered in patients with known untreated CLL. The main imaging feature is single mass-like opacity or multiple nodular/mass-like opacities, associated with intrathoracic lymphadenopathy.
Assuntos
Leucemia Linfocítica Crônica de Células B , Linfadenopatia , Pneumonia , Masculino , Humanos , Idoso , Leucemia Linfocítica Crônica de Células B/complicações , Leucemia Linfocítica Crônica de Células B/diagnóstico por imagem , Leucemia Linfocítica Crônica de Células B/patologia , Linfadenopatia/etiologia , Linfadenopatia/complicaçõesRESUMO
Background: Lung involvement in the context of idiopathic inflammatory myopathies has significant impact on outcome; early and accurate diagnosis is important but can be difficult to achieve. In particular, patients without clinically evident muscle involvement pose a significant diagnostic challenge. Methods: A computer-assisted search was conducted to identify patients with amyopathic interstitial lung disease associated with the presence of myositis-specific autoantibodies. Medical records and chest imaging studies were reviewed to identify clinical and radiologic features at presentation. Results: Of the 35 patients with amyopathic interstitial lung disease associated with myositis-specific autoantibodies, the median age was 65 years (range 43-78) and 20 were women (57%). Of the patients, 34% had previously visited the rheumatology department. Presenting symptoms consisted of dyspnea (94%), cough (43%), and arthritis (23%). Raynaud phenomenon, "mechanic hands," Gottron papules, and inspiratory crackles were present in 23, 31, 9, and 74% of patients, respectively. After a detailed history, none of the patients reported muscle weakness, while four (11%) exhibited increased CK levels; of these four, two had a concomitant increase in aldolase levels. Median FVC was 79% predicted (range: 49-135) and median DLco was 50% predicted (range: 17-103). HRCT pattern was suggestive of an alternative to UIP pattern in 31/33 (94%) patients; the most common imaging patterns were NSIP (49%) and NSIP/OP (39%). Conclusion: In patients with NSIP and NSIP/OP pattern, the presence of amyopathic interstitial lung disease associated with myositis-specific autoantibodies should be considered even in the absence of clinical evident myositis.
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CASE PRESENTATION: A 62-year-old woman came to our hospital with worsening cough and dyspnea over the preceding week, during which time she had been treated with azithromycin and prednisone for suspected pneumonia. She had no fever, chills, or sweats, but her cough had become productive of clear to blood-tinged phlegm during the interval. Medical history was significant for insulin-dependent diabetes mellitus and OSA. She had quit smoking 44 years earlier and had no history of lung disease. She was a bank teller residing in southeastern Minnesota and described no relevant inhalational or environmental exposures, drug use, aspiration, or travels preceding her illness.
Assuntos
Tosse , Dispneia , Tomografia Computadorizada por Raios X , Humanos , Feminino , Pessoa de Meia-Idade , Tosse/etiologia , Tosse/diagnóstico , Dispneia/etiologia , Dispneia/diagnóstico , Diagnóstico Diferencial , Nódulos Pulmonares Múltiplos/diagnóstico , Nódulos Pulmonares Múltiplos/diagnóstico por imagem , Nódulos Pulmonares Múltiplos/etiologia , Neoplasias Pulmonares/diagnóstico , Neoplasias Pulmonares/complicaçõesAssuntos
Neoplasias do Sistema Nervoso , Humanos , Feminino , Masculino , Pessoa de Meia-Idade , Adulto , Idoso , Adolescente , Adulto Jovem , Neoplasias do Sistema Nervoso/terapia , Neoplasias do Sistema Nervoso/diagnóstico , Neoplasias do Sistema Nervoso/patologia , Criança , Idoso de 80 Anos ou mais , Resultado do Tratamento , Pré-EscolarRESUMO
OBJECTIVE: Aspiration can cause a variety of pulmonary syndromes, some of which are not well recognized. The objective of this study was to assess the demographic, clinical, radiological, and histopathological correlates of diffuse aspiration bronchiolitis (DAB), a bronchiolocentric disorder caused by recurrent aspiration. METHODS: This was a retrospective study of 20 consecutive patients with DAB seen at the Mayo Clinic in Rochester, Minnesota, between January 1, 1998 and June 30, 2014. RESULTS: The median age of the patients was 56.5 years (range, 22-76 years), and the male/female ratio was 2.3:1.0. In 18 patients, the diagnosis of DAB was based on the results of a lung biopsy; in the 2 remaining patients, it was based on clinical and radiological features, together with documented aspiration observed in a videofluoroscopic swallow study. In 19 patients (95%), we identified predisposing factors for aspiration, including gastroesophageal reflux disease (GERD), drug abuse, and dysphagia. Common presenting features included cough, sputum production, dyspnea, and fever. Twelve patients (60%) had a history of recurrent pneumonia. In all of the patients, chest CT revealed bilateral pulmonary infiltrates consisting of micronodules and tree-in-bud opacities. In the majority of patients, interventions aimed at preventing recurrent aspiration (e.g., anti-GERD therapies) led to improvement in the symptoms of DAB. CONCLUSIONS: Young to middle-aged subjects with recognizable predisposing factors for aspiration and who report a history of recurrent pneumonia are at increased risk for DAB. Although DAB is not well recognized, certain chest CT features are characteristic of the disorder. .
OBJETIVO: A aspiração pode causar diferentes síndromes pulmonares, algumas das quais não são bem reconhecidas. O objetivo deste estudo foi avaliar as correlações demográficas, clínicas, radiológicas e histopatológicas da bronquiolite aspirativa difusa (BAD), um distúrbio bronquiolocêntrico causado por aspiração recorrente. MÉTODOS: Estudo retrospectivo de 20 pacientes consecutivos com BAD atendidos na Clínica Mayo, em Rochester, Minnesota, entre 1 de janeiro de 1998 e 30 de junho de 2014. RESULTADOS: A mediana de idade dos pacientes foi de 56,5 anos (variação, 22-76 anos), e a relação homem/mulher foi de 2,3:1,0. Em 18 pacientes, o diagnóstico de BAD foi baseado nos resultados de biópsia do pulmão; nos 2 casos restantes, ele foi baseado em características clínicas e radiológicas, em conjunto com aspiração documentada observada em um estudo de deglutição videofluoroscópico. Em 19 pacientes (95%), foram identificados fatores predisponentes para a aspiração, incluindo doença do refluxo gastroesofágico (DRGE), abuso de drogas e disfagia. Características de apresentação comuns incluíam tosse, produção de escarro, dispneia e febre. Doze pacientes (60%) tinham uma história de pneumonias de repetição. Em todos os pacientes, a TC de tórax revelou infiltrado pulmonar bilateral com micronódulos e opacidades do tipo árvore em brotamento. Na maioria dos pacientes, as intervenções destinadas a impedir a aspiração recorrente (por exemplo, terapias anti-DRGE) levaram a uma melhoria nos sintomas de BAD. CONCLUSÕES: Indivíduos jovens até os de meia-idade com fatores predisponentes reconhecíveis para aspiração e que relatam uma história de pneumonias de repetição apresentam um risco aumentado para BAD. Embora BAD não seja bem reconhecida, certos aspectos na TC de tórax são característicos da doença. .