Otolaryngologic Manifestations of Granulomatosis With Polyangiitis

Introduction:

Granulomatosis with polyangiitis (GPA), also formerly known as “Wegener's granulomatosis,” is a rare condition affecting the eyes, nose, ears, throat, kidneys, and respiratory system. It can affect anyone. It is characterized by swelling of the tissues in various body parts like kidneys, blood vessels (vasculitis), and the respiratory tract. Otolaryngologic (ear, nose, and throat) manifestations are the most common and initial symptoms associated with granulomatosis with polyangiitis (GPA).

What Is the Prevalence of Wegner’s Granulomatosis?

• The prevalence of granulomatosis with polyangiitis (GPA) is around three per million people in the general population.

• Otolaryngologic (ear, nose, and throat) manifestations are seen in around 80 to 90 percent of the cases.

• It affects both males and females equally.

• It affects people in the age group of 40 to 65 years old.

What Are the Causes of Wegner’s Granulomatosis?

The exact cause of granulomatosis with polyangiitis is not known, but it is supposed to be caused by the following:

• Autoimmune Causes: It is supposed to be caused by the development of ANCA (anti-neutrophil cytoplasmic antibodies) that is responsible for swelling of the tissues in different body organs. Anti-neutrophil cytoplasmic antibodies (ANCA) react with neutrophils (a component of white blood cells), leading to degranulation (release of cytotoxic contents from the cells) that results in damage to the endothelium (tissues layer lining the body organs).

• Viral Infections: Hepatitis C virus, Epstein-Barr virus (EBV), and cytomegalovirus (CMV) are found to be associated with granulomatosis with polyangiitis in a few cases.

• Bacterial Infections: Bacteria like Staphylococcus aureus are found to be responsible for swelling in the body tissues in some cases.

What Are the Signs and Symptoms of Granulomatosis With Polyangiitis (GPA)?

Otolaryngologist (ear, nose, and throat) manifestations are more common (around 80 to 90 percent of cases) and are initial warning signs seen concerning granulomatosis with polyangiitis (GPA). But it also affects other organs like the eyes, kidneys, skin, and respiratory system. Signs and symptoms associated with Wegener's granulomatosis are categorized as follows:

Otolaryngologist (Ear, Nose, and Throat) Manifestations:

• Hearing loss in one or both ears.

• Chronic otitis media (middle ear infection).

• Mastoiditis (swelling in the mastoid bone due to middle ear infections).

• Runny nose.

• Sinusitis (infection and pain in the sinus- hollow cavity present within the skull bones).

• Nasal discharge.

• Pain or tenderness in the nose and face.

• Epistaxis (nosebleeds).

• Cough or bloody phlegm (blood mixed with sputum) due to swelling in the lungs.

• Shortness of breath.

• Hoarseness of voice (strained voice).

• Change in the pitch of voice.

• Ulcers in the nose.

• Stridor (whistling sound while breathing).

Other Signs and Symptoms:

• General Symptoms: Like weakness and headaches.

• Lower Respiratory Tract Involvement: Like chest pain, tracheal stenosis (narrowing of the trachea or windpipe), and pleural effusion (build-up of fluid between the tissues lining the lungs and the chest walls).

• Bronchi Involvement: Bronchial stenosis (narrowing of the bronchial tube that carries air from the windpipe to the lungs).

• Kidney Disorders: Like glomerulonephritis (swelling of the glomeruli-filters in the kidney).

• Eye Abnormalities: Like scleritis (swelling of the sclera-white part of the eye) and conjunctivitis (swelling of the conjunctiva-transparent membrane that lines the eyelid).

• Skin Manifestations: Skin infections like nodules (abnormal tissue growth) and papules (raised tiny bumps) are seen mostly in lower limbs.

• Nerve Disorders: Like cranial neuropathies (damage to the nerve supplying the brain), cerebritis (swelling of the cerebrum-part of the brain), and seizures (disturbance in the nerve cell activity of the brain).

• Muscle Disorders: Like pain in the joints (arthralgia) and muscles (myalgia).

• Heart Involvement: Like pericarditis (swelling of the pericardium-outer covering of the heart) and coronary arteritis (swelling of the coronary arteries).

What Are the Diagnostic Techniques Used for Granulomatosis With Polyangiitis?

• History and Clinical Examination: A detailed history of the signs and symptoms presented by the patients is recorded. Clinical examination is done by an ENT (ear, nose, and throat) specialist to rule out the sites and extent of Wegner’s granulomatosis disease involvement.

• Biopsy: A biopsy is done to collect the sample of tissues in the affected areas like skin, nasal tissue, and lungs to rule out the exact diagnosis.

• Chest-X ray: It is done to check for the presence of tracheal stenosis (narrowing of the trachea or windpipe) and pleural effusion (build-up of fluid between the tissues lining the lungs and the chest walls).

• Blood Test: A blood test is done to check for the presence of antineutrophil cytoplasmic antibodies (ANCA), confirming the diagnosis of Wegener's granulomatosis.

• Renal Function Panel Test: It is done to rule out kidney dysfunction.

What Is the Treatment of Granulomatosis With Polyangiitis?

Management of granulomatosis with polyangiitis is based on the organ involved, site, and extent of the disease:

Medicinal Therapy Is Divided Into Two Phases:

1) Induction Phase:

Severe Cases: A combination of corticosteroid drugs with immunosuppressant drugs like Cyclophosphamide or Rituximab is given in patients with critical organ involvement. It is effective in life/organ-threatening diseases. Pulse steroid therapy (high-dose steroids given intravenously in a short period) for three days before starting glucocorticoids in patients is recommended.

Less Severe Cases: Corticosteroids in combination with Methotrexate are given initially.

2) Maintenance Phase:

Maintenance Therapy: It is started after the induction phase (3 to 5 months). The patient is on maintenance therapy for 12 to 36 months to avoid relapse. Methotrexate, Azathioprine, and Rituximab are the drugs found to be effective in maintenance therapy.

Plasmapheresis: Plasmapheresis (removal of plasma from blood) is done in patients with kidney dysfunction or pulmonary hemorrhage (bleeding from the lungs). It is recommended for patients in whom medicinal therapy fails.

Can Granulomatosis With Polyangiitis Recur After the Treatment?

Unfortunately, the chances of relapse (recurrence) of the disease are high even after the effective treatment of granulomatosis with polyangiitis.

What Are the Complications Associated With Granulomatosis With Polyangiitis?

• Infections.

• Permanent loss of vision.

• Chronic kidney disease.

• Permanent hearing loss.

• Cancer (due to side effects of immunosuppressant drugs).

Conclusion:

Granulomatosis with polyangiitis is a life-threatening disorder that involves multiple body organs. Otolaryngologic (ear, nose, and throat) manifestations are the most initial warning signs of granulomatosis with polyangiitis. Early diagnosis and management of the condition are crucial to prevent worsening symptoms associated with the disease. Any delay in diagnosis and management of the disease can lead to severe complications and death due to multiple organ failures.