The presence of donor-specific alloantibodies with proteinuria, allograft dysfunction, and biopsy-proven antibody-mediated rejection (AMR) including but not limited to glomerulitis,
capillaritis, and c4d fixation was treated with plasmapheresis and IVIG (200 mg/kg) followed by rituximab.
1 The parallel histologic findings include focal and segmental necrotizing glomerulonephritis with crescents on renal biopsy and alveolar
capillaritis on lung biopsy.
Report for every sample should include the following: site (segment from which the sample comes), size (2 maximum diameters in perpendicular directions assessed at the microscope), "central sampling" (mainly bronchial with cartilage plates identified or bronchiolar structures in greater than 40% of the surface of the sample), "peripheral sampling" (with or without visceral pleura, with alveoli present in at least 60% of the surface of the sample), and histologic pattern (respiratory bronchiolitis, desquamative interstitial pneumonia, NSIP, organizing pneumonia, UIP,
capillaritis, among others).
DAH is often characterized by pathy zones of consolidation that rapidly coalesced to form air-space consolidation of both lungs in the background of ground-glass opacity and interlobular thickening which could be the early sign of pulmonary
capillaritis of ATRA syndrome.[3] As shown in this case, early recognition, instantaneous initiation of dexamethasone and daunorubicin, and adequate respiratory support if needed are the key of successful treatment and may help to improve its prognosis.
Diffuse alveolar hemorrhage (DAH) is a clinicopathological syndrome characterized by intra-alveolar bleeding from damaged pulmonary microvasculature, which can arise due to factors such as pulmonary
capillaritis or humoral immune responses [1-3].
His Cr worsened despite hydration and conservative management for which the patient underwent a renal biopsy on hospital day 4, with findings of acute tubular necrosis, interstitial hemorrhage, and
capillaritis.
Repeat transplant biopsy was performed and showed signs of chronic-active ABMR (peritubular
capillaritis with positive C4d staining, glomerular double contours, and severe atrophy/fibrosis) (Figure 2).
He underwent right wedge resection lung biopsy which revealed evidence of bland pulmonary hemorrhage (Figure 3(a)) with no evidence of
capillaritis or diffuse alveolar damage (DAD).
In 8 of 10 patients, pulmonary
capillaritis was present on biopsy [1].
A first kidney biopsy was done on POD 12 (Figure 3(a)) and revealed thrombotic microangiopathy, glomerulitis (g3), peritubular
capillaritis (cpt2), and a peritubular C4d deposition.
We report a severe NS phenotype with a horseshoe kidney, thrombocytopenia, myeloproliferative disorder (MPD), feeding difficulty secondary to pylorospasm, and respiratory failure with pulmonary
capillaritis and vasculitis.