From the Division of Pulmonary and Critical Care (Drs Moua and Ryu), Department of Radiology (Dr Levin), Mayo Clinic Rochester.
Correspondence to: Teng Moua, MD, Division of Pulmonary and Critical Care Medicine, Gonda 18 S, Mayo Clinic, 200 First St SW, Rochester, MN 55905; e-mail: firstname.lastname@example.org
Financial/nonfinancial disclosures: The authors have reported to CHEST that no potential conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article.
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We are grateful for the thoughtful commentary by Dr Medford relating to our recent article in CHEST.1 We agree that because procedural risk in patients with idiopathic pulmonary arterial hypertension (IPAH) is high, pretest suspicion for a more ominous diagnosis is warranted prior to invasive studies of associated mediastinal lymphadenopathy (MLAD). Specifically, exclusion of malignancy (lymphoma, lung primary, metastatic primary, and so forth) should be the only immediate justification for invasive assessment in this setting.
Unfortunately, few studies have commented on MLAD associated with pulmonary hypertension. Our observed frequency of close to one in five patients with IPAH (18%) involved a selected cohort with both right-sided heart catheterization and chest CT scan for the purposes of correlating lymphadenopathy with severity of cardiac hemodynamics. This may underestimate the true prevalence of MLAD in all patients with IPAH. We did find a similar distribution and size of abnormal lymph nodes when compared with left-sided congestive adenopathy,2 and, although others have shown resolution or improvement in adenopathy following heart failure treatment,3 only one of nine patients with MLAD and follow-up CT scanning in our study had regression, despite receiving therapy. These specific findings from our study contribute to the clinical understanding of MLAD associated with pulmonary hypertension and may be applicable to MLAD with pulmonary hypertension from other causes.
As Dr Medford noted, enlarged nodes of <2 cm on short axis with known left- or right-sided heart disease may justify observation with directed heart failure management for 1 to 3 months prior to invasive assessment. This approach appears reasonable for MLAD and pulmonary hypertension from most causes. For example, pulmonary hypertension associated with sarcoidosis may be a unifying diagnosis in some patients presenting with persistent MLAD.4 PET scanning and other studies may be suggestive in this setting,5 although sarcoidosis without pulmonary parenchymal findings is unlikely to be immediately life threatening, again justifying a period of observation prior to biopsy assessment if not regressing. There is a known frequent association of MLAD with interstitial lung disease,6 and, as such, diagnostic assessment without other clinical features of malignancy is likely unjustified.
We suggest and agree with a cautious approach to MLAD associated with pulmonary hypertension of any cause, particularly if pretest suspicion for malignancy is low. Further prospective and observational work to refine a safe approach to MLAD in this setting is needed, as noted by Dr Medford.
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