1. Chatterjee S, Farver C. Severe pulmonary hypertension in anti–Jo-1 patients. Arthritis Care Res (Hoboken) 2010;62:425–9.

2. Kazerooni EA, Martinez FJ, Flint A, Jamadar DA, Gross BH,Spizarny DL, et al. Thin-section CT obtained at 10-mm incre-ments versus limited three-level thin-section CT for idiopathicpulmonary fibrosis: correlation with pathologic scoring. Am JRoentgenol 1997;169:977–83.

3. Steen V, Graham G, Conte C, Owens G, Medsger TA Jr. Isolateddiffusing capacity reduction in systemic sclerosis. ArthritisRheum 1992;35:765–70.

DOI 10.1002/acr.20391

Reply

To the Editors:

We thank Dr. Cavagna and colleagues for their com-ments regarding our recently published article on PAH inantisynthetase syndrome. We do agree that the appearanceof associated PAH should be taken into account in patientswith antisynthetase syndrome, independent of the dura-tion, disease activity, and extension of ILD. At the time ofsubmission of our case report, we were aware of only 1 otherreported case of antisynthetase syndrome that developedsevere PAH and concomitant ILD (1). That patient’s dis-ease was associated with anti–PL-12 (anti–alanyl–transferRNA synthetase) antibody production (1). Since our casereport was published, more reports highlighting this asso-ciation have been described. A series of 3 cases by Minai(2), from our institution, includes the case that we haddescribed in our case report. The other 2 cases, bothwomen with polymyositis, also had anti–Jo-1 antibodies,significant but stable ILD, and severe and progressive PAH.Like our patient, 1 other patient eventually died fromprogressive respiratory failure despite being on a combi-nation regimen of epoprostenol and sildenafil. Taniguchiet al also reported another case of severe pulmonary hy-pertension in a 62-year-old woman with anti–Jo-1 syn-drome (3). Therefore, if we include the 2 cases reported byCavagna et al, it seems likely that at least in a subset ofpatients with polymyositis (and specifically in patientswith antisynthetase syndrome), PAH tends to develop and

progress independent of the occurrence and progression ofILD.

The exact prevalence of this complication is not knownat this time. The pathophysiologic pathways and patho-genesis of this complication in antisynthetase syndromeneed exploration, and the effectiveness of the novel phar-macologic agents used for idiopathic PAH and for sclero-derma-associated PAH needs evaluation in this subset ofpatients as well. It would seem logical that if PAH contrib-utes to dyspnea in patients with antisynthetase syndrome,it would be appropriate to offer pharmacologic therapy forthe same, with the hope of providing additional symptom-atic relief and disease modification, besides the benefitderived from immunosuppressive treatment of ILD.

We are currently studying this association more system-atically among patients with polymyositis/dermatomyosi-tis (and specifically in patients with antisynthetase syn-drome). If this association is found to be more commonthan realized, it may be worthwhile to routinely evaluatepatients with antisynthetase syndrome for PAH, early inthe course of their presentation, which is similar to what isrecommended in patients with systemic sclerosis.Whether early intervention will halt or delay the progres-sion of PAH in antisynthetase syndrome remains to beseen and may be the subject of another debate.

Soumya Chatterjee, MD, MS, FRCPAshwini Mhatre, MD, MSCarol Farver, MDCleveland ClinicCleveland, OH

1. Handa T, Nagai S, Kawabata D, Nagao T, Takemura M, Kitai-chi M, et al. Long-term clinical course of a patient with antiPL-12 antibody accompanied by interstitial pneumonia andsevere pulmonary hypertension. Intern Med 2005;44:319–25.

2. Minai OA. Pulmonary hypertension in polymyositis-dermatomyositis: clinical and hemodynamic characteristicsand response to vasoactive therapy. Lupus 2009;18:1006–10.

3. Taniguchi Y, Horino T, Kato T, Terada Y. Acute pulmonaryarterial hypertension associated with anti-synthetase syn-drome [letter]. Scand J Rheumatol 2010;39:179–80.

DOI 10.1002/acr.20472

New Manuscript Submissions to Go to Dr. Hannan Starting April 1

Dr. Marian Hannan of Harvard Medical School and the Institute for Aging Research, Hebrew SeniorLifebecomes Editor of Arthritis Care & Research on July 1, 2011. A transition period will take place April 1–June 30, 2011. During this transition, review of new manuscript submissions will be handled by Dr. Hannanand her team, and pending manuscripts will continue to be handled by Drs. Katz and Yelin and their team.There will be no change in the process for submitting manuscripts, i.e., via Manuscript Central at http://mc.manuscriptcentral.com/acr.

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