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What is Wegener’s granulomatosis?
Wegener’s granulomatosis is a distinct form of vasculitis (inflammation of the blood vessels). It affects middle-aged individuals of both genders equally and rarely affects children. It is an autoimmune disorder that mostly affects the upper respiratory tract, the lungs, and the kidneys. Other organs affected in this condition include the joints, the eyes, the skin, and the central nervous system (brain and spinal cord).
What are its main signs and symptoms?
The clinical signs and symptoms are:
- Sinus congestion
- Sore throat
- Coughing with haemoptysis (coughing up blood)
- Generalised muscle pain and weakness
- Nausea
- Loss of appetite
- Kidney dysfunction
- Blood in urine
What are the main causes?
The exact cause of the condition is not yet clear, although, certain factors are thought to increase the risk for the condition. Wegener’s granulomatosis occurs due to the frequent high presence of antibodies that are targeted against substances called as neutrophilic peptides. These peptides are a type of proteins. Genetics is also thought to play a role in the development of the disorder. Certain infections have also been suspected to cause Wegener’s granulomatosis, but no specific infectious agents have yet been identified.
How is it diagnosed and treated?
The diagnostic evaluation of Wegener’s granulomatosis includes chest X-rays, biopsies of the kidneys, the lungs, and the upper respiratory tract, along with bronchoscopy. Urine analysis helps to detect presence of proteins in the urine. Complete blood count (CBC) is used to detect anaemia, and analysis of serum electrolytes is done to determine low sodium levels. Other frequent but non-specific laboratory findings are an increase in the number of white blood cells and platelet count (cells in the blood), along with, an elevated erythrocyte sedimentation rate (ESR).
Initial treatment of Wegener’s granulomatosis includes the use of cyclophosphamides and glucocorticoids. The combination of cyclophosphamide and prednisolone helps to provide better treatment and achieve disease remission. However, there can be severe side effects associated with this regimen. The combination of methotrexate and prednisolone is now used in individuals who do not have other immediate life-threatening conditions. Corticosteroids have proven to be beneficial in the early stages of treatment. Nevertheless, corticosteroid dosing must be tapered gradually, as it has no specific role in maintenance therapy. Several other drugs are administered as prophylaxis against the associated side effects. These drugs include proton pump inhibitors, calcium supplements, sulfamethoxazole, and trimethoprim.
