Distinguishing Clinical Features of GPA and EGPA
Statement (a) is correct: systemic symptoms occur in more than 50% of cases, predominantly in GPA. GPA commonly presents with constitutional symptoms including fever, malaise, and weight loss in the majority of patients, whereas EGPA's systemic manifestations (fatigue, weight loss, myalgia, arthralgia) are present but less emphasized as predominant features 1, 2, 3.
Analysis of Each Statement
Statement A: Systemic Symptoms (>50% of cases, mainly in GPA) - CORRECT
- GPA characteristically presents with systemic symptoms including fever, malaise, and weight loss in the majority of patients, with non-specific systemic symptoms occurring in >95% of those with active disease 2, 3
- EGPA does manifest systemic symptoms (fatigue, weight loss, myalgia, arthralgia), but these are secondary to the disease's defining respiratory and eosinophilic features 1
- The clinical presentation of GPA ranges from subacute nonspecific illness to rapidly progressive disease with prominent constitutional symptoms 2
Statement B: Ocular Involvement More Frequent in EGPA - INCORRECT
- Ocular involvement is actually MORE common in GPA, not EGPA 2
- GPA frequently presents with scleritis and other ocular manifestations as part of its upper respiratory tract involvement 2
- EGPA guidelines do not emphasize ocular involvement as a characteristic feature, focusing instead on asthma, eosinophilia, and peripheral neuropathy 1
Statement C: Peripheral Neuropathy Less Frequent in EGPA - INCORRECT
- This statement is completely backwards: peripheral neuropathy is MUCH MORE common in EGPA than GPA 1
- Peripheral neuropathy occurs in 50-70% of EGPA patients, typically presenting as mononeuritis multiplex with sensory and sometimes motor deficits 1
- While GPA can cause peripheral neuropathy, it is not among its most characteristic features 2, 4
- Peripheral neuropathy is one of the defining features used in EGPA classification criteria, receiving positive scoring in the 2022 ACR-EULAR criteria 1
Statement D: Sinusitis More Common in EGPA - INCORRECT
- Sinusitis is actually MORE characteristic of GPA, not EGPA 2, 4
- GPA involves the nasal cavity and paranasal sinuses in 85-100% of cases, with chronic rhinosinusitis, nasal crusting, stuffiness, and epistaxis being common presenting symptoms 2, 4
- Upper respiratory tract involvement occurs in 70-100% of GPA cases and is often the first clinical manifestation 4
- While EGPA does include chronic rhinosinusitis with nasal polyps as part of its ENT manifestations, this is secondary to the asthma complex rather than a primary vasculitic feature 1
- Paranasal sinus abnormalities are included in both diseases' classification criteria, but are far more prominent and severe in GPA 1, 2
Statement E: Asthma is a Main Feature of GPA - INCORRECT
- This is completely wrong: asthma is the hallmark of EGPA, not GPA 1
- The vast majority (>90%) of EGPA patients are affected by asthma, which usually arises in adulthood and tends to worsen over time 1
- Asthma is so characteristic of EGPA that it receives +3 points in the 2022 ACR-EULAR classification criteria (termed "obstructive airway disease") 1
- GPA does not characteristically present with asthma; its respiratory manifestations include pulmonary nodules, infiltrates, and alveolar hemorrhage rather than obstructive airway disease 2, 3
Key Distinguishing Features Summary
GPA is characterized by:
- Upper respiratory tract involvement (sinusitis, nasal crusting, epistaxis) in 85-100% 2, 4
- Pulmonary nodules and infiltrates 2, 3
- Glomerulonephritis 3, 4
- PR3-ANCA positivity in 80-90% 2
- Prominent systemic symptoms 2, 3
EGPA is characterized by: