Granulomatosis with Polyangiitis
Granulomatosis with polyangiitis is a rare but serious autoimmune disease characterised by inflammation of blood vessels, particularly small- and medium-sized vessels throughout the body. Formerly known as Wegener’s granulomatosis, granulomatosis with polyangiitis often affects the respiratory tract and kidneys, although it can involve many organs. The inflammation can lead to tissue damage, organ dysfunction, and, if left untreated, life-threatening complications.
This condition belongs to a group of disorders known as ANCA-associated vasculitides (AAV), named for the presence of anti-neutrophil cytoplasmic antibodies (ANCAs) found in many affected individuals. Granulomatosis with polyangiitis tends to strike adults aged 40–60 but can occur at any age. Early diagnosis and aggressive treatment with immunosuppressive drugs can help achieve remission and prevent permanent damage.
Key Characteristics
Granulomatosis with polyangiitis is marked by three main pathological features:
Granulomatous inflammation – a type of immune response involving clusters of inflammatory cells
Vasculitis – inflammation of the blood vessel walls
Necrosis – tissue damage resulting from loss of blood supply or immune destruction
These features affect organs such as:
Sinuses, nose, and upper airways
Lungs
Kidneys
Skin, eyes, joints, and nervous system
Symptoms often begin subtly, with fatigue, joint pain, or sinus issues, before progressing to more serious organ involvement.
Epidemiology and Risk
Granulomatosis with polyangiitis is rare, with an estimated annual incidence of 10–20 cases per million people worldwide. Risk factors include:
Middle age (more common in individuals aged 40–65)
Slight male predominance
Caucasian ethnicity (more prevalent in European populations)
Family history of autoimmune disorders (though not directly inherited)
It affects people globally and across all demographics, but detection rates vary due to limited awareness and diagnostic capacity in some regions.
Early Symptoms
Initial symptoms may be vague and resemble common illnesses:
Fatigue and general malaise
Unexplained weight loss
Fever and night sweats
Persistent sinus infections or nasal congestion
Ear pain or hearing loss
Dry cough or shortness of breath
Joint and muscle aches
Because these symptoms mimic those of respiratory infections, sinusitis, or allergies, the condition is often misdiagnosed in its early stages.
Respiratory Involvement
The upper and lower respiratory tracts are commonly affected:
Sinusitis, nasal crusting, or frequent nosebleeds
Saddle-nose deformity due to cartilage destruction
Chronic cough, wheezing, or haemoptysis (coughing up blood)
Lung nodules or infiltrates, visible on chest imaging
These issues can escalate to lung haemorrhage or respiratory failure if not promptly addressed.
Kidney Involvement
One of the most serious aspects of granulomatosis with polyangiitis is glomerulonephritis, or inflammation of the kidneys’ filtering units:
Early signs include blood or protein in the urine
May progress silently to kidney failure
Renal biopsy is often necessary for definitive diagnosis
If untreated, kidney involvement can lead to irreversible damage and the need for dialysis.
Other Organ Systems
The disease may also affect:
Skin – purple spots (purpura), ulcers, or nodules
Eyes – inflammation, dryness, pain, or vision loss
Nerves – tingling, numbness, or muscle weakness
Joints – arthritis-like pain, especially in large joints
Multisystem involvement complicates diagnosis and requires a coordinated approach to treatment.
Diagnosis Challenges
Because of its rarity and non-specific early symptoms, diagnosis is often delayed. The condition must be distinguished from infections, cancers, and other autoimmune diseases. Blood tests, imaging, and biopsy are typically needed for confirmation.
Importance of Early Treatment
Prompt recognition is critical. Without treatment, granulomatosis with polyangiitis can progress rapidly, causing permanent damage or death. With appropriate therapy, however, long-term remission is possible for many patients.
Conclusion | Granulomatosis with Polyangiitis
Granulomatosis with polyangiitis is a complex autoimmune vasculitis that causes systemic inflammation and serious organ damage if left untreated. Its early symptoms often mimic common conditions, making awareness and timely diagnosis vital. Despite its severity, with modern immunosuppressive therapy, granulomatosis with polyangiitis is a manageable condition, and many individuals can achieve lasting remission.
