Granulomatosis with polyangiitis


A vasculitis of small and medium sized vessels characterized by the presence of necrotizing granulomas.

Formerly known as Wegener’s granulomatosis.

Most cases occur between ages 40-60 years.
The disease is more frequent among Caucasians, although all racial and ethnic groups can be affected.
Affects men and women equally.
It is rare with an incidence of 0.4 to 11.9 cases per million person-years and a prevalence of 2.3 to 146 cases per million persons.
Classic triad of organ involvement is that of sinus, respiratory tract, and kidney.

GPA because inflammation of the upper respiratory tract resulting in rhinitis, otitis, subglottic stenosis, and sinusitis.

Organ involvement can be variable, leading to delay in diagnosis due to the incongruous nature of symptoms.

Clinical manifestations are protean.
Paranasal sinuses symptoms or commonly involved in up  to 73% of patients, skin manifestations in 15-50% of patients and is mostly palpable purpura, or less commonly nodules, vesicles or ulcers resembling pyoderma gangrenosum.

Up to 70% of patients with granulomatosis with polyangiitis are chronic nasal carriers of Staphylococcus aureus, with carriers having an eight times increased risk of relapse: considered a type II hypersensitivity reaction.

Acute phase reactants are elevated, and cANCA positivity occurs and 75%-90% of cases.

Diagnostic diagnosis is confirmed with a combination of clinical picture, serpositivity for c-ANCA and biopsy results.

Patients with GPA most commonly have a cytoplasmic ANCA pattern and antibodies directed against proteinase3, located in neutrophils and monocytes.

A small number of patients have a perinucleus staining pattern P-ANCA with antibodies typically directed against myeloperoxidase.33“21

Differential diagnosis includes: Chung-Strauss syndrome, Goodpasture syndrome, and sarcoidosis.

Every patient with suspected granulomatosis with polyangoitid should have cANCA testing, evaluation for sinusitis, and histopathologic examination of biopsy specimens.

Untreated is uniformly fatal.

GPA causes necrotizing granulomatous inflammation in small blood vessels throughout the body, most commonly affecting the capillary beds of the kidneys, lungs, and skin.

Renal and pulmonary involvement can progress rapidly and the diagnosis warrants urgent management.

Rapidly progressive glomerulonephritis occurs in up to 70% the patients with GPA.

commonly affects the lower respiratory tract with lung granuloma, alveolar hemorrhage.

Associated with significantly increased risks of myocardial infarction and ischemic stroke.

Management includes oral steroids.
chronic granulomatous sinusitis due to GPA can lead to bone and cartilage destruction with collapse of the nasal bridge causing a saddlenose deformity.
Dermatological lesions are usually responsive to corticosteroids, cyclophosphamide or both treatments.
With generalized disease pulmonary cavitation and pleural  disease developing over 50% of patients, and glomerulonephritis may develop in almost 80% of cases.
Ocular and orbital involvement occur between 20 and 50% of patients and muscular skeletal, neurological and cardiac disease also may occur.
Rituximab and intravenous immunoglobulin are reserved for refractory disease.
Azothioprine and methotrexatebmay be added as a steroid sparing agent.
With current therapy of immunosuppressive agents five-year survival rate is now greater than 75%-80%.

Relapses occur in more than 50% of patients.

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