Granulomatosis with polyangiitis is also known as granulomatosis polyangiitis (GPA) or microscopic polyangiitis. The cause of inflammatory changes resulting in necrosis of blood vessels is yet not well defined. Published data suggests the disease could be autoimmune condition since 92% patient suffering with GPA had positive anti-neutrophilic cytoplasmic antibodies (ANCA)
The clinical manifestation of disease is associated with microscopic necrotizing inflammation of small and medium size blood vessels. The clinical or pathological evidence of necrotizing granulomatous inflammation may or may not be observed in biopsy tissue or during clinical examination. Granulomatosis Polyangiitis gets confused with malignant lymphomas namely lymphomatoid granulomatosis and lethal midline granuloma. Necrotizing vasculitis was referred as polyarteritis nodosa in articles that were published in journal of pathology before 1950.
Definition of Granulomatosis polyangiitis
Granulomatosis polyangiitis (GPA) is a disorder of the vascular system. The condition most often affects upper respiratory tract mucosa and subcutaneous tissue. The condition causes vasculitis and granulomatous lesions that is spread within subcutaneous tissue. Vasculitis is inflammatory condition of small and medium size blood vessels. Thus, the condition is known as granulomatosis with polyangiitis (GPA). Following are some of the features of Granulomatosis Polyangiitis. Majority of patients of GPA suffer with symptoms involving ear, nose and throat. Published article suggests GPA can caused life threatening condition like subglottic stenosis.
Signs and Symptoms of Granulomatosis Polyangiitis.
Granulomatosis Polyangiitis in majority of cases affects the upper part of the respiratory track. The individual become prone to repeated frequent infection mostly caused by bacteria and viruses. The condition causes granuloma formation and vascular inflammation. Granuloma is form by collection of immune cells that aggregates in subcutaneous tissue of major organ system like respiratory, kidney and gastrointestinal system. The chronic condition often needs long term immunosuppression therapy to prevent exacerbation of symptoms of Granulomatosis Polyangiitis. The frequent area affected by Granulomatosis Polyangiitis is sinus along with kidneys.
Some of the major generalize symptoms of granulomatosis polyangiitis are as follows:
- Loss of weight
- Loss of appetite
- Persistent fever
Most initial symptoms caused by Granulomatosis Polyangiitis are caused by micro and macroscopic granulomatous lesioning as well as vasculitis of blood vessels of subcutaneous tissue or mucosal layer of upper respiratory track. The specific systemic symptoms include:
- Continuously running nose
- Formation of nasal crusts and sores
- Facial or nasal pain
- Nose bleeding with unusual discharge
- Bloody phlegm cough
- Chest discomfort
- Shortness of breath
- Middle ear inflammation
Other Possible Symptoms of Granulomatosis Polyangiitis That Are Not So Common Include:
- Pleural effusion
- Inflammation of eyes without loss of vision
- Arthritis or joint pains
- Skin sores
- Kidney inflammation
- Muscle pain
Epidemiology of Granulomatosis Polyangiitis
Granulomatosis Polyaangiitis is a rare disease. Few published data suggest, as low as 7-20 cases per million of population per year occur in any particular geographical area. Also, this disease is the rarest in Japanese and Africans. Published study involving 1491 Polish patients suggest average annual incidence of GPA in Poland was 7.7/1,000,000.
Prognosis of Granulomatosis Polyangiitis
The survival rate of patient suffering with Granulomatosis Polyarteritis (GPA) for 5 years is over 80% when treated with corticosteroids and cyclophosphamide. Long term complication includes pneumothorax, pleural effusion, kidney failure, loss of hearing and even deafness. The study published by French Vasculiitis Study group suggests 5-year survival rate was 72.2% for patients diagnosed before 1980 and 94.5% after 2010.
The first clinical description of granulomatosis with polyangiitis.
[Article in Spanish; Abstract available in Spanish from the publisher]
Rev Med Inst Mex Seguro Soc. 2017 May-Jun;55(3):380-381.
Granulomatosis with polyangiitis causing subglottic stenosis-two cases and their management.
Blackabey V1, Gan RWC1, Buglass H1, Kaul V1, Ward VMM1., AME Case Rep. 2018 Apr 30;2:17.
Mortality in systemic necrotizing vasculitides: A retrospective analysis of the French Vasculitis Study Group registry.
Jardel S1, Puéchal X2, Le Quellec A3, Pagnoux C2, Hamidou M4, Maurier F5, Aumaitre O6, Aouba A7, Quemeneur T8, Subra JF9, Cottin V10, Sibilia J11, Godmer P12, Cacoub P13, Fauchais AL14, Hachulla E15, Maucort-Boulch D16, Guillevin L2, Lega JC17; French Vasculitis Study Group (FVSG).
Autoimmun Rev. 2018 Jul;17(7):653-659. doi: 10.1016/j.autrev.2018.01.022. Epub 2018 May 3.
Successful treatment of granulomatosis with polyangiitis with hydropneumothorax using corticosteroids and immunosuppressant.
Shi XH1, Zhang YF1, Lu YW1., Exp Ther Med. 2017 Jun;13(6):3586-3590. doi: 10.3892/etm.2017.4440. Epub 2017 May 8.