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.
Pulmonary capillaritis in a patient with moderately positive anti-PL-12 antibodies.
As there are currently no established evidence-based treatment protocols for DAH, cases are generally treated with high-dose corticosteroid therapy or immunosuppressive therapy (e.g., cyclophosphamide), which target
pulmonary capillaritis and vasculitis [12].
The etiology of DAH is multifactorial and can be divided into three categories, namely,
pulmonary capillaritis, bland alveolar hemorrhage, and diffuse alveolar damage (DAD) [10].
West, "Antiphospholipid antibodies as a cause of
pulmonary capillaritis and diffuse alveolar hemorrhage: a case series and literature review," Seminars in Arthritis and Rheumatism, vol.
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.
Microscopic polyangiitis is a necrotizing, pauci-immune, small-vessel vasculitis, commonly resulting in glomerulonephritis and
pulmonary capillaritis in the absence of granulomatous inflammation (8).
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.
However, the data on the role of macrophages on SLE complicated with AS and hemorrhagic
pulmonary capillaritis are scarce.
Several studies have postulated that ENL is an immune-complex mediated process resulting in the overproduction of immunoglobulin, complement and cytokines, whereas DAH is described by
pulmonary capillaritis as a result of the deposition of immune-complexes leading to an inflammatory cascade, finally followed by alveolar hemorrhage.
The histological findings confirmed a diagnosis of Wegener's granulomatosis, with
pulmonary capillaritis and necrogranulomatous dural involvement of the skull base resulting in multiple cranial neuropathies.