Diffuse Alveolar Haemorrhage in ANCA-associated Vasculitis

Abstract

Diffuse alveolar haemorrhage (DAH) is a serious complication of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). A literature review was performed to ascertain the diagnostic features, treatment, and outcome of this rare but serious condition. Haemoptysis and dyspnoea are common but nonspecific features. Chest radiography is usually abnormal, and high-resolution computerised tomographic scanning is more sensitive. Increased uptake of inhaled carbon monoxide and reduced clearance of C 15 O on lung function testing is suggestive of intra-alveolar blood. Fiberoptic bronchoscopy and bronchoalveolar lavage are useful when a super-added infection is suspected. Concurrent renal disease is common and contributes to the morbidity and mortality. Treatment should be individualised, and it is based on glucocorticoid and cyclophosphamide induction with azathioprine maintenance. The role of plasmapheresis is unclear, and is currently being evaluated. Patients are at risk of disease and treatment-related long-term complications. Ongoing research into the most efficacious therapeutic regimens associated with the least side effects is especially important.

Rheumatoid arthritis presenting as rheumatoid meningitis: A case report

“…[2] Serum inflammatory studies typically are of little use, although ferritin and serum IL-2 receptor levels may correlate with meningeal inflammation in some patients. [8] Brain imaging traditionally shows meningeal thickening and localized enhancement. [4] MRI is the preferred imaging modality, although contrast CT can be used for diagnosis, as seen in our patient who could not undergo MRI due to pacemaker placement.…”

“…[2,9] CSF studies often show elevated protein, with potential CSF biomarkers of disease activity including IL-6 and RF. [2,7,8] CSF studies can occasionally show a pleocytosis and low glucose as well. [7] Typically, histopathological examination of meningeal tissue is required which can reveal rheumatoid nodules, nonspecific meningeal inflammation, or vasculitis.…”

Recalcitrant steroid-resistant EGPA with multisystem involvement

“…The concurrent presentation of these two symptoms alone highlights the rarity of our patient's initial presentation. [8,9] The presence of disabling myalgia at the time of hospitalization was also notable, as myalgia rarely represents a main presenting feature of EGPA and is most commonly seen during the prodromal phase of the illness. [4] Gastrointestinal involvement in EGPA is thought to be eosinophil-mediated, [10,11] which was not readily apparent on initial histopathologic evaluation of our patient's intestinal biopsy specimens.…”

Hemorrhagic complications associated with PR3-ANCA crescentic glomerulonephritis

“…[7][8][9][10][11] A systematic review of a total of nine studies performed between 1985 and 2012 described the clinical features of alveolar hemorrhage. 12 The individual studies varied from 5 to 80 patients in size with a total of 207 patients. Hemoptysis and dyspnea were common but non-specific features.…”

“…Fiberoptic bronchoscopy and bronchoalveolar lavage are useful when a superimposed pulmonary infection is suspected. 12 Severe alveolar hemorrhage requiring ventilation occurs in 5% of patients with ANCA disease. 8 Despite increased understanding of pathogenesis and newer therapeutic options, patients with ANCA disease accompanied by severe alveolar hemorrhage have a 5-year patient survival of 75%.…”