Miscellaneous |

Rapidly Progressing Pulmonary Fibrosis From Adult Onset Still's Disease (AOSD) FREE TO VIEW

Quynh Hoang, MD; Rene Patino, MD; Kenneth Wei, MD
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Cedars-Sinai Medical Center, Beverly Hills, CA

Chest. 2015;148(4_MeetingAbstracts):647A. doi:10.1378/chest.2230933
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SESSION TITLE: Miscellaneous Cases

SESSION TYPE: Affiliate Case Report Slide

PRESENTED ON: Wednesday, October 28, 2015 at 11:00 AM - 12:15 PM

INTRODUCTION: Adult onset Still’s disease (AOSD) is a rare disorder characterized by fevers, arthritis and evanescent rash with multi-system involvement.

CASE PRESENTATION: EM is a 42 year old man admitted for fevers, arthralgia and weight loss over six weeks. Initial vital signs: T 101F, BP 133/82, HR 92, RR 18 and oximetry of 94% on RA. Exam noted scattered crackles. Synovitis and weakness involving MCP and PIP joints and salmon-colored rash on his trunk were seen. Lymphadenopathy, organomegaly and clubbing was absent. Laboratory evaluations revealed neutrophilic leukocytosis of 16K, AST/ALT >500, and ferritin of 4384 ng/mL. Hepatitis, HIV antibodies, ANA, RF, CCP, ANCA and Jo-1 were negative. Urinalysis was normal. Chest CT showed nodular and ground glass opacities (GGO) without honeycombing (figure 1). Bronchoscopy with bronchoalveolar lavage was unrevealing for an infectious process. Daily fevers persisted to 106F. He developed hemoptysis and worsening hypoxemia. Repeat CT showed worsening of GGO and pneumomediastinum (figure 2). In the absence of specific infectious or connective tissue disorder, the patient was diagnosed with AOSD based on the Yamaguchi criteria. Pulse-dose corticosteroid achieved rapid improvement of the synovitis and transaminitis. However, his respiratory status worsened requiring mechanical ventilation. Open lung biopsy demonstrated pulmonary fibrosis with NSIP. IVIG and Rituximab failed to achieve response. Patient remained ventilator-dependent.

DISCUSSION: AOSD is a rare, but potentially severe multi-systemic illness. Genetic or infectious triggers have been postulated. The incidence is estimated at 0.16/100,000. Pulmonary manifestation is common and seen in 30-40% of patients, with effusion or transient infiltrates being the most described. ARDS, alveolar hemorrhage and pulmonary hypertension have also been reported. Our patient had pulmonary fibrosis on histology resulting in fulminant respiratory failure. His pulmonary impairment failed to improve on treatment despite rapid improvement in other systemic manifestations. Data on pulmonary response to treatment is limited, with most being case reports or retrospective cohorts. Corticosteroid is generally first-line therapy. Prognosis associated with pulmonary involvement may be variable.

CONCLUSIONS: AOSD is a rare disease with multi-system involvement that includes fulminant respiratory failure from pulmonary involvement.

Reference #1: Magadur-Joly. Ann Rheum Dis 1995; 54:587.

Reference #2: Yamaguchi. J Rheumatol 1992; 19:424.

Reference #3: Khraishi. J Rheumatol 1991; 18:1088.

DISCLOSURE: The following authors have nothing to disclose: Quynh Hoang, Rene Patino, Kenneth Wei

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