Presentation | Medibuddy
16th January 2024

Presentation

As this condition demonstrates multi-organ system involvement, people may present with a wide variety of clinical symptoms and signs. Normally, at least two organ systems are involved at the time of diagnosis, with 90% of patients with GPA experiencing symptoms and signs indicating involvement of the respiratory tract consistent with diagnoses such as LRTI or pneumonia. The table below summarises the different systems that can be involved with some of the associated signs and symptoms that may be present.

Affected organ/system Symptoms Signs
Eyes Double vision, pain, blurry vision (as part of a presentation of scleritis or episcleritis) Erythema, corneal ulcerations, reduced visual acuity, retinal haemorrhage.
Nervous system Headaches, seizures (rare), altered limb sensation, e.g., burning. Focal weakness, peripheral sensorimotor neuropathy on examination.
Upper respiratory tract (ear, nose, throat) Ear and/or sinus pain, hearing loss, hoarse voice. Epistaxis, nasal discharge, crusting, perforated tympanic membrane, saddle nose deformity.
Lower respiratory tract Shortness of breath, cough, chest pain, haemoptysis (due to alveolar haemorrhage). Stridor, wheeze, focal dullness and crackles on examination, reduced air entry on auscultation.
Skin Pain/discomfort/itching over lesion sites. Lower limb purpura, subcutaneous nodules, oral ulcers.
Musculoskeletal Joint and muscle pain and/or stiffness. Joint swelling and muscle weakness, signs of DVT.
Other Fatigue, night sweats. Fever, weight loss, asymptomatic haematuria (as part of a presentation of glomerulonephritis).

Table 1: Summary table of the most common organs/systems affected in GPA, with associated signs and symptoms that can be seen in GPA. 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).

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