From the Guidelines
Severe microangiopathic disease is a life-threatening condition that requires prompt diagnosis and treatment, and the management should be guided by the underlying etiology of the disease, with plasma exchange, glucocorticoid, and rituximab being the cornerstone of treatment for primary or secondary complement-mediated thrombotic microangiopathy, as recommended by the KDIGO 2024 clinical practice guideline for the management of lupus nephritis 1.
Key Characteristics of Severe Microangiopathic Disease
- Severe microangiopathic disease is characterized by damage to the small blood vessels throughout the body, leading to the destruction of red blood cells as they pass through these damaged vessels.
- This process, called microangiopathic hemolytic anemia, results in fragmented red blood cells (schistocytes) visible on blood smears.
- Common forms include thrombotic thrombocytopenic purpura (TTP), hemolytic uremic syndrome (HUS), disseminated intravascular coagulation (DIC), and malignant hypertension.
Diagnosis and Treatment
- Patients typically present with symptoms of anemia (fatigue, weakness), thrombocytopenia (easy bruising, bleeding), and organ dysfunction (kidney failure, neurological symptoms).
- Treatment depends on the underlying cause, but often includes:
- Plasma exchange for TTP
- Antibiotics for infection-related HUS
- Treating the underlying condition for DIC
- Blood pressure control for hypertension-related cases
- The PLASMIC score can be used for rapid assessment of adults with thrombotic microangiopathies, and a score of >5 points indicates a moderate to high risk of thrombotic microangiopathy 1.
Management of Lupus Nephritis and Thrombotic Microangiopathy
- Patients with lupus nephritis and thrombotic microangiopathy should be managed according to the underlying etiology of the thrombotic microangiopathy, as shown in Figure 13 of the KDIGO 2024 clinical practice guideline for the management of lupus nephritis 1.
- The treatment of primary or secondary complement-mediated thrombotic microangiopathy should include plasma exchange, glucocorticoid, and rituximab, with or without caplacizumab, and consideration of eculizumab, as recommended by the KDIGO 2024 clinical practice guideline for the management of lupus nephritis 1.
From the Research
Definition of Severe Microangiopathic Disease
Severe microangiopathic disease refers to a group of disorders characterized by microangiopathic hemolytic anemia, thrombocytopenia, and organ injury due to endothelial damage and vascular occlusion 2. These conditions are associated with significant morbidity and mortality and require urgent recognition and treatment.
Characteristics of Microangiopathic Disease
The key features of microangiopathic disease include:
- Microangiopathic hemolytic anemia
- Thrombocytopenia
- Organ injury due to endothelial damage and vascular occlusion
- Presence of microthrombi in multiple microvascular beds 3
Types of Microangiopathic Disease
There are several types of microangiopathic disease, including:
- Thrombotic thrombocytopenic purpura (TTP)
- Hemolytic uremic syndrome (HUS)
- Atypical HUS
- Familial HUS/TTP
- Secondary TMA associated with infection, pregnancy, autoimmune disease, or malignant hypertension 2, 4
Diagnosis and Management
Diagnosing microangiopathic disease can be challenging due to the limited availability of disease-specific testing 2. However, identifying the underlying etiology is crucial as treatment strategies differ. Treatment approaches include:
- Plasma exchange with fresh frozen plasma replacement
- Immunosuppression with corticosteroids
- Targeted therapeutics such as caplacizumab and rituximab 5
- Supportive therapy and management of underlying conditions 4, 6
Importance of Interdisciplinary Approach
A multidisciplinary team approach is essential for the clinical management of microangiopathic disease, as delays in diagnosis and initiation of therapy are common due to the low incidence and variable presentation of these diseases 3.