What are the key clinical features that suggest a diagnosis of Granulomatosis with Polyangiitis (GPA) in a primary care setting?

In a primary care setting, Granulomatosis with Polyangiitis (GPA) is an ANCA-associated vasculitis that can present with a wide range of non-specific symptoms, often making early diagnosis challenging due to its insidious onset and multi-system involvement (Chevet et al., 2023).

Key clinical features that may suggest GPA involve multiple organ systems:

• Upper Respiratory Tract: Persistent rhinitis, sinusitis, epistaxis, or nasal crusting are common manifestations (Chevet et al., 2023). Patients may also experience nasal bridge collapse (saddle nose deformity) or otitis media (Chevet et al., 2023).
• Lower Respiratory Tract (Lungs): Pulmonary involvement is frequent and can include cough, dyspnoea, haemoptysis, or pleuritic chest pain (Sacoto et al., 2020; Chevet et al., 2023). Imaging may reveal nodules, cavities, or diffuse alveolar haemorrhage (Sacoto et al., 2020).
• Kidneys: Rapidly progressive glomerulonephritis is a serious and common manifestation, often presenting with haematuria and proteinuria, which can lead to renal failure (Chevet et al., 2023).
• General/Systemic Symptoms: Non-specific symptoms such as fever, fatigue, weight loss, and arthralgia are frequently reported (Chevet et al., 2023).
• Skin: Cutaneous manifestations can include palpable purpura, nodules, or ulcers (Chevet et al., 2023).
• Nervous System: Peripheral neuropathy (e.g., mononeuritis multiplex) is a common neurological feature (Chevet et al., 2023).
• Eyes: Ocular involvement can manifest as conjunctivitis, scleritis, or proptosis (Chevet et al., 2023).

Given the varied and often non-specific presentation, a high index of suspicion is required in primary care, especially when patients present with unexplained multi-organ involvement (Chevet et al., 2023).