Herein we report a case of pulmonary hemorrhage with capillaritis
on tissue sections from a case of MMF-associated lung disease.
Here, we report a patient who had a previous MPA diagnosis with pulmonary capillaritis
, no renal involvement and positive p-ANCA testing and new developing coexistence of RA with this vasculitis.
Abstract: Pulmonary capillaritis
presenting as diffuse alveolar hemorrhage is a rare manifestation in patients with IgA nephropathy.
Microscopic polyangiitis (MPA) (6, 11, 12) is characterized by glomerulonephritis in 90% of patients; pulmonary capillaritis
in 50%; gastrointestinal involvement in 50%; skin involvement in 60%; ear, nose, and throat involvement in 35%; and musculoskeletal features in 60%.
The lung biopsy confirmed the hemorrhage, with one case associated with capillaritis
with areas of organization.
41,42) Rarely, alveolar hemorrhage with capillaritis
Cytoplasmic antineutrophil cytoplasmic antibody (C-ANCA) was positive, perinuclear antineutrophil cytoplasmic antibody (P-ANCA) was negative, and open lung biopsy showed capillaritis
Vasculitis in Wegener granulomatosis affects both arteries and veins; occasionally, capillaries may be involved in a process known as necrotizing capillaritis
(132,138,143-145) (see below).
MICROSCOPIC POLYANGIITIS (MPA) is a systemic, small-vessel vasculitis, primarily associated with necrotizing glomerulonephritis and pulmonary capillaritis
Occasionally, diffuse alveolar hemorrhage (DAH) with capillaritis
will present with fulminate respiratory failure.
occasionally, the vasculitis in Wegener granulomatosis affects predominantly alveolar capillaries, resulting in a combination of necrotizing capillaritis
and alveolar hemorrhage, (55) which is manifested by acute inflammation distributed along alveolar septal capillaries, with focal necrosis and karyorrhectic debris (Figure 21).
Although cancer-associated MN has similar histopathologic features to idiopathic or primary MN, Lefaucheur et al (65) recently found that the majority of cancer-associated MNs were in the early stages of development, and many demonstrated increased leukocytes within the glomerular capillaries, which has been termed glomerulitis or glomerular capillaritis