Table 2 Literature review: characteristics of 17 patients
RefsSexAge(years)ANCA-VStatusChronology of PF-ANCA-VFollow-up(months)ClinicalTypeof PFPossible or proven IAHVasculitis on lung biopsyMPO-ANCAs
12F61MPADead: exacerbation of RIPF preceded ANCA-V (4 years)1L KUIP0Yes
M63MPAAliveSame time>12L KNDYesYes
F68MPADead: exacerbation of RISame time>84L KUIPYesYes
M64MPADead: exacerbation of RI + Pneumocystis jirovecili pneumoniaPF preceded ANCA-V (4 years)8L KUIP1NoYes
M79MPADead: exacerbation of RISame time∼12L KUIP0Yes
F78MPADead: exacerbation of RISame timeNDL KUIPYesNoYes
13F48MPAAlivePF preceded ANCA-V (1 year)4L KNDYesYes
M77MPADead: fatal bacterial pneumoniaPF preceded ANCA-V (5 years)19L KND0Yes
F72MPADead: exacerbation of RIPF preceded ANCA-V (6 years)5L KND0Yes
M70MPADead: lung carcinomaPF preceded ANCA-V (4 years)4L KNDYesNoYes
16M75MPADead: exacerbation of RIPF after ANCA-V (3 months)4L KND0Yes
10M65MPAAlivePF preceded ANCA-V (1 year)12L K S N APINS0P-ANCA
14F55MPAAlivePF preceded ANCA-V (1 year)NDL K OND0NoYes
11F77MPAAliveSame time>12L MUIPYesYesYes
15M71NDDead: unknownSame time3L KND0YesND
F74MPADead: perforated diverticulumSame time48L KND0P-ANCA
F72MPADead: unknownSame time72L KND0YesP-ANCA
Total9F/8M69*7 (41%)
  • *Average age.

  • A, arthritis; ANCA-V, systemic vasculitis related to antineutrophil cytoplasmic antibodies; IAH, intra-alveolar haemorrhage; K, kidney; L, lung; M, muscles; MPA, microscopic polyangiitis; MPO-ANCAs, anti-myeloperoxidase-ANCAs; N, neuropathy; ND, not determined; O, ophthalmology; P-ANCA, perinuclear-ANCAs; PF, pulmonary fibrosis; S, skin; UIP, usual interstitial pneumonia.