Introduction:

Allergic angiitis and granulomatosis, also known as Churg-Strauss syndrome (CSS), is a rare autoimmune vasculitis characterized by inflammation of small- to medium-sized blood vessels. This complex disorder primarily affects the respiratory tract, skin, and peripheral nerves, manifesting with a wide range of clinical features. This article aims to provide an overview of allergic angiitis and granulomatosis, including its etiology, clinical manifestations, diagnosis, and management.

Etiology:

The exact cause of allergic angiitis and granulomatosis remains unclear, but it is believed to involve an aberrant immune response triggered by environmental or genetic factors in predisposed individuals. Eosinophilic infiltration and immune complex deposition within blood vessel walls contribute to vascular inflammation and tissue damage.

Clinical Manifestations:

Clinical features of allergic angiitis and granulomatosis can vary widely but often include:

• Asthma: The majority of patients with CSS have a history of asthma, which may precede the onset of vasculitis.
• Systemic Symptoms: Patients may experience fever, fatigue, weight loss, and malaise.
• Skin Involvement: Skin manifestations such as purpura, nodules, livedo reticularis, and urticaria may occur.
• Respiratory Symptoms: Chronic sinusitis, rhinitis, cough, dyspnea, and pulmonary infiltrates are common respiratory manifestations.
• Neurological Involvement: Peripheral neuropathy, mononeuritis multiplex, and CNS vasculitis may result in sensory deficits, weakness, and neuropathic pain.
• Renal Involvement: Glomerulonephritis and renal impairment can occur in some cases.

Diagnosis:

Diagnosis of allergic angiitis and granulomatosis relies on a combination of clinical findings, laboratory tests, imaging studies, and histopathological examination. Key diagnostic criteria include the presence of asthma, eosinophilia, and systemic vasculitis involving multiple organs. Additional investigations may include:

• Blood Tests: Complete blood count with differential, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and serological markers such as antineutrophil cytoplasmic antibodies (ANCA).
• Imaging: Chest X-ray, computed tomography (CT) scans, and pulmonary function tests to assess respiratory involvement.
• Biopsy: Tissue biopsy (e.g., skin, lung, nerve) demonstrating vasculitis, eosinophilic infiltration, and granuloma formation.

Management:

Treatment of allergic angiitis and granulomatosis typically involves immunosuppressive therapy to suppress inflammation and prevent disease progression. Commonly used medications include:

• Corticosteroids: High-dose corticosteroids, such as prednisone, are often initiated initially to induce remission.
• Immunosuppressants: In cases of severe or refractory disease, immunosuppressive agents such as cyclophosphamide, azathioprine, or methotrexate may be added.
• Biologic Therapies: Biological agents targeting specific immune pathways, such as rituximab (anti-CD20 monoclonal antibody), may be considered in select cases.

Prognosis:

The prognosis of allergic angiitis and granulomatosis varies depending on the extent and severity of organ involvement, response to treatment, and presence of complications such as end-organ damage or infections. Early diagnosis and prompt initiation of immunosuppressive therapy are associated with better outcomes and improved long-term survival.

Conclusion:

Allergic angiitis and granulomatosis, although rare, is a serious autoimmune vasculitis syndrome characterized by multi-organ involvement and eosinophilic inflammation. Timely recognition and appropriate management are essential to prevent disease-related complications and improve patient outcomes.

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