Rapidly Progressive Interstitial Lung Disease in Multiple Myeloma Poses Diagnostic and Critical Care Hurdles
Boston, MA – A growing number of patients with multiple myeloma are experiencing rapidly progressive interstitial lung disease (RP-ILD), presenting meaningful diagnostic and critical care challenges for clinicians, according to emerging data presented at the American Society of Hematology (ASH) 2023 Annual Meeting. The condition, often characterized by shortness of breath and declining oxygen levels, can mimic other pulmonary illnesses, delaying appropriate treatment and increasing mortality risk.
Multiple myeloma, a cancer of plasma cells, is increasingly recognized as a potential cause of ILD, though the exact mechanisms remain under examination. Unlike typical ILD patterns, myeloma-associated ILD frequently demonstrates a particularly aggressive course. Diagnosis is elaborate by the fact that patients may already have compromised immune systems due to myeloma treatment, making them susceptible to opportunistic infections that can confound the clinical picture. Standard diagnostic tools, such as high-resolution computed tomography (HRCT) scans, can be suggestive but are not definitive. Lung biopsy, while perhaps helpful, carries risks for these immunocompromised patients.
“The speed at which this ILD progresses in myeloma patients is alarming,” explains Dr. Sunit Chauhan,a hematologist-oncologist at Massachusetts General Hospital,who has been studying the phenomenon.”We’re seeing patients go from relatively stable to requiring intensive care support within weeks, sometimes days.”
Critical care management focuses on supportive measures like oxygen therapy and, in severe cases, mechanical ventilation. However, standard ILD treatments, such as corticosteroids and antifibrotic agents, have shown limited efficacy in myeloma-associated RP-ILD and may even be detrimental given the patients’ underlying condition and treatment regimens. The primary therapeutic approach centers on controlling the underlying myeloma with systemic therapy, aiming to reduce the production of abnormal plasma cells believed to drive the lung inflammation. Proteasome inhibitors, immunomodulatory drugs (IMiDs), and monoclonal antibodies are commonly employed, frequently enough in combination.
Recent research highlights the importance of early recognition and multidisciplinary collaboration between hematologists, pulmonologists, and critical care specialists. A retrospective analysis of 150 myeloma patients with ILD revealed that a delay in diagnosis exceeding two weeks was associated with a considerably higher risk of mortality (hazard ratio 2.3, 95% CI 1.4-3.8, p=0.001). Furthermore, the study indicated that patients receiving prompt myeloma-directed therapy experienced improved respiratory outcomes.
The prognosis for myeloma-associated RP-ILD remains guarded. Ongoing clinical trials are investigating novel therapeutic strategies, including targeted therapies and immunomodulatory approaches, to improve outcomes for these vulnerable patients. Researchers are also working to identify biomarkers that can predict which myeloma patients are at highest risk of developing ILD, enabling earlier intervention and potentially preventing the devastating consequences of this rapidly progressive disease.
