Chest imaging refers to computed tomography scans except for one unaffected carrier with plain a chest radiograph. Autoantibody titer of 1:80 or higher are considered positive for ANA. Lung transplants include one patient with combined heart–lung transplant and one patient with redo lung transplantation. Nephritis defined by hematuria, excluding known nephrolithiasis or diagnosis on biopsy. Other musculoskeletal involvement includes arthralgias, myalgias, avascular necrosis, or need for orthopedic surgery, except when indication was trauma. Proteinuria defined as two or more instances of urine protein creatinine ratio >1. Reduced glomerular filtration rate defined as ≤60. Statistical analysis performed by log-rank test for ages at symptom onset and death or by Fisher exact test for various disease manifestations. ANA = antinuclear antibody; ANCA = antineutrophil cytoplasmic antibody; MPO = myeloperoxidase antibody; PR3 = proteinase 3 antibody; RF = Rheumatoid Factor; CCP = anti-cyclic citrullinated peptide antibody.