December 2023 Pulmonary Case of the Month: A Budding Pneumonia

Ishan A. Patel, MD¹

Sarah Medrek, MD¹

Michael Reyes, MD²

Brannon Raney, MD³

Section of ¹Pulmonary, Critical Care, and Sleep Medicine, ²Pathology, and ³Infectious Disease

VA Albuquerque Health System

Albuquerque, NM USA

History of Present Illness

A 70-year-old man with a history of seropositive rheumatoid arthritis previously well controlled on hydroxychloroquine, methotrexate, and adalimumab was admitted to the hospital with 3 weeks of progressively worsening fatigue, night sweats, chills, and malaise. He did not describe new or worsening cough, shortness of breath, or sputum production. On the day of admission, he had intense nausea and vomiting.

PMH, SH, and FH

Prior to this admission, he was followed in Pulmonary Clinic for asymptomatic mild basilar fibrosis thought to be related to his rheumatoid arthritis and paraseptal emphysema related to prior smoking which was largely stable and unchanged over the previous two years. Previously, he smoked cigarettes at ½ pack per day for about 30 years and quit about 15 years ago. He denied any recent travel and was retired from the last 15 years from being a meat butcher. FH is noncontributory.

Physical Examination

On examination the day after admission from the ER, the patient’s temperature was 37.6C. His pulse was 79 bpm, blood pressure was 142/65 mmHg, and pulse oximetry revealed a saturation of 92% with 2 LPM nasal cannula of O2. He appeared generally weak, but alert. Pulmonary exam was unrevealing as was cardiac exam. He did not have cyanosis, clubbing, delayed capillary refill, or peripheral edema.

Laboratory

Initial blood work showed a WBC count of 7500/µL, hemoglobin level of 9.6 gm/dl, serum blood urea nitrogen of 36 gm/dl, serum creatinine of 2.49 g/dl, and serum calcium that was elevated at 12.3 mg/dl. A T-spot was obtained and was negative. Blood and sputum cultures were obtained and negative.

Radiography

Figure 1. Admission portable chest x-ray in the emergency department. To view Figure 1 in an enlarged, separate window click here.

The patient has a history of rheumatoid arthritis (RA). Which of the following patterns of interstitial lung disease (ILD) is most common in patients with RA? (Click on the correct answer to be directed to the second of seven pages)

1. Acute eosinophilic pneumonia
2. Lymphocytic interstitial pneumonitis
3. Non-specific interstitial pneumonia
4. Organizing pneumonitis
5. Usual interstitial pneumonitis

Cite as: Patel IA, Medrek SK, Reyes MD, Raney BL. December 2023 Pulmonary Case of the Month: A Budding Pneumonia. Southwest J Pulm Crit Care Sleep. 2023;27(6):62-66. doi: https://doi.org/10.13175/swjpccs048-23 PDF

September 2023 Pulmonary Case of the Month: A Bone to Pick

Lewis J. Wesselius MD

Pulmonary Department

Mayo Clinic Arizona

Scottsdale, AZ USA

History of Present Illness

A 56-year-old man presented acute onset of shortness of breath. He denied cough, fever or other symptoms

Past Medical History, Family History and Social History

• Occasional gout
• No relevant family history
• Never smoked

Medications

• Allopurinol
• Multivitamin

Physical Examination

• Other than tachypnea and mild shortness of breath, no significant abnormalities.

Chest X-ray

An AP chest X-ray was performed (Figure 1).

Figure 1. Admission chest X-ray.

Which abnormality is suggested  by the chest X-ray? (Click on the correct answer to the second of seven pages)

1. Calcified micronodules in the right lung
2. Retained secretions with atelectasis left lung
3. Right pneumothorax
4. 1 and 3
5. None. The chest X-ray is within normal limits.

Cite as: Wesselius LJ. September 2023 Pulmonary Case of the Month: A Bone to Pick. Southwest J Pulm Crit Care Sleep. 2023;27(3):26-29. doi: https://doi.org/10.13175/swjpccs037-23 PDF

A Case of Progressive Bleomycin Lung Toxicity Refractory to Steroid Therapy

Christopher S Dossett MD¹, Kelli Kosako Yost MD¹, Christopher Lau MD², Nafis Shamsid-Deen MD²

¹Department of Internal Medicine, University of Arizona – Phoenix

²Department of Pulmonary and Critical Care Medicine, University of Arizona – Phoenix

Address: 475 N. 5th Street, Phoenix, Arizona, United States of America

Abstract

Bleomycin is a common chemotherapy agent used to treat germinative tumors. Bleomycin-induced lung injury (BILI) is an uncommon but devastating adverse effect of its use. It occurs in 10-20% of patients receiving bleomycin, and the initial diagnosis is usually made by new-onset respiratory symptoms and reduced diffusing capacity for carbon monoxide (DLCO). Mainstay treatment includes discontinuing bleomycin, corticosteroids, and supplemental oxygen if needed. We present a case of a 38-year-old male who was found to have a severe presentation of bleomycin-induced lung injury after chemotherapy for metastatic mixed germ cell testicular cancer. During his course, he was treated with the standard of care regimen of corticosteroids and salvage therapy with infliximab but ultimately died from complications of his illness. This case report is noteworthy because our patient had progressive bleomycin-induced lung injury, despite discontinuing bleomycin many months prior, consistent high-dose corticosteroid treatment, and even salvage therapy. In all patients on bleomycin, pulmonary function monitoring is essential, and any complaints of dyspnea should prompt concern for bleomycin-induced lung injury.  If initial treatment does not improve their condition, more aggressive measures may be necessary.

Abbreviations

• ARDS - acute respiratory distress syndrome
• BILI - bleomycin-induced lung injury
• CT - computed tomography
• DLCO - diffusing capacity for carbon monoxide
• ECMO - extracorporeal membrane oxygenation
• FDA - Food and Drug Administration
• IU - international units
• PFT - pulmonary function tests
• ROS - reactive oxygen species

Introduction

Bleomycin is an antibiotic used to treat germinative tumors and Hodgkin’s lymphoma. The major limitation of bleomycin therapy is pulmonary toxicity, which occurs in up to 10-20% of patients receiving the drug, with mortality up to 1-2% (1). The primary mechanism is not entirely understood but is thought to be induced by the generation of reactive oxygen species (ROS) that form free radical oxidants (2). When type I pneumocytes experience oxidation from free radicals, they undergo apoptosis. This release of cellular contents can lead to the activation of neutrophils and pulmonary macrophages. These cells release cytokines and chemokines, which attract more inflammatory cells, amplifying the immune response. This ultimately disrupts the alveolar-capillary interface, causing capillary leak. This inflammation stimulates fibroblasts resulting in collagen deposits and irreversible pulmonary fibrosis.

The mainstay treatment of bleomycin-induced lung injury (BILI) involves discontinuing the medication and initiating corticosteroids to reduce inflammation (3). There has been limited updated evidence on managing BILI since White and Stover (3) in 1984 noted clinical improvement with corticosteroids. Only case series and reports have provided additional clinical experience on the efficacy of this treatment (4-7). As corticosteroids are helpful in acute BILI, patients with more indolent disease may benefit less. Recent case reports have trailed off-label therapies, including tumor necrosis alpha inhibitors, tyrosine kinase inhibitors, and antifibrotics, as potential treatment options with mixed results (8-12). Despite this well-known adverse effect of bleomycin, minimal evidence-based changes have been made in managing BILI, especially when refractory to corticosteroids. We present a case of a patient who developed rapidly progressive bleomycin-induced lung injury despite discontinuing bleomycin, initiation of high-dose corticosteroids, and salvage infliximab therapy.

Case Presentation

A 38-year-old man with a 10-pack-year tobacco use history and metastatic mixed germ cell testicular cancer undergoing bleomycin, etoposide, and cisplatin chemotherapy, with his last treatment a month prior, presented to a nearby emergency department with shortness of breath. He had completed four chemotherapy treatment cycles initiated three months earlier for a combined bleomycin dose of 330,000 IU (330 milligrams). Baseline pulmonary function test (PFT) before initiation of bleomycin showed a normal diffusing capacity for carbon monoxide (DLCO) at 90% of predicted. In the emergency department, he was found to be in respiratory failure with new onset ground-glass opacifications throughout bilateral lung fields by computed tomography (CT) angiogram (Figure 1).

Figure 1. Initial presentation CT pulmonary angiogram demonstrating ground-glass opacities present throughout both lungs. To view Figure 1 in a separate, enlarged window click here.

He denied using any vaping products. Infectious work-up was negative for SARS-CoV-2, influenza, and coccidiosis. He was treated for community-acquired pneumonia with an initial improvement of his respiratory failure and discharged a few days later on ambient air.

Despite oral antibiotic therapy and stopping cigarette use after discharge, the patient’s dyspnea and cough recurred less than a week after hospitalization. Repeat PFTs demonstrated new findings of reduced DLCO at 31% of predicted. Bleomycin was discontinued from his chemotherapy regimen due to concern of BILI. He was started on daily prednisone 60 mg, or approximately 1 mg/kg. He was tapered to 40 mg of prednisone daily over four weeks, but due to worsening dyspnea symptoms it had to be increased to 50 mg daily.

The patient re-presented to the emergency department one month after his initial hospitalization for acute on chronic shortness of breath and a persistent cough. Between these hospitalizations, the patient had not received etoposide or cisplatin treatment. His heart rate was 111 beats per minute, his respiratory rate was 16 breaths per minute, and his oxygen saturation was 95% at ambient air. Laboratory data was mainly unremarkable, except for a white blood cell count of 18.4 K/uL with neutrophilic predominance at 16.23 K/uL, hemoglobin 10.7 g/dL with an MCV of 103 fL, and C-reactive protein of 38.1 mg/L. A CT pulmonary angiogram demonstrated worsening interstitial and airspace opacities (Figure 2).

Figure 2. CT pulmonary angiogram shows significant interstitial and airspace opacity progression throughout the lungs. To view Figure 2 in a separate, enlarged window click here.

He was admitted and treated with broad-spectrum antibiotics due to concern of recurrent pneumonia, as he was not on antibiotic prophylaxis with his chronic steroids. The patient was resumed on his outpatient dose of oral prednisone 50 mg daily. Infectious work-up including blood, sputum, and fungal cultures, legionella antibodies, streptococcus pneumonia urinary antigen, mycoplasma antibodies, aspergillus, fungitell, coccidioides serologies, HIV, and viral etiologies including SARS-CoV-2, influenza, and cytomegalovirus were all unremarkable. Due to a broad negative infectious work-up, BILI was highly thought to be the original diagnosis. He was switched to intravenous methylprednisolone 60 mg every 12 hours for more aggressive BILI treatment.

Two days after admission, he became acutely dyspneic. A repeat chest radiographic demonstrated continued bilateral airspace opacities with new moderate to large right apical pneumothorax. He underwent CT-guided right thoracostomy tube placement for the new pneumothorax. His respiratory status deteriorated over the next five days requiring endotracheal intubation. Bronchoalveolar lavage performed during intubation was unremarkable for infectious etiologies or malignant cells. Due to continued deterioration, his methylprednisolone was increased to 250 mg every 6 hours. Six days after intubation, he had minimal improvement, so salvage therapy with 300 mg of infliximab was initiated. The patient had worsening oxygenation despite mechanical ventilation. Ventilation strategies were also limited due to high peak inspiratory pressures.

Repeat CT chest without contrast demonstrated worsening extensive interstitial and airspace opacities throughout bilateral lungs (Figure 3).

Figure 3. CT chest without contrast after an acute respiratory decompensation demonstrated persistent interstitial and airspace opacities throughout bilateral lungs, significantly worse than the prior CT. To view Figure 3 in a separate, enlarged window click here.

With minimal improvement in his respiratory failure, the patient was transferred to a university hospital to initiate inhaled prostacyclin therapy in an attempt to improve oxygenation and ventilation. He was started on inhaled epoprostenol and cisatracurium infusion. Despite these measures, the patient had no improvement in his respiratory failure with persistent extensive interstitial and airspace opacities throughout bilateral lungs on repeat CT (Figure 4).

Figure 4. CT pulmonary angiogram showing persistent extensive bilateral ground-glass opacities, scattered consolidative opacities, bronchiectasis, and new pneumomediastinum. To view Figure 4 in a separate, enlarged window click here.

Extracorporeal membrane oxygenation (ECMO) was considered, but he was deemed not an appropriate candidate given the irreversible lung injury. With all avenues for recovery exhausted, the poor prognosis was discussed with the family, who decided to transition to comfort-only care. He expired shortly after cessation of aggressive life support measures.

Discussion

Bleomycin-induced lung injury (BILI) is thought to be due to the development of pulmonary fibrosis, characterized by enhanced production and deposition of collagen and other matrix components (1). Pulmonary toxicity is dose-dependent, with most of these injuries occurring with doses above 400,000 IU. Other risk factors include kidney dysfunction, older age, supplemental oxygen exposure, bolus delivery of infusion, extent of lung metastases, and established lung disease (13). Symptoms and signs include nonproductive cough, dyspnea, pleuritic or substernal chest pain, fever, tachypnea, crackles, lung restriction, and hypoxemia. Clinical manifestations usually develop indolently between one and six months after treatment initiation, but they may persist more than six months after treatment discontinuation. The earliest manifestation of BILI is dyspnea with a reduction in the DLCO (14-15). Best-practice clinical guidelines and the U.S. Food and Drug Administration (FDA) recommend PFTs at baseline and monthly or after each new treatment cycle (16). A DLCO reduction of more than 30-35% should prompt providers to discontinue bleomycin, even if asymptomatic, due to the concern of BILI. However, a recent randomized phase III trial demonstrated that the presence of cough had a higher association with BILI than PFT changes, questioning the benefit of routine PFTs (17).

BILI treatment involves prompt discontinuation of all chemotherapeutic agents. Corticosteroids are given to patients with symptomatic lung toxicity. The suggested prednisone dosing is 0.75 to 1 mg/kg (based on ideal body weight) per day, to a maximum of 100 mg/day, for the first four to six weeks based on clinical data and case reports (3-7). Clinical and radiographic improvement varies by report from 7 to 12 days after early initiation of high-dose corticosteroid therapy (6). There have been two fatal cases of BILI that were attributed to insufficient corticosteroid doses (18). This emphasizes the need for a higher dose to treat the condition effectively. Most patients respond after treatment with limited case reports discussing corticosteroid refractory BILI. These cases have led to the evaluation of off-label therapies as potential treatments. Recent case reports have described imatinib, infliximab, and pirfenidone to have variable success in treating BILI, including those cases refractory to corticosteroids; however, these require long treatment durations for clinical success (8-12).

Etoposide and bleomycin can both cause lung injury; however, the two drugs' mechanisms of injury and clinical presentations can differ (19). Etoposide-induced lung injury typically presents as acute respiratory distress syndrome (ARDS) within hours to days of exposure. In contrast, BILI typically presents as a more gradual onset of pulmonary fibrosis, which can occur weeks to months after exposure. Our patient's clinical course was indolent after discontinuing his chemotherapeutics which is more consistent with a BILI presentation. However, it is difficult to say with the utmost certainty that our patient’s lung injury was not worsened by etoposide.

We present an unusual case of corticosteroid refractory BILI in a young patient with minimal tobacco history and no end-organ dysfunctions. Given the enormous respiratory reserve, most young, healthy patients will develop symptoms only after a severe reduction in diffusion. Our patient did not have the recommended interval PFT monitoring described by clinical guidelines and the FDA. This highlights the importance of interval monitoring, including symptomatic tracking, especially in young patients, in the hopes of early diagnosis of BILI. As this disease usually progresses indolently, monthly PFTs can capture the subtle advancement of lung injury in younger patients. It is uncertain when the initial lung injury began in our patient due to a lack of PFT monitoring during the four treatment cycles of bleomycin. However, if changes had been detected, earlier management could have been implemented, such as earlier discontinuation of chemotherapy, increasing corticosteroids, or off-label therapies.

This case also accentuates the limited data on off-label treatment options for corticosteroid refractory BILI. Our patient developed progressive pulmonary fibrosis, ultimately leading to his demise. Although he received infliximab as salvage therapy, it is improbable that this treatment would have had benefit due to the late fibrosing stage of his disease presentation. Universally, an immunomodulatory agent’s efficacy wanes dramatically once in the terminal fibrosing stages of many interstitial lung diseases, reiterating the need for early diagnosis and aggressive treatment during the inflammatory phase (20). If our patient had been identified sooner as refractory to corticosteroids, prompter introduction of second-line agents might have resulted in an alternative clinical outcome. Maximizing medical management in this patient population is particularly critical given that other salvage treatments like ECMO and lung transplantation are not recommended and are usually contraindicated. Additional prospective investigation in refractory disease is necessary to better validate and quantify the therapeutic efficacy of available second-line and off-line medical therapies.

Conclusion

Patients on bleomycin therapy are at risk of developing BILI associated dyspnea that may present as progressive pulmonary fibrosis, hypersensitivity pneumonitis, or organizing pneumonia. If a patient treated with bleomycin continues to have unremitting shortness of breath, the concern for BILI should be high and may warrant earlier evaluation and intervention.

Acknowledgments

Christopher S Dossett, Kelli Kosako Yost, Christopher Lau, and Nafis Shamsid-Deen contributed to the drafting and revising of this manuscript. The authors have no conflict of interest. All authors have consented to the approval of this manuscript.

References

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2. Hay J, Shahzeidi S, Laurent G. Mechanisms of bleomycin-induced lung damage. Arch Toxicol. 1991;65(2):81-94. [CrossRef] [PubMed]
3. White DA, Stover DE. Severe bleomycin-induced pneumonitis. Clinical features and response to corticosteroids. Chest. 1984 Nov;86(5):723-8. [CrossRef] [PubMed]
4. Ghalamkari M, Khatuni M, Toogeh G, Haghighi S, Taherkhani M. Reversible Acute Lung Injury due to Bleomycin. Tanaffos. 2022 Feb;21(2):253-256. [PubMed]
5. Rashid RS. Bleomycin lung: a case report. BMJ Case Rep. 2009;2009:bcr11.2008.1175. [CrossRef] [PubMed]
6. Gupta R, Ettinger NA. Beyond conventional therapy: role of pulse steroids in bleomycin induced lung injury. Respir Care. 2014 Jan;59(1):e9-e12. [CrossRef] [PubMed]
7. Wang, X, Deng, J, Sothwal, A, Gordon, E, Patel, G. Bleomycin-Induced Pneumonitis Responds To Super-High-Dose Steroid and Monitored By LDH and PAO2/FIO2.  Critical Care Medicine 2016;44(12):558. [CrossRef]
8. Banakh I, Lam A, Tiruvoipati R, Carney I, Botha J. Imatinib for bleomycin induced pulmonary toxicity: a case report and evidence-base review. Clin Case Rep. 2016 Apr 1;4(5):486-90. [CrossRef] [PubMed]
9. Ge V, Banakh I, Tiruvoipati R, Haji K. Bleomycin-induced pulmonary toxicity and treatment with infliximab: A case report. Clin Case Rep. 2018 Sep 4;6(10):2011-2014. [CrossRef] [PubMed]
10. Carnevale-Schianca F, Gallo S, Rota-Scalabrini D, Sangiolo D, Fizzotti M, Caravelli D, Capaldi A, Anselmetti G, Palesandro E, D'Ambrosio L, Coha V, Obert R, Aglietta M, Grignani G. Complete resolution of life-threatening bleomycin-induced pneumonitis after treatment with imatinib mesylate in a patient with Hodgkin's lymphoma: hope for severe chemotherapy-induced toxicity? J Clin Oncol. 2011 Aug 20;29(24):e691-3. [CrossRef] [PubMed]
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13. Comis RL. Bleomycin pulmonary toxicity: current status and future directions. Semin Oncol. 1992 Apr;19(2 Suppl 5):64-70. [PubMed]
14. Lucraft HH, Wilkinson PM, Stretton TB, Read G. Role of pulmonary function tests in the prevention of bleomycin pulmonary toxicity during chemotherapy for metastatic testicular teratoma. Eur J Cancer Clin Oncol. 1982 Feb;18(2):133-9. [CrossRef] [PubMed]
15. Nippon Kayaku Co., Ltd. Blenoxane (bleomycin sulfate) [package insert]. U.S. Food and Drug Administration website. https://www.accessdata.fda.gov/drugsatfda_docs/label/2010/050443s036lbl.pdf. Revised April 2010. Accessed April 15, 2023.
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17. Shamash J, Sarker SJ, Huddart R, et al. A randomized phase III study of 72 h infusional versus bolus bleomycin in BEP (bleomycin, etoposide and cisplatin) chemotherapy to treat IGCCCG good prognosis metastatic germ cell tumours (TE-3). Ann Oncol. 2017 Jun 1;28(6):1333-1338. [CrossRef] [PubMed]
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Cite as: Dossett CS, Yost KK, Lau C, Shamsid-Deen N. A case of progressive bleomycin lung toxicity refractory to steroid therapy. Southwest J Pulm Crit Care Sleep. 2023;26(6):90-96. doi: https://doi.org/10.13175/swjpccs013-23 PDF

June 2023 Pulmonary Case of the Month: An Invisible Disease

Lewis J. Wesselius MD

Pulmonary Department

Mayo Clinic Arizona

Scottsdale, AZ USA

History of Present Illness

A 78-year-old man presented to the Emergency Department on April 7 for shortness of breath and weakness over the last 2 weeks. He was in good health prior to an outside hospitalization March 29-April 3 for pneumonia and a possible non-ST-elevation myocardial infarction (elevated troponins). He had a bronchoscopy during his recent outside hospitalization without specific pathogen identified but was treated with antibiotics and discharged on levofloxacin. Since his hospital discharge 4 days previously he feels weaker and increasingly short of breath. He is short of breath even walking around his home. He denies fever or a productive cough.

Past Medical History, Family History and Social History

• Atrial fibrillation, s/p ablation. On Eliquis.
• Prior renal cell carcinoma, s/p resection, no recurrence
• DM Type 2
• GERD
• OSA
• Essential tremor
• Never smoked

Medications

• Apixaban
• Aspirin
• Atorvastatin
• Flecanide
• Insulin
• Levofloxacin
• Lisinopril
• Pantoprazole
• Tamsulosin

Physical Examination

• General: The patient looks comfortable and is in no distress
• Vital Signs: BP 110/62 O2 Sat 94% on room air
• CVS: Heart sounds are regular
• Lungs: Clear to auscultation
• Abdomen: Soft, nontender, bowel sounds present
• Extremities: No edema
• Neuro: Alert and oriented
• Skin: Warm and dry, no rashes

Chest X-ray

A portable chest X-ray was performed (Figure 1).

Figure 1. Portable chest X-ray obtained in the emergency department.

Which of the following should be done next? Click on the correct answer to be directed to the second of six pages)

1. Arterial blood gases
2. Bronchoscopy
3. Thoracic CT scan
4. 1 and 3
5. All of the above

Cite as: Wesselius LJ. June 2023 Pulmonary Case of the Month: An Invisible Disease. Southwest J Pulm Crit Care Sleep. 2023;26(6):83-86. doi: https://doi.org/10.13175/swjpccs022-23 PDF

February 2023 Pulmonary Case of the Month: SCID-ing to a Diagnosis

Lewis J. Wesselius MD

Pulmonary Department

Mayo Clinic Arizona

Scottsdale, AZ USA

History of Present Illness

A 40-year-old man was referred for management of respiratory symptoms of cough, sputum production and shortness of breath. He has a history of respiratory infections that began in early childhood. Sputum cultures were positive for Pseudomonas. He is currently using oxygen at night and occasionally during the day.

Past Medical History, Family History and Social History

• Childhood diagnosis of asthma.
• Multiple colds and pneumonias in the past.
• No family history of a similar problem.
• He has never smoked.
• Denies any occupational exposure.

Physical Examination

• Vital Signs: O2 Sat 88% on RA
• Chest: diminished breath sounds, no wheezes
• Heart:  regular rate and rhythm without murmur
• Extremities: mild clubbing present, no edema

Pulmonary Function Testing

Pulmonary function testing (PFTs) was performed with results as below (Figure 1).

Figure 1. Pulmonary function testing.

Thoracic CT Scan

A thoracic CT was performed (Figure 2).

Figure 2. Representative images from the thoracic CT in lung windows (A-C) and soft tissue windows (D). To view Figure 2 in a separate enlarged window click here

Which of the following is/are true? (Click on the correct answer to be directed to the second of six pages)

1. PFTs show severe obstructive disease
2. The thoracic CT shows a normal mediastinum
3. Bronchiectasis is shown in the CT scan lung windows
4. 1 and 3
5. All of the above

Cite as: Wesselius LJ. February 2023 Pulmonary Case of the Month: SCID-ing to a Diagnosis. Southwest J Pulm Crit Care Sleep. 2023;26(2):18-20. doi: https://doi.org/10.13175/swjpccs005-23 PDF