Table 2.
Clinical Characteristics, Treatment, and Outcomes of Patients With Antineutrophil Cytoplasmic Antibody-Positive Idiopathic Pulmonary Fibrosis
| Identification/ Cohort | Age (years)/Sex | ANCA Type | Vasculitis Diagnosis | Other Autoantibodies, Titers | Lung Biopsy Results | Outcome | Treatment | Months of Database Follow-Up | Months of Chart Follow-Up |
|---|---|---|---|---|---|---|---|---|---|
| 1/D | 72/F | PR3 | No | a | Emphysema, some areas of fibroblast foci, lung fibrosis with architectural distortion and subpleural accentuation. Nonclassifiable fibrosing interstitial pneumonitis. | Deceased | AZA | 25 | 0 |
| 2/D | 73/F | MPO | No | a | UIP | Deceased | AZA | 59 | 0 |
| 3/D | 54/F | MPO | MPA | ANA 1:160, RF 157 | a | Deceased | Steroids, AZA, CYC, MMF, RTX | 101 | 102 |
| 4/D | 65/M | PR3 | No | None | Moderate to severe fibrosis with subpleural and bronchiolocentric accentuation, fibroblast foci, and patchy airway-centered fibrosis. No well-developed microscopic honeycombing. | Transplant | Pirfenidone, nintedanib | 60 | 73 |
| 5/D | 63/F | MPO | MPA | ANA 1:160 | a | Alive | Steroids, RTX, NAC | 48 | 27 |
| 6/D | 67/M | MPO | No | ANA 1:40 | a | Alive | a | 42 | 0.0 |
| 7/D | 65/F | MPO | No | None | a | Alive | Nintedanib | 36 | 46 |
| 8/D | 67/M | MPO | No | ANA 1:640, RF 351 | a | Alive | Nintedanib | 25 | 34 |
| 9/D | 59/M | PR3 | No | ANA 1:160, anti-dsDNA, anti-CCP | UIP | Alive | a | 16 | 0 |
| 10/D | 55/F | PR3 | No | a | UIP | Alive | Pirfenidone | 12 | 18 |
| 11/D | 84/M | PR3 | No | ANA 1:40 | a | Alive | Nintedanib | 14 | 20 |
| 12/D | 65/M | PR3 | No | None | Emphysema, insufficient material | Alive | Pirfenidone, nintedanib | 11 | 18 |
| 13/D | 76/M | PR3 | No | None | UIP, scattered lymphoid aggregates with occasional germinal centers noted in the fibrotic regions. Pulmonary arteries show mild to moderate thickening. | Alive | Pirfenidone, nintedanib | 11 | 7 |
| 14/D | 67/M | PR3 | No | None | a | Alive | Nintedanib | 5 | 11 |
| 15/R | 84/M | NS | No | SSA | a | Alive | None | 4 | 7 |
| 16/R | 53/M | NS | No | ANA 1:160 | Extensive OP, superimposed on UIP | Alive | Steroids, AZA | 8 | 7 |
| 17/R | 65/M | PR3 | No | ANA 1:160 | UIP, emphysema, moderate to severe pulmonary hypertension | Alive | Nintedanib, pirfenidone | 26 | 32 |
| 18/R | 75/F | MPO | No | ANA 1:320, anti-CCP | a | Alive | Steroids, nintedanib | 14 | 16 |
| 19/R | 72/M | MPO | No | RF 166, Scl70 | UIP, centrilobular emphysema, scattered regions of dendriform calcifications | Alive | Steroids, MMF, pirfenidone | 0 | 15 |
| 20/R | 82/F | MPO | No | RF 37, SSA, SSB, anti-Ku | a | Alive | Steroids, MMF, pirfenidone | 3 | 2 |
| 21/R | 46/F | MPO | no | ANA 1:160, RF 62 | UIP with poorly formed granulomas consistent with chronic hypersensitivity pneumonitis, secondary pulmonary hypertension | Alive | Steroids, AZA, RTX, CYC | 26 | 22 |
| 22/R | 67/F | MPO | MPA | ANA 1:160 | UIP | Alive | Steroids, AZA, MMF, RTX | 0 | 4 |
| 23/R | 61/F | MPO | AAV | ANA 1:320 | UIP, NSIP in less involved areas, rare multinucleated giant cells | Alive | Steroids, AZA, MMF | 60 | 66 |
| 24/R | 71/M | NS | No | ANA 1:40 | a | Alive | Steroids | 2 | 12 |
| 25/R | 69/M | MPO | No | ANA 1:1,280, RF 78 | UIP, lymphoid follicles with germinal centers. Focal giant cells without granulomas | Transplant | None | 24 | 31 |
| 26/R | 70/M | NS | No | ANA 1:160 | a | Alive | Steroids, pirfenidone | 1 | 2 |
| 27/R | 74/M | NS | No | ANA 1:2,560 | UIP, few nonnecrotizing granulomas consistent with chronic hypersensitivity pneumonitis | Alive | Steroids, MMF | 11 | 15 |
| 28/R | 62/F | MPO | No | ANA 1:640 | UIP with extensive OP | Deceased | Steroids | 3 | 2 |
| 29/R | 76/F | MPO | No | ANA 1:80, RF, SSA | a | Alive | Steroids, AZA | 72 | 0 |
| 30/R | 55/F | NS | No | ANA 1:160 | a | Alive | Steroids, AZA | 29 | 48 |
| 31/R | 77/F | PR3 | No | ANA 1:320 | UIP | Alive | None | 80 | 85 |
| 32/R | 77/M | MPO | No | RF 23 | a | Deceased | None | 8 | 0 |
| 33/R | 71/M | MPO | No | ANA 1:160 | a | Deceased | Steroids | 5 | 5 |
| 34/R | 68/F | MPO | AAV | RF 20 | a | Deceased | Steroids, CYC | 45 | 9 |
AAV = ANCA-associated vasculitis; ANA = antinuclear antibody; Anti-CCP = anti-cyclic citrullinated peptide antibody; Anti-dsDNA = anti-double stranded DNA antibody; AZA, azathioprine; CYC = cyclophosphamide; D = discovery cohort; F = female; M = male; MMF = mycophenolate mofetil; Months of chart follow-up = time from study enrollment to most recent clinical documentation for review in the patient’s electronic medical record; Months of database follow-up = time since study enrollment to most recent vital status check; MPA = microscopic polyangiitis; MPO = myeloperoxidase; NAC = N-acetylcysteine; NS = nonspecific ANCA; NSIP = nonspecific interstitial pneumonia; OP = organizing pneumonia; PR3 = proteinase 3 antibody; R = replication cohort; RF = rheumatoid factor; RTX = rituximab; SSA = anti-Ro; SSB = anti-La; Treatment = medication recommended by consulting pulmonologist or medication that patient was recorded as taking; UIP = usual interstitial pneumonia. See Table 1 legend for expansion of other abbreviation.
No data available.