Vasculitis and Hemorrhagic Complications: An Overview

What Is Vasculitis?

Vasculitis refers to the inflammation of the vasculature of the body. The vascular system of the body is highly complex and comprises three main components, namely the arteries, veins, and capillaries. The arteries are responsible for carrying blood away from the heart to all parts of the body. The veins collectively bring all the deoxygenated blood from other parts of the body back to the heart. The capillaries are where the exchange of nutrients and gasses takes place between the blood and tissues. They form the link between the arteries and the veins. Autoimmunity refers to one’s immune system being highly active, generating an abnormal response. The immune system targets the healthy tissues of the body by producing inflammatory chemokines that lead to cell death. In vasculitis, characteristic inflammation of the blood vessels is observed. The vessels in the affected region become highly inflamed. The walls of the vessels are weakened over time due to persistent stretching. The blood flow is significantly impaired owing to the constricted vessel lumen. The possibility of bursting the vessel walls is increased multifolds. This phenomenon that leads to bleeding into the surrounding tissues and structures is defined as hemorrhage.

Vasculitis carries the full potential to cause strokes, aneurysms, blood clots, and swelling of affected organs and organ systems. This article reviews some important hemorrhagic complications of vasculitis.

What Are the Various Types Of Hemorrhages Encountered In Vasculitis?

The hemorrhagic complications associated with vasculitis are regarded to be life-threatening in more ways than one. Any organ or organ system may be affected by this debilitating condition. The cerebral, gastrointestinal, and alveolar hemorrhages are among the commonly reported cases. Other complications involving the skin, soft tissues, and muscles are also quite commonly seen.

1. Cerebral Hemorrhage

• Also referred to as hemorrhagic stroke.

• Characterized by intracranial bleeding, resulting in the accumulation of blood between the brain and the skull.

• A Blood vessel located in the brain bursts owing to the weakened vessel wall and chronic inflammation caused by vasculitis.

• The brain bleed must be addressed as soon as possible as a lack of blood supply to the brain for more than a specified time may result in serious physical or mental disability.

• In cases of severe blood loss, death is certain.

• Cerebral aneurysms also lead to the development of stroke.

• The blood flowing through the inflamed vessel may eventually clot, thereby obstructing the blood flow. This leads to an ischemic stroke that is characterized by a lack of availability of oxygen to the surrounding tissue structure.

• General symptoms may include fever, restlessness, weight loss, joint aches and pains, and muscle fatigue.

• Specific symptoms associated with the nervous system include headaches, transient ischemic attack (TIA), swelling of the brain, forgetfulness, partial paralysis of one side of the body, seizures, compromised vision, and verbal disturbances.

2. Gastrointestinal Hemorrhage

• This particular condition is characterized by bleeding that may occur in any part of the digestive tract (from mouth to anus).

• The bleeding may either be acute or chronic. The sudden onset of rapid bleeding may be life-threatening and require immediate medical care.

• There may be the presence of blood in the excreted feces due to leaked blood vessels.

• The symptoms generally consist of abdominal cramps, dark stools, shortness of breath, difficulty breathing, tiredness, fatigue, and vomiting accompanied by blood in them.

3. Alveolar Hemorrhage

• Also called diffuse alveolar hemorrhage or pulmonary hemorrhage.

• This is the most common type of hemorrhagic complication associated with vasculitis.

• It is characterized by pooling of blood into the alveolar space.

• The primary cause of alveolar hemorrhage is due to inflammation of the capillaries.

• Classic symptoms involve dyspnea (shortness of breath), pulmonary infiltrates, sudden lowering of blood hemoglobin, and coughing of blood from the lungs (hemoptysis).

• Other external factors like adverse drug effects, coagulation disorders, inhalation of toxins, or organ transplantation may also contribute to the incidence of alveolar hemorrhage.

• A biopsy of the involved regions is a reliable diagnostic approach for alveolar hemorrhage.

• Early diagnosis facilitates a good prognosis. A prolonged delay in initiating treatment may result in hypoxemic respiratory failure (insufficient oxygen in blood).

• Symptoms include dyspnea, non-specific cough, expulsion of cough laced with blood, chest pains, and fever.

4. Henoch-Schonlein Purpura

• Also called IgA Vasculitis.

• It is a vasculitis-associated disorder characterized by inflamed and bleeding small vessels present in the skin, joints, intestines, and kidneys.

• Bleeding of the skin vessels leads to the development of a reddish-purple rash, usually on the lower legs and buttocks.

• Abdominal cramps accompanied by minor joint aches and pains are also observed in this disorder.

• This disorder is more commonly found in children below 10 years; however, adults may also be affected. Males are more predisposed to acquiring the disease compared to females.

• Involvement of the kidneys is also a symptom of this disorder. It is detected clinically by performing urine analysis to check the excretion of protein or blood through the urine.

• Severe complications involve unresolved bowel obstruction or irreparable kidney damage.

5. Muscle Hematoma

• Muscles are highly vascular structures that receive a consistent, widespread blood supply from a large number of blood vessels.

• Vasculitis in any of the affected vessels may cause it to burst open, resulting in extravascular bleeding.

• This condition is characterized by bleeding into a particular muscle group.

• The accumulation of blood results in the formation of a hard, localized mass of blood. This may obstruct blood flow to the surrounding region, leading to necrosis and cell death.

• In severe cases where timely treatment is not initiated, gangrene of the limb is evident.

• Other diagnostic tools that may help identify an intramuscular hemorrhage include computed tomography (CT) or Magnetic resonance imaging (MRI).

• Muscle hematomas are usually rare, although they carry a significant percentage of morbidity.

Conclusion

Vasculitis is an atypical disease that may present multiple difficulties concerning all aspects of the human body. The gold standard management of vasculitis involves the use of immunosuppressive therapy and symptomatic relief from the respective complications that are presented along with it. One must remain cautious of all the life-threatening possibilities involving vasculitis and maintain regular visits to the doctor irrespective of symptom flare-ups. Hemorrhagic complications are always considered a priority when planning the treatment of vasculitis.