Granulomatosis with Polyangiitis (Wegener Granulomatosis)- GPA:

Question 1

Granulomatosis with Polyangiitis (Wegener Granulomatosis)- GPA:

Involved sites:

Answer 1

Question 2

Granulomatosis with Polyangiitis (Wegener Granulomatosis)- GPA:

three different clinical patterns highlight

Answer 2

The disease can involve almost every organ system in the body

1. With classic Wegener granulomatosis, patients initially show involvement of the upper and lower respiratory tract; if the condition remains untreated, then renal involvement often rapidly develops (generalized Wegener granulomatosis) upper and lower respiratory tract; if the condition remains untreated, then renal
2. Limited GPA is diagnosed when there is involvement of the respiratory system without rapid development of renal lesions
3. One subset of patients exhibits lesions primarily of the skin and mucosa, a disease, systemic involvement develops slowly condition termed superficial Wegener granulomatosis. In this form of the disease, systemic involvement develops slowly

These three different clinical patterns highlight the variability of the clinical aggressiveness that can occur in patients with GPA.

• Patients with lower respiratory tract involvement may be asymptomatic, or they may have dry cough, hemoptysis, dyspnea, or chest pain
• Renal involvement usually occurs late in the disease process and is the most frequent cause of death. The glomerulonephritis results in proteinuria and red blood cell casts
• Occasionally, the eyes, ears, and skin also are involved
• Purulent nasal drainage, chronic sinus pain, nasal ulceration, congestion, and fever are frequent findings from upper respiratory tract involvement Persistent otitis media, sore throat, and epistaxis also are reported

Question 3

Granulomatosis with Polyangiitis (Wegener Granulomatosis)- GPA:

Clinical Features GPA:

Answer 3

• The reported prevalence of oral lesions varies widely with oral involvement representing the initial presentation in 2% of affected patients – but when present is characteristic
• The most characteristic oral manifestation is strawberry gingivitis
• This distinctive but uncommon pattern of gingival alteration appears to be an early manifestation of GPA and has been documented before renal involvement in most cases
• The affected gingiva demonstrates a florid and granular hyperplasia which is red to purple with many petechiae, and usually limited to the attached gingiva
• The surface forms numerous short bulbous projections, which are hemorrhagic and friable; this red, bumpy surface is responsible for the strawberry-like appearance

Question 4

Granulomatosis with Polyangiitis (Wegener Granulomatosis)- GPA:

Oral ulceration:

Answer 4

• Oral ulceration also may be a manifestation
• These lesions are clinically nonspecific and may occur on any mucosal surface
• In contrast to the gingival changes, the oral ulcerations are diagnosed at a later stage of the disease, with more than 60% of the affected patients demonstrating renal involvement

Question 5

Granulomatosis with Polyangiitis (Wegener Granulomatosis)- GPA:

Other less common orofacial mani- festations:

Answer 5

• Other less common orofacial manifestations include facial paralysis, labial mucosal nodules, sinusitis- related toothache, arthralgia of the temporomandibular joint (TMJ), jaw claudication, palatal ulceration from nasal extension, oral-antral fistulae, and poorly healing extraction sites
• Enlargement of one or more major salivary glands from primary involvement of the granulomatous process also has been reported
• The glandular involvement also appears early in the course of the disease and may lead to early diagnosis and treatment

Question 6

Granulomatosis with Polyangiitis (Wegener Granulomatosis)- GPA:

Oral Mana- gement:

Answer 6

• Once a diagnosis of GPA is established, it is important to perform a thorough oral evaluation to identify any potential infectious sources that should be addressed before instituting immunosuppressive therapy
• Intralesional steroid injections (triamcinolone acetonide 10 mg/ mL) may assist in the prompt resolution of gingival disease. During induction therapy, only emergency dental treatment should be performed
• Daily antimicrobial mouth rinses may be helpful during induction and maintenance therapy During remission, frequent recall and prophylaxis should be performed
• Before any invasive procedure, the managing physician should be consulted
• Antibacterial prophylaxis and corticosteroid supplementation should be employed as required
• If oral lesions suggestive of relapse appear, immediate specialist referral is indicated