Okay, so Dr. Buckley and I are splitting this session, so I'm going to go through the first 30 minutes or so, try to get through six or seven questions, and then we'll turn it over to Jack. So I think we'll go fairly fast. It actually are topics we happen to have touched on many of these throughout the day. So let's go ahead with the first question. I am on the SEEK editorial board, but no conflicts. So this is a 45-year-old woman referred for mild shortness of breath, and the findings noted on the CT, abdomen, and chest. Which of the following statements is correct regarding the disease process? Lung tissue is staying positive with S100, a cholesterol pleural effusion develops in one-third of patients, the disease does not recur following transplantation, rapamycin may be therapeutic. So again, a lot of this will be straightforward since we've covered it today. Okay, good, and three-quarters of you do have the correct answer. So I think what's good about SEEK questions, you learn as much from the negative distractors as you do from the positive. So RAPA is the correct answer. And let's go through this. We're just going to touch on the, we talked about the incidence today, again, remember it's a smooth muscle disease, the sporadic mutation being most common in what we see accounting for about 85%. We had talked about the exam and the PFTs, we'll talk again about the CT. One word about the effusions, so they're chylous, true chylous effusion, meaning triglyceride effusions, not cholesterol, which are pseudo-chylous effusions. So that's why that was incorrect. They do have the angiomyolipomas, and the tissue stain buzzword is the HMB45. Again, I got several questions in the morning that people had a case that met all other criteria, but their VEGF level was less than 800, but they happened to have other LAM criteria that would still make the diagnosis. But in general, the VEGF is going to be greater than 800 in the serum. And we talked about this morning why rapamycin is beneficial, progesterones can help, and we tried to avoid estrogens and pregnancy. It is a disease that can recur following transplantation. So this also gives me an opportunity to compare and contrast two of our main cystic lung diseases that show up on board, so LAM and LCH. So remember LAM, premenopausal women, LCH is men more common than females. They tend to be younger, and they're almost always smokers for LCH. Radiographically in LAM, multiple thin-walled, round cysts that can be small or large, they're diffused throughout the lungs, whereas in LCH, as you heard from Dr. Ryu, they're eccentric cysts, is kind of what is commonly described there, and they're more in the mid and upper lung fields, and there are associated nodules. So big difference there, purely cystic versus cystic nodules. They both get spontaneous pneumothoraces. For LAM, the VEGFD, and remember the HMB45, and as you heard from Jay this morning, for LCH, you think about CD1A and S100 are your stains there. Complications for LAM, chylis effusions and chylis societies, and the angiomyolipomas, and with LCH, they can get diabetes insipidus when you get granulomas in the hypothalamic pituitary tract and bone lesions. Treatment for LAM, serolimus, and lung transplant for LCH, really to stop smoking is your best treatment. You can try steroids, you can try cladribine, and lung transplant can be performed for both. So let's look at why the others were wrong. So the S100, that's LCH, HMB45 is for LAM. The cholesterol effusions, no, they're true, chylis orthoses with triglycerides, and it does recur following transplantation. Okay, this is a 65-year-old man who presents with a 20-pound weight loss, night sweats, fevers, and a productive cough with scant hemoptysis for four weeks, a history of 40 pack years tobacco use. He's thin, temperature's 38.3, otherwise a stable vital signs, poor dentition, right upper lobe agophony, scattered wheezes. A white count of 10,000, hemoglobin 11.5, the sputum of AFV is negative, times three, bronchoscopy is performed, the stains are AFV negative on the BAL. The appropriate treatment for the most likely diagnosis is, and there's the imaging. So you see the radiographs, the CT, there's an H and E stain, and there's a silver stain. So would you do PEN IV for four to six weeks and PO for six to 12 months, imipenem IV for six weeks, Bactrim PO for six months, or a PEN PO for three months? What do you think's the best treatment for this likely diagnosis? And again, we heard about this from Marcos Restrepo this morning. All right, good, and half of you have the right answer, and we'll go through why the other ones are incorrect. So actino, as you heard, there can be cervical, facial, abdominal, or thoracic. Usually the thoracic is what we would see most commonly, and it's an extension of aspiration from the oropharynx. Remember, it's gram-positive, filamentous, it's anaerobic. They do have sulfur granules with actino, and they're AFB negative. Clinically, subacute to chronic presentation with constitutional symptoms, like this patient had weight loss, chest pain, you can have hemoptysis, anemia. The imaging can show a mass, it can easily cavitate, and you may have pleural disease. And actino can often cross the fissures and into the chest wall, and that's called an empyeminocytins. The diagnosis can be made with FNA, or with bronchoscopy, or biopsy, or a culture. And the treatment for severe disease is pen IV for four to six weeks, then an oral penicillin, usually for six to 12 months. Other agents that can be used, ceftriaxone or moxicillin. If you're pen allergic, you can think about doxy, or the macrolides, questionably clindamycin, or the carbapenems. And in isolated cases, and you can't get control of the disease, you can consider surgery. Now, nocardia, in contrast, it's weakly acid fast, so that was the big difference there, and you would treat with trimethoprim, sulfurobacter. So comparing and contrast to actino, it's gram-positive anaerobe, nocardia, gram-positive aerobe. Clinically, for actino, pulmonary, pelvic, cervical, facial, nocardia, pulmonary, but remember CNS involvement, and you can also get skin involvement. Generally, actino patients are immunocompetent, but in nocardia, they're often, not always, but often immunocompromised. AFB negative for actino, weakly positive for nocardia. The sulfur granules are associated with actino, nocardia does not have them. Radiographically, for actino, as I showed here, infiltrates cavities and the occasionally pleural involvement, and nocardia tends to be more nodular with infiltrates. And the treatment is different. Actino, our go-to drug, are the penicillins, and for nocardia, it's the sulfur drugs. So why are the others incorrect? Imipenem works, but it's kind of big guns for this case, and the duration would be too short. Bactrim's incorrect, it does not cover, trimethoprim itself does not cover actino, and penpo for three months, that's the wrong initial route for someone this sick and too short a duration. Okay, here's a 20-year-old patient. She has AML, and she status posed an autologous stem cell transplantation 16 days prior. During the past few days, she has developed a nonproductive cough, dyspnea, and fever. She's had scant hemoptysis. Her O2 saturation has fallen, she now requires supplemental oxygen. She then requires intubation and mechanical ventilation. Bronchoscopy is performed, and serial left-to-right aliquots, make sure you can see it, of BAL fluid are shown. The BAL fluid recovered from several other segments has a similar appearance. Bacterial fungal and AFB smears are negative, and the cytology of the BAL fluid is negative for infectious agents. Which of the following statements regarding this condition is true? And there you have the imaging, as well as the BAL. Which is true? Associated mortality may be more than 60 to 80 percent. The condition is more common in allo than otto stem cell transplant recipients. Hemoptysis occurs in about 90 percent of patients with this condition. Transbronchial biopsy is often necessary to confirm the diagnosis. So actually, we ended up hearing about DAH as well earlier from Rodrigo's talk. Okay. So a little bit of a split here, and we'll go through the correct answer. So the mortality is extremely high with this condition. So for DAH, it complicates hematopoietic stem cell transplant. It's pretty rare. It's less than 1 to 2 percent in most series, about 5 percent in others. Out of interest of the BMT complications, this is one of the ones where it's as equal or maybe more in the otto's than the allo's. Most of the other complications, like graft-versus-host disease, and some of the infectious complications are more common in the allo's because we immunosuppress them more. So risk factors for DAH in stem cells pre-transplant, intensive chemotherapy, if they had total body and or thoracic radiation, renal dysfunction, weakly associated thrombocytopenia, which many of these patients have, but if you correct that, it doesn't always correct the DAH. So there certainly is an association, and it's a risk factor, but it's not corrected by correcting the thrombocytopenia. Patients present, as this patient did, with progressive dyspnea and fevers, chills, diffuse alveolar opacities. They may or may not have hemoptysis, and it's absent, actually, in about a third of cases and even a higher rate of being absent in this particular kind of DAH in stem cell transplants. As you heard earlier, diffusing capacity can be increased due to the blood in the alveoli, but of course, these patients are so sick that that's rarely performed. The timing in BMT, it's an early complication, usually within the first 30 days and especially around the time of engraftment, and radiographically, as in this case, you're going to see GGO and bilateral, what looks like pulmonary edema on imaging. The diagnosis can be made with BAL with serial aliquots, and you see a progressively bloodier return and or you can analyze the BAL for hemocytin-laden max, and it's greater than or equal to 20%. You also want to exclude infectious etiologies. In the treatment, as someone asked a question earlier today, you would try to correct underlying risk factors when you can. You would, if they're thrombocytopenia, you would, of course, try to get them to a more acceptable platelet count, and most people, without great clinical evidence, but most people do treat this with steroids in this setting. There are cases of using Factor VIIa, of using plasmapheresis. Now I think we use Inhale TXA for so many things that we would try it here as well. Many patients do require mechanical ventilatory support, and the prognosis is poor with mortality rates approaching 80%, so that was the correct answer in the question. What is it due to? It's probably due to underlying lung injury and cytokine release, some microthrombi formation as well, and this may be exacerbated by pre-existing treatment with chemotherapy and or radiation. And again, it usually occurs at the time of white cell recovery and engraftment. So B was incorrect. It's at least equal, and in fact, it's probably a little more common in auto than allo stem cell transplants. This occurs in much less than 90% of cases, and you definitely don't need a transbronchial biopsy. In fact, they're so thrombocytopenic that it would be risky, and there's no need to perform a transbronchial biopsy. The differential diagnosis of DH we've gone through pretty extensively today, but remember the vasculitides, connective tissue diseases, anti-GBM, formerly called good pasture, certain drugs such as penicillamine and cocaine, coagulopathy, something called idiopathic pulmonary hemocytosis or bland hemorrhage, as we saw earlier, and then mitral stenosis, the non-pulmonary cause of DH. Okay, here we have a 63-year-old woman who presents with fatigue, vague right anterior chest pain, dry cough, and occasional dyspnea. Her past medical history is unremarkable. Her chest CT is pale, but otherwise a normal exam. Hemoglobin's eight with normal indices, otherwise normal CBC. The chest CT is shown, and the remainder of the chest CT is unremarkable without parenchymal disease or mediastinal adenopathy. An I-131 scan is done. It's negative for uptake in the thorax. And what is the most likely diagnosis in this patient? So there's her imaging. Is this a teratoma, a lymphoma, an intrathoracic thyroid, or a thymoma? Okay, so we have some split, but the majority have the correct answer, and this is a thymoma. So the thymoma, as you can see here, so round and well demarcated. It's in the anterior mediastinum, as we heard from Dr. Ayou. The differential diagnosis, the teratomas, those are germ cell tumors in children and young adults. They're usually symptomatic. Lymphomas, also young adults, often with systemic B-type symptoms. If they're large, you can have airway obstruction. In intrathoracic thyroid, middle-aged women, and they usually extend above the thoracic inlet and often have a positive I-131 scan, which this patient did not. And then lipomas, you would know from the fat density on the CT, which is not shown here. You can see that that's certainly soft tissue density. So as you heard from Jay, it's the most common cause of an anterior mediastinal mass in adults. Usually, just as this is located adjacent to the junction of the heart and great vessels, round or oval, smooth margins, as this mass has. It develops in patients usually in the middle age range, 40 to 60, equal, male and female. About 60% have some symptoms, about 40% are asymptomatic. And the symptoms can be vague. It can be cough, dyspnea, or chest pain. The paraneoplastic syndromes are interesting, and Jay went through these in pretty great detail. It occurs in myasthenia gravis in 10% to 50% of patients with thymomas. And it's due to thymic production of autoantibodies against the acetylcholine receptors. It usually does resolve with resection, so that's one of the paraneoplastic syndromes. It gets better, and they present with diplopliatosis, weakness, dysphagia, fatigue. Pure red cell aplasia, which is what actually this patient had, that occurs in 5% to 15% of patients with thymomas. They have a normocytic, normochromic anemia, and this may resolve with resection, not as absolute as with the MG. For hypogammaglobulinemia, rare, less than 5%, but that's good syndrome. You heard about 10% of patients with hypogammaglobulinemia have a thymoma. And then another one that's well-described is Cushing syndrome. Malignant characteristic imaging is for the diagnosis. It is in the family of malignant epithelial neoplasms, and has malignant potential. And it's based on the extent of primary tumor, and if they're invading through the capsule. And you would resect, if possible, and sometimes, depending on what you find, chemotherapy and or XRT may be needed. Okay, here's a 57-year-old man. He underwent a BLT for COPD. The donor was CMV positive, the recipient CMV negative. Post-op course was complicated by initial weaning difficulties, but he improved, discharged to rehab two months post-op. After that, he remained well with no pulmonary complaints. He underwent routine surveillance transbronchial biopsies to monitor for rejection at one and two months after transplant, and no abnormalities were noted. Monthly monitoring of the blood for CMV antigenemia have been negative post-op, and you're now seeing at the four-month follow-up visit. He feels pretty well, but has noted some low-grade fever, mild cough with yellow sputum. His current medications include tacrolimus twice daily, prednisone five milligrams daily, mycophenolate twice daily, trimethoprim, sulfa three times weekly, and valgancyclovir daily decreased recently for renal insufficiency. Exam is normal. PFT's FEV1 is decreased 12 percent from baseline. The tacrolimus level is therapeutic, CMV antigen results are pending, transbronchial biopsy is performed for all of the above reasons, and the pathology is shown. So the question is, the abnormalities are most likely due to PTLD, or post-transplant lymphoproliferative disorder. Is this acute cellular rejection, cytomegalovirus infection, or PJP infection? What do you think we are seeing here with that story and this imaging? Okay, great, and three-quarters of you have the correct answer. This is CMV infection, CMV pneumonitis. So we heard our lung transplant talk earlier today. Immunosuppression usually peaks in the person who's kind of on schedule doing well post-transplant, usually by six months post-transplant, and in that period is when we see most of our opportunistic infections, including that due to CMV, PJP, aspergillus, or candida. So CMV is associated with development of bronchiolitis obliterans syndrome, or CLAD, chronic lung allograft dysfunction, as you heard earlier. And the most severe CMV infections occur in what we call mismatch, meaning our donor is CMV positive and our recipient is CMV negative, which is what happened with this patient. The negative negatives have the lowest risk of CMV, and then the other permutations have the intermediate risk, CMV. Most of the time, the severe infections, as in this case, are due to primary infections. You can also get secondary CMV infection, which is reactivation of latent disease. There can be a spectrum of presentation. They can be mildly asymptomatic or mildly flu-like illness is one of the presentations. They can have a full-blown pneumonitis, like this patient ended up having a retinitis. You can get hepatitis, colitis, or gastroenteritis. And we monitor them closely with serial blood, looking for CMV, PCRs, and antigens at every clinic visit. The other things we do in terms of management, we prophylaxis patients for at least the first year post-transplant, especially if in the mismatch group. We sometimes continue that longer in those patients who have either had infection or are mismatched. And Valcite is what we use for both prophylaxis and higher dose for treatment. You heard about ACR this morning. It's usually in the first, earlier post-transplant, but can happen later if you stop your immunosuppression. And that's also a nonspecific presentation with URI, perhaps symptoms of dyspnea, but you do have a decrease in your PFTs with that. On X-ray, you can have a perihyal or infiltrate or effusion. And as Debbie said this morning, remember, it's a diagnosis made by transbronchial biopsy. So ACR is a histologic diagnosis, and you treat that with steroids. PTLD, you also saw that highest instance in the first post-transplant year. And remember, with that, EBV is our buzz association. So it's patients who were EBV negative and have an EBV positive donor will be the highest risk for developing this. And they can have nodules or adenopathy on CT. You can have extra pulmonic involvement. And the treatment for PTLD is rituximab and a decrease in your immunosuppression. So just remember the characteristic appearance for CMV. It's described as the owl's eyes, and you have these intranuclear occlusion bodies, as shown here. They're markedly enlarged. That's how it gets its name, cytomegalic cells, so large cells. And they have the purple intranuclear inclusions and some basophilic, small intracytoplasmic occlusions. And I think they would show you a very classic owl's eyes appearance. Radiographically, you can have areas of GGO. You can have some interstitial disease. You can even have some tree and bud changes. And the treatment, IV, or now often oral valgancyclovir at high doses. And then, as I mentioned, we do prophylaxis at highest risk groups. So why were these others incorrect? It would have been a little early for PTLD. We didn't see the classic lymphocytes that you would expect. The timing was OK for ACR, but again, we didn't see the lymphocytic vasculitis that you usually see on biopsy. The CT findings would be unusual. And for PJP, he was on adequate prophylaxis. Not that that's 100%, but it certainly decreases the incidence of PJP. We didn't see it on the transbronchial biopsy. And again, it wouldn't be a perfect CT for PJP. OK, this is a quick, short answer. Which of the following underlying diseases is well described to recur in the allograft following lung transplantation? Sarcoidosis, cystic fibrosis, idiopathic pulmonary arterial hypertension, PAH, or UIP, which is well described to recur? And I don't think we touched on this this morning, but this could be something to be familiar with. All right, great. And two-thirds of you had the right answer. Sarcoidosis is the one well described to recur following transplantation. So here's a huge list of those diseases that have been described. But by far, the most common is actually sarcoidosis. We mentioned LAM, and you can see the other ones here. BAC we'll mention in just a moment. But for sarcoidosis, it's the most common. Often it's an incidental asymptomatic finding, meaning on one of your routine biopsies, either surveillance or indicated you see a granuloma, also found occasionally on autopsy. For symptomatic recurrence, you would increase your corticosteroids, as with standard sarcoid treatment. But again, it's rare to be newly symptomatic from recurrent sarcoid in the graft. When they've examined molecular studies, it's suggested if insects mismatch recipients, that the recurrent granulomas are of recipient origin. And probably the reason that we don't see more recurrence than all the other diseases listed is just that the survival of a lung transplant patient. Remember, it takes years and years for some of those diseases to develop. A word about what we used to call BAC, so now adenocarcinoma in situ. When those were transplanted, there were some long-term survivors, but there was also a lot of recurrence in the transplant recipients. And most of that was within 10 to 48 months following transplantation. Langerhans recurrence in 20%. Okay, my last question. I think timing's okay, Jack? Okay, 69-year-old woman presents with dry cough, fatigue, and shin pain. Morning headaches improved with eating. And these have been going on for about six weeks. And she has also noticed the recent onset, which she described as nail changes, but you know it as clubbing, for about eight months. No chest pain, fevers, chills, or weight loss. She does have a history of breast cancer and status post a bilateral mastectomy about 20 years prior. No diabetes, no meds. She's a never smoker. She's married to a brake mechanic for the past 40 years. Exam, lungs are clear. She has diffused shin tenderness and does have clubbing. Her CBC is normal. Her glucose is 45. There's no adenopathy on her CT. You don't have any prior imaging for comparison. So there's the imaging, but which of the following is the most likely diagnosis? Is this a solitary fibrous tumor of the pleura? Is it a loculated empyema? Is it an extra pulmonary metastasis? Is it bronchogenic carcinoma? Or is it a mesothelioma? Okay, so about half of you do have the correct answer. And we'll go through this. So this is one topic we didn't touch on yet today. So it gives me a little bit of an opportunity to review it. So this is a solitary fibrous tumor. It's rare, accounts for less than 5% of pleural tumors, incidence about 2.8 per 100,000, usually in the fifth to seventh decade. And even though we think of it as intrathoracic, actually when you look at all comers, only about a third of these are located in the thoracic cavity. But it's the ones that we almost always see. The presentation, 40 to 60% have vague symptoms of cough, shortness of breath, or chest pain. They may be asymptomatic with just an abnormality seen on radiograph in about a third of cases. And they do tend to arise from the visceral pleura in about two-thirds of cases. They're slow growing. They can have local invasion. Associated pleural effusion, as opposed to mesothelioma, where you almost always have the effusion, is rare in solitary fibrous tumors. So on imaging, you'll see a well-demarcated mass arising from the pleura, but it can also come from the fissures as well. Occasionally, you'll see it look like that. On CT, it's a well-circumscribed soft tissue density mass, usually homogenous, but sometimes there can be cystic areas or necrotic areas or hemorrhagic areas. They can have calcifications. And there are associated peroneoplastic syndromes. And this patient has one of them. So her peroneoplastic finding was hypoglycemia. Affects up to 4% of patients with solitary fibrous tumors, and it's due to IGF-2 non-islet cell tumor production. And it's sometimes called the Doge-Potter syndrome. They also, in about 20% of cases, get hypertrophic osteoarthropathy, the clubbing and the shin pain. The treatment and prognosis, there is no association with asbestos exposure. So the brake lining exposure was really a red herring here. The histology, fibroblastic soft tissue neoplasms, but they do have components of hemangiopericytomas. The treatment is usually surgical removal, and the peroneoplastic syndromes usually resolve. But because these can have low-grade malignant potential, you still have to continue with surveillance. So the others, for aloculated empyema, you'd expect signs of infection, which this patient didn't particularly have. And if it's really going to be something paranormonic, you would expect parenchymal disease, which I know I didn't show you the lung windows, but as I described, this was the only abnormality. For an extrapolymer test, I described that there was no lymphadenopathy, and you would expect that, or not 100%, but often. Such as liver, you may see effusions with it as well. For bronchogenic carcinoma, no parenchymal lesion that I described to indicate that that was what was going on. And then for mesothelioma, you almost always have an effusion. You have dyspnea. They have sometimes excruciating pain, a clear history of asbestos exposure. And the peroneoplastic syndromes are actually described, including the hypoglycemia, but less common than with the solitary fibrous tumor. So I think that was my last question, and I want to thank you, and I'm going to turn it over to Dr. Buckley, but I also want to thank Dr. Fayez, because she redid this. These were multiple slides. This is like an 80 slides that Sadia combined to, you know, basically have the whole question on one slide. So that helped a lot. Thank you. All right. Welcome. Well, welcome, everybody, for our first day. I've just got two seat questions for this afternoon. I have a couple more tomorrow and some on Tuesday as well. So just two more, and I think that's it for the day, right? Okay, great. So we'll start off. I have no conflicts of interest. All right. First question here. A 65-year-old man with idiopathic pulmonary fibrosis reports a distressing dry cough that interferes with his usual daytime activities and being out in public. He uses perfenadone and a histamine receptor antagonist. Pulmonary function tests revere a severe restriction without evidence of obstruction. He's had esophageal manometry, which has been normal. He's been benzonatate, has been tried and ineffective. So in addition to speech and language therapy interventions, which of the following agents should you be considered next to address the cough in this patient? Would it be a proton pump inhibitor, gabapentin, a low-dose opioid, or thalidomide? What would be the next agent to be considered? Okay, the vast majority of you got this answer correct. The correct answer is gabapentin. So let's go through this in some detail as to why that's the correct choice. So gabapentin is typically recommended for the treatment of chronic cough in patients with ILD when no clear cofactor has been identified as an alternative explanation for the cough. So that's why choice B is the correct answer here. So gabapentin is a neuromodulating agent that can suppress the central cough reflex. And so clinical trials have shown that in patients with IPF who have a refractory cough, gabapentin can help based on results with the Lister cough questionnaire. So it has been shown to decrease cough frequency and severity and improve quality of life. When you add a multimodality speech therapy, which include cough suppression techniques, vocal hygiene, and psychoeducational counseling to the treatment with gabapentin, you can see a decrease in your LCQ score. Unfortunately, the doses of gabapentin that are often needed are on the high side. And while you titrate up, you may see improvement of cough, but that's also when you see some of the dose-related side effects, which can include fatigue, dizziness, confusion, and dry mouth and nausea. So the cough in patients with IPF is typically described as persistent, dry, and worse during the daytime. It's been reported in more than 80% of IPF patients, more common individuals with advanced disease, and has often had a major effect on the quality of life. So treatment aimed at targeting the cofactors when they're found is important, particularly that the IPF-related cough is typically refractory to antitussive agents in the vast majority of patients. So gastroesophageal reflux disease, which is highly prevalent in IPF, is often clinically silent. Studies results, however, have failed to demonstrate a change in objective cough counts when a proton pump is added to the regimen, even at high doses. Despite showing that it can decrease the acid in the stomach. So the cough doesn't improve, but the acid content can go down. Although conditionally recommended in patients with IPF, regular antacid treatment for patients with IPF should be prescribed only for patients with a positive workup for gastroesophageal reflux. So choice A is incorrect. Opiates are indicated for unexplained and very refractory cough that has a substantial effect on quality of life after all alternative treatments have failed. So choice C was incorrect. Guidelines recommend that low-dose opiates be considered for symptomatic relief in intractable dry cough in patients with advanced IPF receiving palliative care. Thalidomide, perhaps because of its immunomodulatory properties, has been suggested as a potential agent to treat IPF-related cough on the basis of results of a small, single-center, double-blind crossover trial. Thalidomide improved cough quality of life, although an objective cough measurements were not assessed. More than three-quarters of patients experienced adverse effects attributed to the treatment, constipation, dizziness, and malaise. Although no serious side effects were reported. At this time, thalidomide cannot be considered a routine treatment for cough in IPF patients, so choice D was incorrect. All right, last question. A 65-year-old woman presents with an 18-month history of dry cough and progressive dyspnea and exertion for the past 12 months. She is a former cigarette smoker, having smoked 20 pack years. Her respiratory symptoms worsened after having an influenza infection eight months ago for which she was treated with a course of antibiotics and prednisone. Her history is significant for hypertension, hyperlipidemia, and early gray hair. Her family history is notable for her father who died from a type of pulmonary fibrosis. No more details are available. Review of systems was otherwise unremarkable. There's no environmental exposures were noted either at home or at work. Physical exam was normal except for bi-basal or crackles noted on lung examination. Laboratory testing revealed an elevated anti-nuclear antibody at 1 to 320. Serologic testing was otherwise negative. Pulmonary function tests showed a total lung capacity of 70% predicted, an FEC 70% predicted, and an FEV1 75% predicted with a diffusing capacity of 70% predicted. On a six-minute walk, she covered 1,400 feet with a room air pulse oximetry of 96% and room air pulse oximetry of 96% at rest, 94% with exertion. Which is the best initial management of this patient's condition? And here is a representative image of her lungs on CT scan. So your choices are prednisone, 40 milligrams a day, Nintedanib, 150 milligrams twice a day, azathioprine, 150 milligrams daily, or choice 4D, an urgent referral for lung transplantation. It's the next best initial management. And the vast majority of you, again, has got the answer correct. Let's go through this and why the Nintedanib choice B is the correct answer. So this patient has idiopathic pulmonary fibrosis on the basis of her higher resolution CT scan, which shows the typical usual interstitial pneumonia pattern, which is, I'm sure it was covered this morning, peripheral changes with bibasilar predominant reticulation with traction bronchiectasis as well as honeycomb fibrosis. This UIP pattern on HRCT is diagnostic of IPF if, after all the other evaluation, there's no relevant home or work exposures that could suggest hypersensitivity pneumonitis or an occupational lung disease or clinical evidence of a connective tissue disease. So in this situation, a surgical lung biopsy is unnecessary for the diagnosis and would subject the patient to increased risk of morbidity and mortality, which has been noted in patients with pulmonary fibrosis. Patients with IPF and mild to moderate impairment of their lung function, like this patient, should be offered an antifibrotic agent with either Nintedanib or profenadone, either of which has been demonstrated to slow progression in the decline of FEC by 50% over a 12-month period. This is why choice B is correct. A mildly elevated ANA, like this patient had, is common with age, so the otherwise negative serologies and unrevealing review of systems and physical exam do not support a connective tissue disease as the cause of interstitial lung disease in this patient. Her early gray hair and possible family history of interstitial lung disease suggest a short telomere syndrome may be present. This is associated with a more rapid progression of interstitial lung disease, so referral for discussion of lung transplantation is appropriate but not urgent because of the lung function shows relatively mild restriction and she has no oxygen desaturation on ambulation. So that's why choice D would be incorrect. I've already discussed why choice B is correct. The prednisone and azathioprine therapies are associated with worse outcomes in patients with IPF, including risk of mortality and hospitalization that was demonstrated in the PANTHER study, especially in the setting of short telomere disorder as is suggested by the premature gray hair and family history in this patient. So these should not be given as maintenance therapy for patients with a suspected IPF. So choices A and C are incorrect. All right, and that concludes my two questions for today. Thank you.

pulmonary diseases

rapamycin

idiopathic pulmonary fibrosis

high-resolution CT scans

lung transplantation

Lymphangioleiomyomatosis

Cytomegalovirus infections

pulmonary disease management