Wegener’s Granulomatosis - Causes, Symptoms, Diagnosis, and Treatment

Introduction

Wegner’s granulomatosis is also known as granulomatosis with polyangiitis (GPA), which causes blood vessels in the throat, sinuses, nose, kidneys, and lungs to become inflamed. The condition belongs to a group of blood vessel diseases called vasculitis (inflammation and damage of the blood vessels).

The condition affects the flow of blood to the organs and also their working, by forming granulomas or areas of inflammation. Wegener’s granulomatosis can lead to full recovery with timely diagnosis and treatment. However, a delay or absence of treatment can cause a life-threatening situation.

What Are the Causes of Wegener's Granulomatosis?

The actual cause of Wegener’s granulomatosis is not known. Research-based evidence shows that the immune system plays a vital role in the development of this condition, causing inflammation of the tissues or blood vessels, and further damaging them. The condition can occur at any age and mostly affects people aged 60 to 65.

What Are the Symptoms of Wegener's Granulomatosis?

The symptoms of Wegener’s granulomatosis include

• Fever.

• Weight loss.

• Muscle pain (myalgia).

• Pain in multiple joints (polyarthralgia).

• Cough with thick, bloody mucus, occasionally.

• Wheezing.

• Shortness of breath.

• Blood in urine.

• Fatigue.

• Numbness in toes, fingers, and limbs.

• Nasal discharge with crust from the nasal cavity, sinus infection, nose bleed, stuffiness.

• Pain or burning sensation, redness in eyes, and vision problems.

• Rash, bruising, and skin sores.

• Heart problem, middle ear infection, or otitis media.

• Inflammation of the kidneys.

• Facial pain.

How Is Wegener's Granulomatosis Diagnosed?

Diagnostic Criteria for Wegener’s Granulomatosis:

According to the American College of Rheumatology, the diagnostic criteria for Wegner’s granulomatosis are as follows:

• Chest X-rays show infiltrates, nodules, and cavities.

• Nasal pain, inflammation, along with nasal discharge, and oral ulcers.

• Biopsy reveals granulomatosis and inflammation.

• Kidneys show red cell casts (bleeding in kidneys) and blood in urine or hematuria.

Following are the diagnostic procedures for Wegener’s granulomatosis:

1. Laboratory Tests

Blood Tests:

• Kidney function test.

• High level of C-reactive protein or SED (sedimentation) rate or high erythrocyte sedimentation rate to look for signs of inflammation.

• Hemoglobin test to rule out anemia (lack of enough healthy red blood cells in the blood).

• Look for the presence of anti-neutrophil cytoplasmic antibodies in the blood.

Urine Test: The presence of red blood cells and high protein levels in the urine is checked in urine examination to rule out the effects of disease on the kidneys.

Biopsy: In this procedure, a sample is taken from the kidneys and lungs to be examined under the microscope to confirm the diagnosis of Wegener’s granulomatosis. The tissues from the infected area may appear inflamed with non-cancerous cells, necrosis centrally, and giant cells and histocytes in the surroundings.

2. Imaging Tests: Magnetic resonance imaging (MRI) and computed tomography scans (CT scans). MRI is more precise than CT and reveals malformations and blood flow in the vessels. It is also much safer than other radio diagnostic procedures, as it does not produce radiation and therefore, does not induce the formation of cancer cells. A CT scan, conversely, helps detect thickening of the pulmonary artery that runs from the heart to the lungs, pleural or lung wall thickening, and bronchial wall thickening.

What Is the Management of Wegener's Granulomatosis?

Wegener's granulomatosis is a severe, life-threatening condition, and its management is done with a heavy dose of immunosuppressive drugs, depending on the severity of the condition.

Initially, the patient is given corticosteroids, such as Prednisone, combined with other immunosuppressive drugs, such as Methotrexate, Rituximab, Cyclophosphamide, and Azathioprine.

These drugs are effective in treating this condition, but they may have some side effects also. Usually, the doctor prescribes these drugs to prevent relapse for a year or two (12 to 24 months).

Additionally, some medicines are also prescribed to treat Wegener’s disease, such as folinic or folic acid along with Methotrexate, medication for preventing bone loss caused by Prednisone, and various antibiotics for treating lung infections.

When to See a Doctor?

One must consult the doctor if;

• They have shortness of breath and chest pain.

• One has blood in urine.

• Blood in sputum is present.

If other symptoms of Wegener’s disease are experienced, one must consult the healthcare provider immediately.

What Are the Complications of Wegener's Granulomatosis?

The complications associated with Wegener’s granulomatosis along with the throat, nose, sinuses, lungs, and kidney, also affect other organs in the body. These include:

• Formation of blood clots in deep veins, mainly in lower limbs or legs.

• Saddling or loss of height in the nasal bridge due to weakened cartilage.

• Pulmonary hemorrhage.

• Bleeding in the gut.

• Myocarditis or inflammation in the heart muscles (myocardium).

• Pericarditis or inflammation in the heart walls,.

• Cranial neuropathy (damage to nerves of the brainstem or brain).

• Hearing loss.

• Kidney damage.

• Skin scarring.

What Is the Differential Diagnosis for Wegner's Granulomatosis?

The differential diagnosis for Wegner’s granulomatosis is broad, as this condition could be mimicked by various other conditions that need to be ruled out.

• Sarcoidosis (a condition causing accumulation of inflammatory cells in various body parts).

• Amyloidosis (amyloid protein build-up in the organs).

• Systemic lupus erythematosus (an autoimmune disease affecting various organs in the body).

• Rheumatoid arthritis ( an inflammatory disorder of joints).

• Drug-induced ANCA-associated vasculitis.

• Polyarteritis nodosa (an inflammatory disease of blood vessels).

• Microscopic polyangiitis (an inflammation of small blood vessels).

• Goodpasture syndrome (an acute illness that affects kidneys and lungs).

• Churg-Strauss syndrome (a disorder causing blood vessel inflammation, thus restricting blood flow into tissues and organs).

• Renal-limited vasculitis (a disease that causes acute renal insufficiency).

• Henoch Schonlein purpura (bleeding and inflammation of small blood vessels).

• Mixed cryoglobulinemia.

• Castleman's disease (a condition that causes overgrowth of lymph nodes).

• Lymphomatoid granulomatosis (it is caused by the Epistein-Barr virus.

• Carcinomatosis (a condition in which cancer cells spread from the original tumor to other parts of the body to form many other tumors)

• Lymphomas (these are the cancers occurring in the lymphatic system).

• Mycobacterial infections.

• Infective endocarditis ( an infection of the heart's inner lining called endocardium).

• Disseminated gonococcal infection (it spreads from sexually transmitted bacteria).

• Sepsis (a serious complication due to an infection in the body).

• Idiopathic pulmonary alveolar hemorrhage (rare disease found in children).

• Streptococcal pneumonia with glomerulonephritis (a rare kidney disease resulting from streptococcal infection).

• Disseminated fungal infections.

• Amphetamines drug toxicity.

• Intranasal cocaine drug toxicity.

• Levamisole drug toxicity.

Conclusion

Wegener’s granulomatosis is a fatal disease that causes damage to the internal organs, and a person with severe symptoms can die without treatment. However, with timely diagnosis and treatment, the disease can be managed successfully. Though relapse may be experienced by a person, it requires the same treatment as a newly diagnosed case. Therefore, one must report the symptoms of Wegener’s granulomatosis to the doctor immediately for timely intervention, which could be life-saving.