Pulmonary Manifestations in Microscopic Polyangiitis and Granulomatosis with Polyangiitis: A Multicenter Cohort Analysis

Saara M. Rawn¹, Gerard Cox¹, Christian Pagnoux², David Cuthbertson³, Simon Carette², Curry L. Koening⁴, Carol A. Langford⁵, Carol A. McAlear⁶, Paul A. Monach⁷, Larry W. Moreland⁸, Philip Seo⁹, Ulrich Specks¹⁰, Antoine G. Sreih¹¹, Steven R. Ytterberg¹², Renee Borchin¹³, Peter A. Merkel¹¹ and Nader A. Khalidi¹, ¹St. Joseph's Healthcare, McMaster University, Hamilton, ON, Canada, ²Division of Rheumatology, Mount Sinai Hospital, University of Toronto, Toronto, ON, Canada, ³Department of Biostatistics, University of South Florida, Tampa, FL, ⁴Rheumatology, University of Utah, Salt Lake City, UT, ⁵Rheumatic and Immunologic Diseases, Cleveland Clinic, Cleveland, OH, ⁶Division of Rheumatology, University of Pennsylvania, Philadelphia, PA, ⁷Rheumatology, Boston University, Boston, MA, ⁸Rheumatology & Clinical Immunology, University of Pittsburgh, Pittsburgh, PA, ⁹Division of Rheumatology, Johns Hopkins University, Baltimore, MD, ¹⁰Mayo Clinic, Rochester, MN, ¹¹Rheumatology, University of Pennsylvania, Philadelphia, PA, ¹²Division of Rheumatology, Mayo Clinic, Rochester, MN, ¹³University of South Florida, Tampa, FL

Meeting: 2017 ACR/ARHP Annual Meeting

Date of first publication: September 18, 2017

Keywords: Pulmonary Involvement and vasculitis

Session Information

Date: Monday, November 6, 2017

Title: Vasculitis Poster II: ANCA-Associated Vasculitis

Session Type: ACR Poster Session B

Session Time: 9:00AM-11:00AM

Background/Purpose:

Pulmonary involvement in microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA) includes pulmonary nodules (PN), diffuse alveolar hemorrhage (DAH), and infiltrates attributed to vasculitis, each of which can co-exist.

The primary aim of this study was to determine the frequency of patients with both PN and DAH within the Vasculitis Clinical Research Consortium (VCRC) Longitudinal Study cohort. Additionally, it was determined whether patients with both PN and DAH had higher mortality or experienced a higher frequency of disease flares as compared to patients with only PN, only DAH, only infiltrates, or patients with GPA/MPA without these pulmonary manifestations.

Methods:

Data were extracted from the VCRC Longitudinal Study cohort. All patients in the cohort satisfied the modified ACR classification criteria for GPA or Chapel Hill Consensus definition of MPA. Pulmonary manifestations were classified as per assessment by site investigators and reported to the database via a standard protocol.

Results:

Of 736 patients with GPA/MPA in the cohort, 250 (34.0%) never developed pulmonary findings and 486 (66.0%) developed lung manifestations (Figure 1). If PN were not the first lung manifestation of the 343 patients to ever develop nodules, only 20 (5.8%) patients developed them at a later date. If patients had DAH as their initial manifestation, only 4 (2.2%) patients developed PN later in their disease course. PN and DAH were noted to be contemporaneous in 71 (14.6%) of patients. Nine patients (2.6%) with PN first went on to develop DAH, compared with 4 (2.4%) of patients who had infiltrates first.

Patients with GPA/MPA without pulmonary findings experienced 0.77 deaths/100 patient-years, which was comparable to patients with PN alone at 0.66/100 patient-years (p=0.79), DAH alone at 0.89/100 patient-years (p=0.78), and PN and DAH together at 0.69/100 patient-years (p=0.83). In patients with each of PN, DAH and infiltrates there were 1.84 deaths/100 patient-years, or 3 deaths out of 32 patients (p=0.25).

Patients without pulmonary findings had disease flares at a rate of 8.68/100 patient-years. PN alone had a flare rate of: 5.71/100 patient-years (p=0.07), DAH alone: 7.54/100 patient-years (p=0.92), PN and DAH: 7.96/100 patient-years (p=0.80). Those with each of PN, DAH and infiltrates had a flare-rate of 23.31/100 patient-years (p<0.01).

Conclusion:

Presentation with PN and DAH, but not infiltrates, occurs at a frequency of 10.5% in patients with GPA/MPA. Such patients have similar outcomes compared to patients without lung manifestations of vasculitis, those with only PN, or those with only DAH. However, those subjects who ever manifested each of PN, DAH and infiltrates during the course of their illness are at higher risk of flare.

Disclosure: S. M. Rawn, None; G. Cox, None; C. Pagnoux, None; D. Cuthbertson, None; S. Carette, None; C. L. Koening, None; C. A. Langford, None; C. A. McAlear, None; P. A. Monach, None; L. W. Moreland, None; P. Seo, None; U. Specks, None; A. G. Sreih, None; S. R. Ytterberg, None; R. Borchin, None; P. A. Merkel, None; N. A. Khalidi, None.

To cite this abstract in AMA style:

Rawn SM, Cox G, Pagnoux C, Cuthbertson D, Carette S, Koening CL, Langford CA, McAlear CA, Monach PA, Moreland LW, Seo P, Specks U, Sreih AG, Ytterberg SR, Borchin R, Merkel PA, Khalidi NA. Pulmonary Manifestations in Microscopic Polyangiitis and Granulomatosis with Polyangiitis: A Multicenter Cohort Analysis [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/pulmonary-manifestations-in-microscopic-polyangiitis-and-granulomatosis-with-polyangiitis-a-multicenter-cohort-analysis/. Accessed .

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