Pulmonary Pathology Society

Mystery Case Presentations:

The following 10 cases have been carefully selected from a number of submissions on interstitial lung disease from registrants for the 2015 PPS Biennial Meeting. The cases will be presented by the submitting pathologist, at which time an expert panel will attempt to diagnose the case and discuss pertinent issues.

Submit your own answers to the mystery case presentations below. You may enter either your real name or an anonymous codename. We will compile the answers we receive and prizes will be awarded to the top 3 participants with the most correct answers.

The winners will be announced at the meeting by Dr. Mary Beth Beasley. Top prize is an additional year's membership to the PPS!

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#1 Click here to see slide
A 64 year-old Chinese female never-smoker who had immigrated to the United States 20 years prior presented with life-threatening hemoptysis. Computed tomography of the chest disclosed mediastinal and hilar lymphadenopathy, complete collapse of the right middle lobe, scattered mucous plugging with airways thickening, and patchy ground glass nodules and tree-in-bud opacities in the right upper lobe and lower lobes bilaterally. At bronchoscopy, there was patchy dark pigmentation of the bronchial mucosa, the right middle lobe bronchus was obstructed, and thrombi were seen extending from the carina into the left main bronchus. She underwent interventional radiologic-guided embolization of the left internal mammary artery and a left intercostal artery. Due to persistent hemoptysis, left pneumonectomy was performed.   K Butnor

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#2 Click here to see slide
The patient is a 52 year old Indian lady that works at the Subway restaurant in our hospital. She has had progressive shortness of breath for about two years and fibrosis on CT, more so in lower lobes with focal honeycombing. She is a never smoker, with no drug use history. Medications include benzonatate for cough and vitamins. No history of other medications.
During workup, the patient was found to have obstructive sleep apnea, occasional reflux, but no obvious rheumatologic symptoms or infectious symptoms.   She has had an esophogram that did not show reflux, but mild dysmotility. One clinic note mentions finger swelling, but clinically thought to be nonspecific. She has no other joint symptoms, rashes, and all rheumatologic workup was negative. She has been noted to have a peripheral eosinophilia on several occasions. CT scan from 3/14 showed interstitial infiltrates consistent with fibrosis, without frank honeycombing but with lower lobe predominance of fibrosis. VATS wedge biopsies of the right upper and lower lobes were performed at an outside hospital 4/14 and called consistent with UIP. CT scan from March 2015 shows traction bronchiectasis, bronchiolectasis with superimposed ground glass opacities and patulous esophagus (she was hospitalized in January with positive influenza culture and flu symptoms which have now resolved). Both CT scans show lower lobe predominant process. Focal lower lobe honeycombing is noted on the more recent CT and in the area previously biopsied in the lower lobe. A Gonzalez

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#3 Click here to see slide
This 62 year old woman presented in July 2012 with dyspnea, and imaging studies showed bilateral diffuse interstitial fibrosis with traction bronchiectasis, most severe subpleurally and in the lower lung zones. A chest x-ray from two years prior had been interpreted as normal. An open lung biopsy from August 2012 was interpreted as acute lung injury and pulmonary fibrosis. Autoimmune serologies were negative. Her disease progressed and in 2014 she underwent bilateral lung transplantation. She was a lifelong nonsmoker. She worked for Imagineering Finishing Technologies, which used blasting media to etch different materials, typically signs and plaques. Beginning in 2010 she began doing blasting jobs using plastic media. She did not use respiratory protection. J Carney & V Roggli

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#4 Click here to see slide
A 42-year old female smoker had 2 recent (within 3 months) admissions to outside hospitals for respiratory distress and bilateral ground glass opacities treated with prednisone taper. Past medical history was significant for anxiety, depression, gastroesophageal reflux disease, lower back pain, urinary tract infection, and latent tuberculosis treated with isoniazid. Patient was readmitted for a third time with worsening dyspnea and transferred to a medical center for open lung biopsy. The patient was treated with mechanical ventilation andsteroids. She improved dramatically and was discharged on post-operative day 5 on tapered oral prednisone, nicotine patch, fluoexetine, lactulose, and sulfamethoxazole-trimethoprim. A. Burke

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#5 Click here to see slide
68 year old female with reported history of pulmonary sarcoidosis (? lung biopsy – 30 years back)VATS wedge at OSH in Sept 2013 after two months of prednisone - ? Advanced sarcoidosis. Presented to local hospital in Oct 2014 with two weeks of worsening lower extremity edema and two days of shortness of breath. At OSH, chest X-ray with bilateral infiltrates ?pneumonia – Rx IV Rocephin and azithromycin. OSH echocardiogram (10/14) – LVEF 20% (decreased from 40-45% in 04/14), LVH and global hypokinesis. Referred to HUP cardiology service for management of heart failure. Allergic to hydralazine and losartan AT HUP – endomyocardial biopsy performed. C Deshpande

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#6 Click here to see slide.
65F sudden onset of acute shortness of breath, swelling in shoulders, elbows, hands and knees, joint pain, dry cough and heaviness in the chest
Spirometry: moderate restriction, TLC 2.2 liters (49% pred), DLCO 31% pred; no evidence of airflow obstruction FEV1 of 1.4 liters (63% pred) normal FEV1/FVC 83%.
Chest CT (April 2011): Bilateral predominantly lower zone peribronchial patchy areas of consolidation and ground glass densities with features of organizing pneumonia .
Started Prednisone May 2011, 35mg/day.
Chest CT (July 2011): Overall interval progression with involvement to mid lung zone, increase in peribronchial GGO, worsening septal thickening and appearance of traction bronchiectasis and architectural distortion. CT features suggestive of NSIP pattern of fibrosis with interspersed areas of OP.
Wedge Lung Biopsy (September 2011) M Gomes

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#7 Click here to see slide a Click here to see slide b
A 58 year old female complained of shortness of breath while jogging. During the next several months her symptoms gradually worsened. Oxygen saturation was normal. Chest x-ray showed reticular linear peripheral opacities predominantly involving the upper lung zones without pleural effusions or pneumothorax. EKG was normal. Pulmonary function tests were normal. Imaging studies of the chest showed pleural parenchymal opacities in both upper lung zones. E Mark

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#8 Click here to see slide
A 53-year-old man underwent right single-lung transplant for pulmonary fibrosis. His clinical history included cough, progressive dyspnea, and hypoxemia. Pre-transplant chest CT showed bilateral asymmetric coarse reticular interstitial thickening with relative sparing of the perihilar left lung. Additional features included bronchiectasis, mild ground glass opacification, and scattered cystic changes predominantly in areas of reticulation. The scanned slide represents the explanted lung. A Khoor

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#9 Click here to see slide
History: 67-year-old woman with CT imaging showed innumerable small apical nodules in random distribution. Her medical history is otherwise remarkable for 30-years of cigarette smoking. K Jones & A Urisman

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#10 Click here to see slide
A 9-year-old girl with multiple previous respiratory infections and multiple previous admissions for severe hemolytic anemia with a positive DAT test, jaundice, and chronic hypoxia has required inhaled O2 at night for the last 6 months. She now presents again with increased pallor and hypoxia with increasing O2 requirements. Thoracic CT imaging shows diffuse bilateral airspace filling / GGOs, most pronounced in the upper lobes and centrally. Pulmonary function testing shows restriction, suspicious for interstitial lung disease, prompting an open lung biopsy. B Larsen

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