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Symptoms of Granulomatosis with Polyangiitis

Red rash and inflammation on palms due to granulomatosis with polyangiitis

Visible skin symptoms of granulomatosis with polyangiitis, showing red blotchy rash on the palms of both hands

Symptoms of Granulomatosis with Polyangiitis

The symptoms of granulomatosis with polyangiitis vary widely depending on which organs are affected, but typically include a mix of respiratory, renal, and generalised inflammatory signs. The symptoms of granulomatosis with polyangiitis often start gradually and may initially resemble common illnesses such as sinusitis or the flu, making early diagnosis challenging. However, as the disease progresses, organ damage may become more pronounced, sometimes resulting in life-threatening complications if left untreated.

Granulomatosis with polyangiitis (GPA) is a rare autoimmune vasculitis that causes inflammation of small- and medium-sized blood vessels, particularly in the lungs, kidneys, and upper airways. Granulomas—clusters of inflammatory cells—also form in tissues, further contributing to damage. Because GPA can affect virtually any organ system, the symptoms are diverse and may develop over days, weeks, or even months.

General Symptoms

In the early stages, people often experience non-specific systemic symptoms such as:

Fatigue and low energy

Weight loss without trying

Fever and night sweats

Loss of appetite

Joint and muscle aches resembling arthritis

Mild anaemia or generalised weakness

These symptoms often precede more localised organ involvement and are sometimes mistaken for viral infections or chronic fatigue syndrome.

Upper Respiratory Tract Involvement

The upper respiratory tract is involved in over 90% of cases. Common symptoms include:

Chronic sinusitis or persistent nasal congestion

Bloody or crusted nasal discharge

Nosebleeds (epistaxis)

Nasal septal perforation, which may lead to a saddle-nose deformity

Hearing loss due to eustachian tube dysfunction

Ear pain, fullness, or infections

These symptoms are often recurrent and may resist standard treatment with antibiotics or decongestants, raising suspicion of an underlying inflammatory process.

Lower Respiratory Tract Symptoms

Lung involvement is another hallmark of GPA and may present with:

Chronic dry cough

Shortness of breath

Chest pain, especially on deep breathing

Coughing up blood (haemoptysis)

Wheezing or crackles on examination

Chest X-rays or CT scans may reveal nodules, infiltrates, or cavities—sometimes misinterpreted as infections, tuberculosis, or cancer.

In severe cases, lung haemorrhage can occur, requiring urgent treatment.

Kidney Involvement

Kidney inflammation, or glomerulonephritis, may be silent in early stages but is one of the most serious manifestations:

Blood in the urine (haematuria)

Protein in the urine (proteinuria)

Foamy urine

High blood pressure

Swelling in the ankles or around the eyes

Without treatment, kidney function may deteriorate rapidly, leading to chronic kidney disease or kidney failure.

Eye and Vision Problems

Inflammation may extend to the eyes, resulting in:

Red, painful eyes

Blurred vision or double vision

Dry eyes

Episcleritis or scleritis (inflammation of the white part of the eye)

Vision loss in severe cases

Prompt ophthalmologic assessment is essential to preserve vision in affected individuals.

Skin Lesions

Skin symptoms occur in approximately 30–50% of cases. These may include:

Reddish or purple spots (purpura), particularly on the legs

Ulcers, especially on the extremities

Tender nodules or lumps

Rashes resembling allergic reactions

Skin biopsy may help confirm vasculitis as the cause of these lesions.

Nervous System Symptoms

The nervous system may also be affected, causing:

Peripheral neuropathy (numbness, tingling, or burning in the hands or feet)

Muscle weakness

Cranial nerve palsies

Rarely, stroke-like episodes or central nervous system involvement

Neurological symptoms can be subtle or debilitating, depending on the nerves involved.

Gastrointestinal Involvement

Although less common, the gastrointestinal system can also be affected, leading to:

Abdominal pain

Nausea or vomiting

Gastrointestinal bleeding or ulcers

These symptoms may mimic other GI conditions and complicate diagnosis.

Symptoms in Children

Granulomatosis with polyangiitis is rare in children but presents similarly:

Fever, weight loss, and fatigue

Ear, nose, and throat infections that do not resolve

Joint pain or swelling

Haematuria or proteinuria

Paediatric diagnosis can be particularly challenging due to overlap with common childhood illnesses.

Patterns and Progression

There is no fixed sequence for symptom development:

Some individuals have isolated respiratory symptoms for months before kidney involvement appears

Others may present suddenly with multisystem disease

Flare-ups can alternate with periods of remission

This unpredictable pattern underscores the need for ongoing monitoring and early specialist referral.

Conclusion | Symptoms of Granulomatosis with Polyangiitis

The symptoms of granulomatosis with polyangiitis are diverse, ranging from sinus congestion and joint pain to life-threatening kidney failure or lung haemorrhage. Because the symptoms of granulomatosis with polyangiitis often mimic more common conditions, early recognition requires a high index of suspicion, especially in persistent or multisystem disease. Accurate diagnosis and early treatment are essential to reduce the risk of long-term complications and achieve remission.

[Next: Diagnosis of Granulomatosis with Polyangiitis →]

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