Lung Pathology |

An Unusual Suspect: Hypoxemic Respiratory Failure in Antisynthetase Syndrome FREE TO VIEW

Masooma Aqeel, MBBS; Jayshil Patel, MD
Author and Funding Information

Medical College of Wisconsin, Wauwatosa, WI

Chest. 2015;148(4_MeetingAbstracts):609A. doi:10.1378/chest.2261044
Text Size: A A A
Published online


SESSION TITLE: Lung Pathology Case Report Posters

SESSION TYPE: Affiliate Case Report Poster

PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM

INTRODUCTION: Anti-synthetase syndrome is characterized by the presence of anti-Jo 1 antibodies in conjunction with clinical findings of fever, polymyositis-dermatomyositis and interstitial lung disease (ILD). Inflammatory myopathies are associated with an increased risk of malignancy, but this association is less well-outlined for patients with anti-synthetase syndrome. In fact, reports suggest the presence anti-Jo1 antibodies are protective against malignancy. We present a rare case of peripheral T-cell lymphoma presenting as hypoxemic respiratory failure in a patient with anti-synthetase syndrome

CASE PRESENTATION: A 44-year old female with anti-synthetase syndrome presented with progressively worsening dyspnea, a dry cough and developed acute hypoxemic respiratory failure. She was empirically treated with broad spectrum antibiotics for presumed health-care associated pneumonia; however, her clinical condition did not improve. Chest computed tomography (CT) revealed extensive bilateral ground glass opacities and axillary and mediastinal lymphadenopathy. Infectious work-up was negative. Surgical lung biopsy revealed peripheral T-cell lymphoma (PTCL). The patient was started on combination cyclophosphamide, doxorubicin, vincristine, and prednisone. Two days later, both hypoxemia and bilateral infiltrates resolved and the patient was successfully extubated.

DISCUSSION: Our case illustrates a unique presentation of PTCL in a patient with anti-synthetase syndrome. First, lung involvement in PTCL is exceedingly rare (8%) [1] and can be diagnostically challenging. Few case reports document lung involvement in PTCL in the form of a ‘crazy-paving pattern’ and ‘pulmonary nodules’ [2]; however, none of these patients presented with acute hypoxemic respiratory failure nor had anti-synthetase syndrome. Second, contrary to prior reports, anti-Jo 1 antibodies were not protective against malignancy in our patient, thus highlighting an important dissociation.

CONCLUSIONS: To our knowledge, ours is the first case of PTCL presenting in a patient with anti-synthetase syndrome as hypoxemic respiratory failure with bilateral multifocal lung consolidation.

Reference #1: Weisenburger, D.D., et al., Peripheral T-cell lymphoma, not otherwise specified: a report of 340 cases from the International Peripheral T-cell Lymphoma Project. Blood, 2011.

Reference #2: Vahid, B., Pulmonary manifestations of peripheral T-cell lymphoma: case report and review of the literature. Clin Respir J, 2007.

DISCLOSURE: The following authors have nothing to disclose: Masooma Aqeel, Jayshil Patel

No Product/Research Disclosure Information




Citing articles are presented as examples only. In non-demo SCM6 implementation, integration with CrossRef’s "Cited By" API will populate this tab (http://www.crossref.org/citedby.html).

Some tools below are only available to our subscribers or users with an online account.

Related Content

Customize your page view by dragging & repositioning the boxes below.

Find Similar Articles
CHEST Journal Articles
  • CHEST Journal
    Print ISSN: 0012-3692
    Online ISSN: 1931-3543