Image Type: Vasculitis
Body Site: Legs
Description: Vasculitis refers to a group of uncommon diseases characterized by inflammation and damage to blood vessel walls. The vascular system includes arteries that carry oxygen-rich blood to tissues and veins that return oxygen-depleted blood to the lungs. The cause of vasculitis is often unknown, but it typically involves immune system abnormalities and blood vessel inflammation. Symptoms vary based on the affected organs.
Laboratory tests of blood or body fluids usually show signs of inflammation, and organ function tests may be abnormal if organs are involved. Diagnosis is confirmed through a biopsy of affected tissue, such as skin, sinuses, lungs, nerves, or kidneys. Alternatively, an angiogram, an X-ray test of blood vessels, can be used to identify inflammation patterns.
Differential diagnosis of vasculitis:
Infection (sepsis, endocarditis): Heart murmur, rash, and musculoskeletal symptoms can occur in bacterial endocarditis, sterile emboli, cardiac myxoma, and antiphospholipid antibody syndrome. Tests include obtaining blood cultures and echocardiograms, check for antiphospholipid antibodies
Drug toxicity/poisoning: This may produce vasospasm resulting in symptoms due to ischemia. Checking for cocaine, amphetamines, ephedra alkaloids, and phenylpropanolamine can be helpful.
Coagulopathy: Occlusive diseases such as disseminated intravascular coagulation, antiphospholipid antibody syndrome, and thrombotic thrombocytopenic purpura can cause ischemic symptoms. It is important to check the hypercoagulability panel.
Malignancy: Paraneoplastic vasculitis is rare. Any organ system may be affected, but the skin and nervous system are the most common. Vasculitis symptoms may precede, occur simultaneously with, or follow a diagnosis of cancer. Consider malignancy in patients with incomplete or no response to therapy for idiopathic vasculitis.
Atrial myxoma: Classic triad of symptoms: Embolism, intracardiac obstruction leading to pulmonary congestion or congestive heart failure, and constitutional symptoms (fatigue, weight loss, fever). Skin lesions can be identical to those seen in leukocytoclastic vasculitis. Atrial myxomas are rare, but they are the most common primary intracardiac tumor. Myxomas also can occur in other cardiac chambers.
Multiple cholesterol emboli: Typically seen in patients with severe atherosclerosis. Embolization may occur after abdominal trauma, aortic surgery, or angiography. May also occur after heparin, warfarin, or thrombolytic therapy. Patients may have livedo reticularis, petechiae and purpuric lesions, and localized skin necrosis.
Congenital collagen disorders (Ehlers-Danlos syndrome, Marfan syndrome): Patients with hereditary conditions may have joint laxity. Marfan syndrome is characterized by tall stature, scoliosis, arachnodactyly, pectus excavatum, high-arched palate, subluxation of the lens, mitral valve prolapses, and dilatation of the ascending aorta. Ehlers-Danlos syndrome is associated with easy bruising, distensible skin, arterial aneurysms/rupture, enteric/uterine rupture, spontaneous pneumothorax, hernias, and a history of poor wound healing. The presentation of these conditions can be heterogeneous, and not all patients have all the features of the disease. Some patients may not exhibit obvious joint or skin manifestations of Ehlers-Danlos syndrome, and there are no associated systemic symptoms such as fever or malaise.
Fibromuscular dysplasia: More common in females than males. Stenoses are more common than aneurysms. Renal arteries are the most commonly involved vessels. No associated systemic symptoms (e.g., fever, malaise).
Treatment: The goal of vasculitis treatment is to reduce inflammation in the blood vessels. Mild cases can be managed with over-the-counter pain relievers, while severe cases require prescription medications. These include non-steroidal anti-inflammatory medicines (NSAIDs) for pain and inflammation, corticosteroids to decrease immune activity, and immunosuppressive medicines to lower immune responses. Other treatments involve immunomodulators, interferon therapy, interleukin antagonists, and intravenous immunoglobulin (IVIG) to control immune responses and fight infection. Monoclonal antibodies and tumor necrosis factor inhibitors also suppress the immune system. Procedures like plasmapheresis to replace blood plasma and surgical bypass to restore blood flow in severe cases are sometimes used, though surgery is rare.