Authors: Zayan Musa (Department of Medicine, University of California at Riverside, Riverside, California, USA), Carson Tyler Moss (Department of Medicine, Stanford University School of Medicine, Stanford, California, USA), Carlos Daniel Hernandez Borges (Department of Medicine, Alta Bates Summit Health Care, Oakland, California, USA), Husham Sharifi (Department of Medicine, Division of Pulmonary, Allergy and Critical Care Medicine, Stanford University School of Medicine, Stanford, California, USA), Joe Hsu (Department of Medicine, Division of Pulmonary, Allergy and Critical Care Medicine, Stanford University School of Medicine, Stanford, California, USA)
Categories: Case Report, chronic graft‐versus‐host‐disease, haematopoietic cell transplant, home spirometry, organising pneumonia, pulmonary function tests
Source: Respirology Case Reports
Doi: 10.1002/rcr2.70607
Use of Home Spirometry for Steroid Tapering
Authors: Zayan Musa, Carson Tyler Moss, Carlos Daniel Hernandez Borges, Husham Sharifi, Joe Hsu
Abstract
Organising pneumonia (OP) is a late, non‐infectious pulmonary complication that occurs following haematopoietic cell transplant (HCT) and may be associated with chronic graft‐versus‐host‐disease (cGVHD). Treatment typically involves protracted courses of corticosteroids that are associated with dose‐limiting adverse effects and pulmonary exacerbations upon corticosteroid tapering. Here, we describe the case of a 43‐year‐old male with OP associated with cGVHD who experienced difficulty tapering prednisone, complicated by frequent OP exacerbations. Weekly home spirometry was used to closely monitor trends in pulmonary function and help successfully guide the prednisone taper. Ultimately, prednisone dose was reduced by 80% while maintaining clinical and functional stability.
Introduction
Organising pneumonia (OP) is a late, non‐infectious lung complication after haematopoietic cell transplantation (HCT) with many aetiologies including chronic graft‐versus‐host‐disease (cGVHD). OP is characterized by the proliferation of fibroblasts and granulation tissue in the alveolar spaces and results in significant morbidity and mortality [1].
A presumptive diagnosis of OP following HCT (HCT‐OP) has been suggested to include clinical, radiologic and physiologic criteria, allogeneic HCT history; concurrent or prior GVHD in any organ; chest CT findings consistent with OP; restrictive physiology on pulmonary function testing (PFT) with reduced forced vital capacity (FVC) and diffusion capacity of carbon monoxide (DLCO), but preserved typically FEV1/FVC ratio; and exclusion of infection [1]. Lung biopsy is often deferred unless in cases of diagnostic uncertainty or disease refractory to treatment. Characteristic CT findings include bilateral, asymmetric consolidation and ground‐glass opacities in a sub‐pleural and/or peribronchovascular distribution with lower lobe predominance in most cases; the reverse‐halo sign and nodules/masses occur less frequently [2].
Treatment typically includes high‐dose corticosteroids and pulmonary exacerbations can occur in up to 30%–50% of patients [1]. Initial prednisone dosing ranges from 0.5 mg/kg/day in non‐hypoxemic patients to 1–2 mg/kg/day in severe hypoxemia, with tapering by 5 mg every 2 weeks. Exacerbations often occur during tapering of corticosteroids [1]. However, long‐term, high‐dose corticosteroid treatment may be associated with adverse effects, including weight gain, hyperglycemia, osteoporosis and immunosuppression [1]. This report highlights the challenges in managing HCT‐OP, particularly, minimising the iatrogenic effects of therapy and the benefit of home spirometry to provide real‐time testing of lung function.
Case Report
A 43‐year‐old male with myelodysplastic syndrome underwent allogeneic HCT, with post‐transplantation course complicated by cGVHD affecting the mouth, eyes, gastrointestinal tract and liver. The patient presented in January 2020 with dyspnea and pulmonary ground‐glass opacities on CT scan consistent with HCT‐OP (Figure 1A). His pre‐transplant PFT in 2017 showed no evidence of abnormality; however, PFT at time of illness showed restrictive 45% percent predicted forced expiratory volume in 1 s (ppFEV1), 39% percent predicted forced vital capacity (ppFVC), 0.88 FEV1/FVC ratio, with 38% percent predicted diffusion capacity of carbon monoxide (ppDLCO). Medication review excluded drug‐induced pneumonitis, which can mimic HCT‐OP and has been associated with antimicrobials (amphotericin, nitrofurantoin), antiepileptics (carbamazepine, phenytoin), cardiovascular agents (amiodarone) and immunotherapy (bleomycin, methotrexate, cyclophosphamide) [3].
Upon diagnosis, prednisone 40 mg daily was initiated leading to improvement in dyspnoea, exercise tolerance and measures of pulmonary function (FEV1 and FVC). Treatment was complicated by steroid‐induced hyperglycemia and weight gain. Dose reduction to 30 mg coincided with increased fatigue, lip discoloration, xerosis and pleuritic chest pain. A prolonged taper to 7 mg resulted in dyspnoea and pleuritic chest pain; hospitalisation for HCT‐OP flare and escalation of prednisone to 40 mg was required. Steroid sparing agents—ruxolitinib and mycophenolate—were trialled but failed to decrease prednisone dosing or improve symptoms. In March 2022, he enrolled in a clinical trial with Axatilimab, an inhibitor of macrophage and monocyte colony‐stimulating factor‐1 receptor (CSF‐1R) [4]. Prednisone use reduced by 33% while on trial, however, was discontinued after 9 months due to infusion reactions. A progressive enlarging pulmonary nodule prompted bronchoscopy with transbronchial cryo‐biopsy, which demonstrated mononuclear cell interstitial pneumonitis with granulation tissue plugs consistent with OP; infectious studies were negative.
Over 26 months, clinic‐based PFTs proved insufficient to guide steroid tapering. Home spirometry monitoring was initiated in March 2022, with weekly testing thereafter. Decreases in home spirometry ppFEV1 were treated with nebulized budesonide to facilitate weaning of systemic corticosteroids. Throughout the home spirometry directed taper, one escalation of prednisone to 25 mg occurred due to infections with rhinovirus and Influenza A in December 2022. By November 2023, despite reducing prednisone to 10 mg and discontinuation of nebulized budesonide, he demonstrated improving exercise tolerance, FVC and FEV1. A CT chest scan at this time showed near complete radiologic resolution of HCT‐OP (Figure 1B). As of March 2026, the dose was further reduced to 7 mg, while preserving disease stability (Figure 1C).
Discussion
To our knowledge, this is the first report of home spirometry used in the management of HCT‐OP. Prior to home spirometry, the patient was monitored via exercise capacity, oxygen saturation and symptoms (e.g., dyspnoea, pleuritic chest pain). These measures lacked sensitivity for timely intervention, as significant symptoms often emerged only after disease had progressed to require hospitalisation. Spirometry provides objective data that can help guide therapies, however, formal testing is limited by infrequent measurements, patient burden and facility capabilities. In other conditions such as sarcoidosis, home spirometry has facilitated personalised prednisone treatment plans [5].
Handheld, wireless devices enable remote surveillance and early detection of early pulmonary complications in high‐risk populations. This modality has been shown to closely correlate with formal spirometry studies in asthma, cystic fibrosis and chronic obstructive pulmonary disease [6].
Although some systems underpredict FEV1, its use allows for close monitoring of pulmonary trajectory, thereby detecting early lung disease and improving health outcomes [7]. Following HCT, it has been shown to be reliable for following cGVHD patients with bronchiolitis obliterans syndrome (BOS) (Table 1) [7, 8, 9, 10, 11]. Currently, the National Institutes of Health recommends spirometry every 3 months after the diagnosis of cGVHD for up to 2 years [12], yet this strategy may be too infrequent to detect pre‐clinical changes in flares of OP. As there are no current publications that describe the use of home spirometry for HCT‐OP, further investigation is needed to validate its use as an effective tool for exacerbation monitoring. Our case highlights handheld spirometry as a feasible, accessible adjunct to lab‐based pulmonary testing.
Author Contributions
Z.M., C.T.M., C.H.B, H.S. and J.L.H. contributed to the design and implementation of the research, to the analysis of the results and to the writing of the manuscript. Z.M. and C.T.M. are in medical school and residency in different institutions but work as research staff at Stanford in the Hsu Lab.
Funding
J.L.H. is funded by NIH/NHLBI R01HL157414 and NIH/NCAT R21TR005507.
Consent
The authors declare that written informed consent was obtained for the publication of this manuscript and accompanying images and attest that the form used to obtain consent from the patient complies with the Journal requirements as outlined in the author guidelines.
Conflicts of Interest
The authors declare no conflicts of interest.