A spontaneous intercostal artery hemorrhage in systemic lupus erythematosus

Abstract

Systemic lupus erythematosus (SLE) is a chronic inflammatory disease that can lead to damage to several vital organs. Antiphospholipid syndrome (APS), manifesting as vascular thromboembolic events and morbidities of pregnancy in the presence of antiphospholipid antibodies (aPL), has been described in patients with SLE. Catastrophic antiphospholipid syndrome (CAPS), in contradistinction to APS, is defined as three or more organs affected by thrombotic microangiopathy in patients demonstrating aPL and can result in mortality up to 50%. We describe a unique SLE patient who was diagnosed with recurrent APS presented with axillary venous thrombosis and subsequent superficial edema and compartment syndrome. The CAPS followed and revealed thromboses over liver, spleen, and acute pancreatitis. The spontaneous hemorrhage of left fourth intercostal artery (ICA) and left axillary artery occured at the same time without vasculitis or severe trauma. Though emergency transcatheter arterial embolization (TAE) of the left fourth ICA was successfully accomplished by the radiologist. The repeated computed tomography angiogram of chest demonstrated remission of ruptured ICA. Nevertheless, the patient died of diffuse alveolar hemorrhage and respiratory failure and shock. Both disseminated intravascular coagulation (DIC) and CAPS share similar characteristics encompassing thrombotic microangiopathy, bleeding, thromboembolism, and multiple organ dysfunction. It is difficult to distinguish between them, especially in cases such as our uremic SLE patient with a calamitous disease progression. The emphasis of treatment for DIC is on platelet and fresh plasma transfusion, in contrast with anti-coagulant for CAPS. To the best of our knowledge, this is the first report describing ICA hemorrhage in an SLE patient without vasculitis or aneurysm. The lupus flare initiated a pathological immunological cascade and resulted in the CAPS and the vascular damage.

Massive Hemorrhage From the Posterior Intercostal Artery Following Lower Partial Sternotomy: a Case Report

“…Trauma including blunt chest and iatrogenic injuries is the most common cause of intercostal artery bleeding [5]. Spontaneous intercostal artery bleeding is generally observed in patients with disorders, such as neuro bromatosis type 1, systemic lupus erythematosus, or coarctation of aorta [6,7,8]. In our case, the CT did not reveal any rib fractures or evidence of trauma.…”

Spontaneous rupture of an intercostal artery

“…Of the seven reported cases found of spontaneous haemorrhage, most occurring in elderly patients, and in three of them, no predisposing factor was identified 3–9. Our patient is also elderly, chronically medicated with an antiplatelet agent and under a haemodialysis programme, which may be possible causes that favour haemorrhage.…”

Intercostal artery hemorrhage with hemothorax following combined lateral and posterior lumbar interbody fusion: a case report

“…To our knowledge, this is the first case description of ICA bleeding associated with LLIF surgery. Besides trauma, which is the most common reason for intercostal bleeding, predisposing factors include Neurofibromatosis Type 1, systemic lupus erythematosus (SLE), uncontrolled hypertension, anticoagulation and bleeding disorders [15,16,19,20,22,23]. Neurofibromatosis Type 1 is associated with arterial stenosis due to intimal or medial dysplasia and also arteriovenous malformations and aneurysms, which may lead to spontaneous vessel rupture in rare cases.…”