Granulomatosis

Overview

Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, is a rare autoimmune disorder characterized by inflammation of small and medium-sized blood vessels in various organs including the nose, sinuses, throat, lungs, and kidneys. This inflammation can lead to the formation of granulomas, affecting the normal functioning of these organs. Early diagnosis and treatment are crucial as untreated GPA can lead to severe complications and even be life-threatening.

Types of Granulomatosis

1. Limited GPA: Primarily affects the respiratory tract, including the nose, sinuses, and lungs.

2. Severe GPA: Involves multiple organ systems, including the kidneys, leading to renal involvement and potentially renal failure.

Causes of Granulomatosis

• Autoimmune Reaction: The exact cause is not fully understood, but GPA is considered an autoimmune disease where the immune system mistakenly attacks healthy tissues, particularly blood vessels.
• Genetic Factors: Certain genetic predispositions may increase susceptibility.

Risk Factors of Granulomatosis

• Age and Gender: GPA most commonly affects adults, with peak onset between ages 40 and 65. It appears to slightly more frequently affect males than females.
• Genetic Predisposition: Family history of autoimmune diseases may increase the risk.
• Environmental Factors: Exposure to certain environmental triggers may play a role in triggering the disease.

Symptoms of Granulomatosis

Preventions of Granulomatosis

There are no known preventive measures for GPA due to its autoimmune nature and unclear triggers. However, early recognition of symptoms and prompt medical evaluation may help in early diagnosis and management.

Diagnosis of Granulomatosis

• Clinical Evaluation: Based on symptoms, medical history, and physical examination.
• Blood Tests: Including tests for markers of inflammation and autoantibodies.
• Imaging Studies: X-rays, CT scans, or MRI to assess organ involvement.
• Biopsy: Often of the affected tissue (e.g., nasal or kidney biopsy) to confirm diagnosis.

Treatment for Granulomatosis

1. Immunosuppressive Therapy: Corticosteroids and other immunosuppressive medications to reduce inflammation and control the immune response.

2. Rituximab: A monoclonal antibody that targets specific immune cells involved in GPA.

3. Plasma Exchange (Plasmapheresis): In severe cases where organs are significantly affected.

4. Supportive Care: Managing symptoms and complications such as kidney failure or lung disease.

When to seek medical attention ?

• Persistent Symptoms: Especially respiratory symptoms or signs of kidney involvement.
• New or Worsening Symptoms: Including unexplained fever, weight loss, or fatigue.
• Previous Diagnosis: If already diagnosed with GPA, regular follow-up with healthcare providers is essential.