We report an autopsy case of elderly-onset
anticentromere antibody-positive
pulmonary-renal syndrome. An 84-year-old woman was admitted to our hospital with complaints of leg
edema and general malaise. Neither skin rush nor
arthritis was seen. Because of
hematuria,
proteinuria with various casts, renal dysfunction and
anemia, a clinically diagnosis of rapidly progressive
glomerulonephritis was made. Slight
pulmonary hypertension was observed in ultrasonic cardiography. Hypocomplementemia was not seen. Tests for MPO- and PR 3-anti-neutrophil cytoplasmic
antibodies and anti-glomerular basement membrane antibody were negative, but a high titer of
antinuclear antibody with a discrete speckled pattern on immunofluorescent staining was disclosed. Results for
anticentromere antibody and anti-Ki antibody were positive, but for anti-Sm antibody and
anti-double stranded DNA antibody were both negative. She did not present any clinical features of
systemic sclerosis or
CREST syndrome. Subsequently,
prednisolone was administered, but pulmonary alveolar
hemorrhage occurred and the patient died of acute
respiratory failure caused by massive pulmonary
hemorrhage. Autopsy revealed crescentic
glomerulonephritis including glomerular capillaritis and pulmonary capillaritis with positive granular deposits of
immunoglobulins and compliment on the glomerular and pulmonary capillary walls. Immunologically mediated crescentic
glomerulonephritis and pulmonary capillaritis was then diagnosed histopathologically. The main pathological feature of the case was small-vessel
vasculitis with
immune-complex deposition. Although this case did not fulfill the clinical criteria for
systemic lupus erythematosus (SLE), its histological features resembled those of
lupus nephritis and acute lupus
pneumonitis. We speculated that
anticentromere antibody-positive
pulmonary-renal syndrome without any other symptoms or signs of
connective tissue disease, such as our case, is a clinical entity distinct from typical SLE or
CREST syndrome.