Antiphospholipid antibodies-associated diffuse alveolar hemorrhage

Difficulties in the Treatment of Recurring Diffuse Alveolar Hemorrhage Accompanying Primary Antiphospholipid Syndrome: A Case Report and Literature Review

“…It should be noted that DAH symptoms may be mild enough to be overlooked in a proportion of patients. As reported by Yachoui et al [4], such patients may experience complete and sustained remission without any immunosuppression. Moreover, not all patients will develop a relapse and require long-term treatment [6].…”

“…Unfortunately, not all patients who present with DAH in a PAPS/APLA setting actually exhibit neutrophilic vasculitis on pathologic examination. Yachoui et al [4] published a case series report in which only one out of six patients subjected to lung biopsy showed neutrophilic infiltration, while the remaining five presented as bland hemorrhage [4]. Others have underlined [2] that neutrophilic interstitial infiltration alone, [3,4,10,11] without perivascular or vascular infiltrates does not allow for recognizing vasculitis.…”

“…Yachoui et al [4] published a case series report in which only one out of six patients subjected to lung biopsy showed neutrophilic infiltration, while the remaining five presented as bland hemorrhage [4]. Others have underlined [2] that neutrophilic interstitial infiltration alone, [3,4,10,11] without perivascular or vascular infiltrates does not allow for recognizing vasculitis. However, lack of these features does not allow for a virtual ruling out of vasculitis because histological findings may vary depending on both the lung region and the activity of the disease [12].…”

“…Our patient is a male [3-6, 10, 11], in the fourth decade of life [3,4,6,10,11], with a neutrophilic (49%) BALF [4,10] and high APLA titer [3-5, 10, 11]. Due to lack of the histopathological examination and normal bone density values, we cannot dismiss that pathological fractures, which our patient experienced, were a result of osteonecrosis, reported in other patients with PAPS as well [2].…”

“…Other manifestations such as diffuse alveolar hemorrhage (DAH), Libman-Sacks endocarditis or chorea cannot be easily linked to the influence of APLA on blood coagulation [2]. Diffuse alveolar hemorrhage (DAH), which may be the first or isolated symptom in PAPS/APLA positive patients [3][4][5][6], is a clinical syndrome of multi-causal pathological background. As DAH symptoms are a result of an erythrocyte accumulation in the alveolar air space, most common features are diffuse bilateral airspace opacities on radiological examination, hypoxemic respiratory failure, anemia, and hemoptysis.…”