What are the characteristics of restrictive lung disease?
- Decreased lung volume
- Decreased oxygen diffusing capacity
A patient presents with acute dyspnea, cough and mild fever. PFTs are performed showing a restrictive pattern characterized by decreased compliance, reduced diffusion capacity, and hypoxemia. The Bronchoalveolar lavage shows mostly CD8+ T lymphocytosis. The patient's Syx resolve in 36 hours. He claims that he is a farmer. What is the Dx?
Acute Hypersensitivity Pneumonitis (Extrinsic Allergic Alveolitis)
What histology is seen in a patient with acute hypersensitivity pneumonitis? Chronic?
- Acute: T lymphocytosis, PMNs
- Chronic: Poorly formed noncaseating granulomas, lymphocytes, plasma cell
What type of hypersensitivity is hypersensitivity pneumonitis?
- Immunce Complex Mediated (Type III)
- Cell-mediated (Type IV)
A lung biopsy shows a mild peribronchiolar chronic inflammatory interstitial infiltrate with a focus of intraluminal organizing fibrosis and poorlyformed granulomas. What is the Dx?
A young black patient presents with cough and dyspnea. No infectious etiology can be found. A lung biopsy is performed and shown below. The biopsy shows noncaseating granulomas along the bronchovascular interstitium and asteroid bodies. The patient responds well to corticosteroid treatment and is released. What is your Dx?
What laboratory test is specific for sarcoidosis?
There is none? Multiple performed tests along with clinical and radiologic findings allow for the Dx of sarcoidosis
What is the current believed molecular pathogenesis of sarcoidosis?
Exaggerated helper/inducer T cell response to exogenous or autologous antigens
What are the typical findings in bronchoalveolar lavage of a patient with sarcoidosis?
Increased proportion of CD4+ lymphocytes
What is a characteristic cellular feature of active sarcoidosis?
Nonspecific polyclonal activation of B cells by T-helper cells leading to hyperglobulinemia
Although nonspecific for sarcoidosis, this histology can be frequently found. What is it?
A patient presents to your clinic with dyspnea on exertion and dry cough which he states has been getting worse over the past couple of years. A PFT reveals restrictive lung disease and auscultation reveals fine "velcro" inspiratory crackles. A lower lobe lung biopsy is performed and shows patchy subplural fibrosis with uscopic honeycomb fibrosis. What is the Dx? What is the Px?
Usual Interstitial Pneumonitis; Tachypnea, Cyanosis, Cor pulnonale, Death (w/i 4-6 years)
What is the cause of Usual Interstitial Pneumonia?
Immunologic, viral and genetic factors are thought to play a role.
Lungs of a patient with Usual Interstitial Pneumonia are shown below. What are typical features of the specimen that point to UIP?
- Small lungs
- Fibrosis (especially in the lower lobes)
- Patchy interstitial fibrosis with areas of normal lung adjacent to fibrotic areas
- Honeycomb lung
A patient complains of shortness of breath and cough over the past several months. A lung biopsy is performed and cellular and fibrosing patterns are found. The observed changes are diffuse and uniform. CT reveals "ground glass" changes in the lower lobes bilaterally. What is the Dx?
Nonspecific Interstitial Pneumonia
What are the two types of Nonspecific Interstitial Pneumonia? What characteristics are generally absent in NSIP?
- Cellular - alveolar septa are mildly to moderately infiltrated with lymphocytes
- Fibrosing - Septa show diffuse fibrosis
Honeycombing and fibroblastic foci are absent/inconspicuous
A male smoker presents to the clinic and is experiencing mild respiratory distress. Chest imaging reveals ground glass infiltrates in the lower lobes. The lung biopsy shows an accumulation of alveolar macrophages containing a fine golden-brown pigment, preservation of the alveolar architecture, mild alveolar septal fibrosis and type II pneumocyte hyperplasia. What is the Dx?
Desquamative Interstitial Pneumonia
A patient presents with mild respiratory dysfn. with radiographic findings predominating in the upper lobes. Lung biopsy reveals patchy accumulation of macrophages with brown, finely granular pigment centered around bronchioles. What is your Dx? What probably caused this disease?
Respiratory Bronchiolitis; Smoking
A 55 yo patient presents with acute fever, cough and dyspnea and a Hx of flulike illness 4-6 wks prior. CXR reveals localized opacities or bilateral interstitial infiltrates. PFT reveals restrictive ventilatory pattern. Lung biopsy reveals fibroblastic tissue within bronchioles and alveolar ducts. Lung architecture is preserved. What is the Dx? What is the cause?
Organizing pneumonia pattern (Bronchiolitis obliterans-organizing pneumonia [BOOP]); Most cases are idiopathic so referred to as Cryptogenic organizing pneumonia or Idiopathy BOOP
A patient with a Hx of Sjoegren's Syndrome presents with cough and progressive dyspnea. Lung biopsy shows infiltation of alveolar septa and peribronchiolar spaces by lymphocytes, plasma cells and macrophages. Alveolar architecture is preserved. Hyperplasia of type II pneumocytes and proteinaceous exudate are present. What is your Dx? What other conditions are associated with this disease?
Lymphoid Interstitial Pneumonia; Collagen vascular diseases, dysproteinemia, and HIV infection
A 35 yo patient presents with nonproductive cough, dyspnea on exertion, and spontaneous pneumothorax. CXR reveals diffuse, bilateral reticulonodular lesions in the upper lobes. The lung biopsy reveals scattered nodular infiltrates with a stellate border extending into the surrounding interstitium. The lesions are centered on bronchioles and contain varying proportions of Langerhans cells admixed with lymphocytes, eosinophils and macrophages. What is the Dx? What caused this disease?
Langerhans Cell Histiocytosis (Histiocytosis X); Likely SMOKING
Below is a lung biopsy that reveals Langerhans cells. What are the distinctive characteristics of these cells?
- Birbeck Granules
- C3, IgG-Fc receptors, CD1a and human leukocyte antigen (HLA)-DR
- S-100 protein expression
A patient presents to your clinic with a spontaneous pneumothorax. Previously the patient had shortness of breath, hemoptysis, cough, and chylous effusions. Her PFT shows increased total lung capacity, decreased diffusion capacity and obstructive and restrictive features. A lung biopsy reveals a cystic space lined with smooth muscle bundles. What is the Dx? What are major associations of this disease?
Lymphangioleiomyomatosis; Women of child bearing age, Tuberous Sclerosis
What are procedures that have helped to curtail the smooth muscle proliferation found in Lymphangioleiomyomatosis?
Oophorectomy and progesterone Tx
What disease does Lymphangioleiomyomatosis resemble?