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Case Reports and Clinical Images

Dyspnea in a Patient with Asthma, Interstitial Lung Disease, and Dermatomyositis

Siu Yu Zoe Lau, MD*, Lawrence Jacobs, MD, FRCPC

The University of Western Ontario, London, Ontario, Canada

Abstract

We describe the case of a 32-year-old woman with interstitial lung disease (ILD) secondary to dermatomyositis and severe asthma who presented with 2 months of progressive dyspnea and hypoxemia while on rituximab, prednisone, and omalizumab. No clear etiology of her worsening symptoms was identified on previous presentations to care. Repeat CT chest revealed bilateral cystic lung changes and ground glass opacities. She was found to have Pneumocystis jirovecii pneumonia (PJP). This case highlights PJP as an important cause of dyspnea to consider in immunosuppressed patients with these radiographic findings.

Résumé

Nous décrivons le cas d’une femme de 32 ans atteinte d’une pneumopathie interstitielle consécutive à une dermatomyosite et à un asthme grave et qui présente une dyspnée et une hypoxémie progressives depuis deux mois. Elle prend du rituximab, de la prednisone et de l’omalizumab. Aucune étiologie claire de l’aggravation de ses symptômes n’a été cernée lors de ses visites précédentes. Une nouvelle tomodensitométrie des poumons révèle des modifications kystiques bilatérales des poumons et des opacités en verre dépoli. Il s’avère qu’elle est atteinte d’une pneumonie à Pneumocystis jirovecii (PPJ). Ce cas met en évidence la PPJ comme étant une cause importante de dyspnée à considérer chez les patients immunodéprimés présentant de tels résultats radiographiques.

Corresponding Author: Siu Yu Zoe Lau: slau93@uwo.ca

Submitted: 10 December 2020; Accepted: 1 May 2021; Published: 23 December 2021

Doi: http://dx.doi.org/10.22374/cjgim.v16i4.505

All articles published in DPG Open Access journals
This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 International (CC BY-NC 4.0)(https://creativecommons.org/licenses/by-nc/4.0/).

Case Presentation

A 32-year-old woman with severe asthma and dermatomyositis-associated interstitial lung disease (ILD) presented to hospital with 2 months of progressive dyspnea and hypoxemia requiring new home oxygen. She also had a worsening nonproductive cough. She had presented to care multiple times and despite escalating doses of prednisone, she showed no improvement. Other notable medications included rituximab and omalizumab.

On presentation, her oxygen saturation was 74% on 2L of oxygen via nasal prongs. She required 3–4 L of oxygen to maintain saturations above 90%. Her exam showed mild respiratory distress, bilateral fine crackles of her lungs, and Gottron’s papules. Chest x-ray demonstrated bilateral hilar opacities. CTPA was negative for pulmonary embolism but showed an increase in ground-glass opacification when compared to prior and new small cysts throughout both lung fields (Figure 1).

Figure 1. CTPA showing fine and diffuse ground-glass opacities, more predominant in upper lung zones, and small cysts throughout the lung parenchyma bilaterally.

Given the patient’s immunosuppression and radiographic findings, we considered Pneumocystis jirovecii pneumonia (PJP). Previous attempts at PJP chemoprophylaxis were unsuccessful as she developed a rash with trimethoprim-sulfamethoxazole (TMP-SMX) and methemoglobinemia with dapsone. Lymphangioleiomyomatosis, an uncommon condition that predominantly affects females and also causes cystic lung destruction, was another differential diagnosis. She was admitted under Internal Medicine and treated with atovaquone and methylprednisolone empirically for PJP. Bronchoscopy ultimately identified Pneumocystis jirovecii. With treatment, her dyspnea improved. At post-discharge follow-up, she no longer required supplemental oxygen.

This case highlights the importance of considering PJP as a cause of dyspnea in immunosuppressed patients. Outcomes for PJP are worse in human immune deficiency (HIV)-negative patients than HIV-positive patients,1 with a 32% mortality rate in patients with autoimmune diseases.2 First-line treatment for PJP is TMP-SMX.3 For allergic patients, primaquine plus clindamycin or atovaquone alone are second-line options. Intravenous pentamidine is another alternative, but the aerosolized form is rarely used. Evidence for adjunctive corticosteroids is limited to patients with HIV.

In terms of PJP prophylaxis, TMP-SMX is also first line.3 Second-line agents are dapsone, which requires screening for glucose-6-phosphate dehydrogenase deficiency,4 and atovaquone. The last option is aerosolized pentamidine.3 No consensus guidelines exist for PJP prophylaxis in rheumatologic diseases; however, PJP occurs almost exclusively in patients not on TMP-SMX prophylaxis.1 Patients with connective tissue diseases are at risk of opportunistic infections both from disease-related immune dysregulation and from immunosuppressive therapies.5 Clinicians should thus be cognisant of the prophylaxis options to help protect patients from this severe respiratory infection.

Consent

The patient provided informed consent for the publication of this report.

Statement of Contributions

All authors contributed to the conception, design, drafting, and reviewing of the original manuscript.

Conflict of Interest

None.

Funding

None.

REFERENCES

1. Schmidt JJ, Lueck C, Ziesing S, et al. Clinical course, treatment and outcome of Pneumocystis pneumonia in immunocompromised adults: a retrospective analysis over 17 years. Crit Care. 2018;22:307. 10.1186/s13054-018-2221-8

2. Chen M, Tian X, Qin F, et al. Pneumocystis pneumonia in patients with autoimmune diseases: a retrospective study focused on clinical characteristics and prognostic factors related to death. PLoS One. 2015;10(9):e0139144. 10.1371/journal.pone.0139144

3. Limper AH, Knox KS, Sarosi GA, et al. An official American Thoracic Society Statement: treatment of fungal infections in adult and pulmonary and critical care patients. Am J Resp Crit Care Med. 2011;183(1):96–128. 10.1164/rccm.2008-740ST

4. Lee SM, Geetha D. Dapsone induced hemolysis in a patient with ANCA associated glomerulonephritis and normal G6PD level and implications for clinical practice: case report and review of the literature. SpringerPlus. 2015(4):29. 10.1186/s40064-015-0816-y

5. Braga BP, Prieto-González S, Hernández-Rodríguez J. Pneumocystis jirovecii pneumonia prophylaxis in immunocompromised patients with systemic autoimmune diseases. Med Clin (Barc). 2019;152(12):502–7. 10.1016/j.medcli.2019.01.010