Diagnosis | Medibuddy
16th January 2024

Diagnosis

Although GPA is a rare condition, it should be considered in any patient with atypical or persistent pneumonia. Several classification systems exist but were not intended for diagnostic use; therefore, they have not been included in this section. The main investigations which should be performed as part of confirming a diagnosis are summarised below.

Investigation Findings in keeping with GPA
Urinalysis Microscopic haematuria, proteinuria, and red blood cell casts can be seen in renal involvement.
Bloods Normocytic normochromic anaemia can occur. Creatinine, CRP and/or ESR may be elevated, but serum albumin may be low. LFTs tend to be normal in GPA.

Up to 75% of patients with GPA are c-ANCA/PR3-ANCA positive on immunoassay testing, and in conjunction with typical symptoms and signs, this is usually sufficient for diagnosis. A negative result does not exclude GPA as a diagnosis, and ANCA negative patients can become positive over time.

Imaging Chest CT has higher sensitivity than plain film; cavitating nodules, opacification (particularly alveolar) and pleural effusion may be seen. Depending on the clinical presentation, other area-specific imaging may also be required.
Biopsy of the affected organ This is recommended where possible to ensure confirmation of diagnosis prior to treatment initiation. It is also useful in cases where patients are ANCA negative, but GPA remains suspected. Common sample sites are skin, kidney or lung. In positive cases, skin biopsy shows vasculitis in small skin vessels, kidney biopsy shows glomerulonephritis and occasionally granulomas, and lung biopsy shows granulomatous inflammation.

Table 2: Summary of investigations used in diagnosis of GPA, with explanation of findings. Adapted from: Falk, R., Merkel, P. and King Jr, T. (2022) Granulomatosis with polyangiitis and microscopic polyangiitis: Clinical manifestations and diagnosis, UpToDate. Edited by R. Glassock et al. Available at: https://www.uptodate.com/contents/granulomatosis-with-polyangiitis-and-microscopic-polyangiitis-clinical-manifestations-and-diagnosis?search=gpa&source=search_result&selectedTitle=1~150&usage_type=default&display_rank=1 (Accessed: January 15, 2023) and Molloy, E. (2022) BMJ Best Practice Granulomatosis with polyangiitis. Available at: https://bestpractice.bmj.com/topics/en-gb/327/pdf/327/Granulomatosis%20with%20polyangiitis.pdf (Accessed: January 15, 2023). 

Depending on the symptoms present, other investigations may be needed e.g., lung function or bronchoscopy may be needed to further explore signs like shortness of breath and haemoptysis, to rule out other conditions such as PE.

Moreover, a positive ANCA result on immunoassay can also occur in other disorders, e.g., IBD, infective endocarditis and malignancy. A careful history and examination are needed when considering GPA as a diagnosis, and if there is clinical suspicion of alternative disease/conditions, these should be excluded with further investigations if needed.

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