Granulomatosis with polyangiitis

Wegener's granulomatosiscoin lesion, pulmonary
Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis (WG), is a long-term systemic disorder that involves the formation of granulomas and inflammation of blood vessels (vasculitis).wikipedia
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Rituximab

RituxanMabThera(R)'''ituximab
Severe disease is typically treated with a combination of immunosuppressive medications such as rituximab or cyclophosphamide and high-dose corticosteroids to control the symptoms of the disease and azathioprine, methotrexate, or rituximab to keep the disease under control. Less toxic immunosuppressing medications such as rituximab, methotrexate, azathioprine, leflunomide, or mycophenolate mofetil are used.
It is used for non-Hodgkin's lymphoma, chronic lymphocytic leukemia, rheumatoid arthritis, granulomatosis with polyangiitis, idiopathic thrombocytopenic purpura, pemphigus vulgaris, myasthenia gravis and Epstein-Barr virus-positive mucocutaneous ulcers.

Azathioprine

ImuranAzahexalAzasan
Severe disease is typically treated with a combination of immunosuppressive medications such as rituximab or cyclophosphamide and high-dose corticosteroids to control the symptoms of the disease and azathioprine, methotrexate, or rituximab to keep the disease under control. Less toxic immunosuppressing medications such as rituximab, methotrexate, azathioprine, leflunomide, or mycophenolate mofetil are used.
It is used in rheumatoid arthritis, granulomatosis with polyangiitis, Crohn's disease, ulcerative colitis, systemic lupus erythematosus, and in kidney transplants to prevent rejection.

Cyclophosphamide

(C)'''yclophosphamidealdophosphamideCytoxan
Severe disease is typically treated with a combination of immunosuppressive medications such as rituximab or cyclophosphamide and high-dose corticosteroids to control the symptoms of the disease and azathioprine, methotrexate, or rituximab to keep the disease under control.
As an immune suppressor it is used in nephrotic syndrome, granulomatosis with polyangiitis, and following organ transplant, among other conditions.

Uveitis

iritisiridocyclitisanterior uveitis
Typical signs and symptoms include nosebleeds, stuffy nose and crustiness of nasal secretions, and inflammation of the uveal layer of the eye.

Vasculitis

vasculitidesvasculiticvasculitis, central nervous system
Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis (WG), is a long-term systemic disorder that involves the formation of granulomas and inflammation of blood vessels (vasculitis).

Scleritis

Inflammation of the outer layers of the eye (scleritis)
Inflammation of the outer layers of the eye (scleritis) and conjunctivitis are the most common signs of GPA in the eye; involvement of the eyes is common and occurs in slightly more than half of people with the disease.
The disease is often contracted through association with other diseases of the body, such as granulomatosis with polyangiitis or rheumatoid arthritis.

Rapidly progressive glomerulonephritis

Rapidly progressing glomerulonephritiscrescentsglomerular crescent
In 50% of cases, RPGN is associated with an underlying disease such as Goodpasture syndrome, systemic lupus erythematosus or granulomatosis with polyangiitis; the remaining cases are idiopathic.

Saddle nose

saddle deformitysaddle-nose
Typical signs and symptoms of nose or sinus involvement include crusting around the nose, stuffiness, nosebleeds, runny nose, and saddle-nose deformity due to a hole in the septum of the nose.
Saddle nose is a condition associated with nasal trauma, congenital syphilis, relapsing polychondritis, granulomatosis with polyangiitis, cocaine abuse, and leprosy, among other conditions.

Nosebleed

epistaxisnosebleedsnose bleeds
Typical signs and symptoms include nosebleeds, stuffy nose and crustiness of nasal secretions, and inflammation of the uveal layer of the eye. Typical signs and symptoms of nose or sinus involvement include crusting around the nose, stuffiness, nosebleeds, runny nose, and saddle-nose deformity due to a hole in the septum of the nose.

Lung

lungspulmonaryright lung
Damage to the heart, lungs and kidneys can be fatal.
Other rarer conditions may also affect the blood supply of the lung, such as granulomatosis with polyangiitis, which causes inflammation of the small blood vessels of the lungs and kidneys.

Hemoptysis

coughing up bloodhaemoptysishæmoptysis
Rarer causes include hereditary hemorrhagic telangiectasia (HHT or Rendu-Osler-Weber syndrome), Goodpasture's syndrome, and granulomatosis with polyangiitis.

Necrotizing vasculitis

Systemic vasculitissystemic vasculitidesacute necrotizing vasculitis
Granulomatosis with polyangiitis is part of a larger group of vasculitic syndromes called systemic vasculitides or necrotizing vasculopathies, all of which feature an autoimmune attack by an abnormal type of circulating antibody termed ANCAs (antineutrophil cytoplasmic antibodies) against small and medium-size blood vessels.
Examples include giant cell arteritis, microscopic polyangiitis, and granulomatosis with polyangiitis.

Nasal septum perforation

hole in the septum of the nosenasal septum rupturedperforated nasal septum
Typical signs and symptoms of nose or sinus involvement include crusting around the nose, stuffiness, nosebleeds, runny nose, and saddle-nose deformity due to a hole in the septum of the nose.
Much less common causes for perforated nasal septums include rare granulomatous inflammatory conditions like granulomatosis with polyangiitis.

Pulmonary-renal syndrome

Plasmapheresis is sometimes recommended for very severe manifestations of GPA, such as diffuse alveolar hemorrhage and rapidly progressive glomerulonephritis (as seen in pulmonary-renal syndrome).
PRS is most commonly due to ANCA-associated vasculitides (e.g., granulomatosis with polyangiitis) or due to anti-basement membrane diseases (e.g., Goodpasture's syndrome).

Leflunomide

SU101AravaArava (drug)
Less toxic immunosuppressing medications such as rituximab, methotrexate, azathioprine, leflunomide, or mycophenolate mofetil are used.

Lethal midline granuloma

granuloma, lethal midline
The disease is still sometimes confused with lethal midline granuloma and lymphomatoid granulomatosis, both malignant lymphomas.
LMG was thought to be a manifestation of three or four different diseases: the well-characterized disease of granulomatosis with polyangiitis, the ill-defined disorders of polymorphic reticulosis or mid-line malignant reticulosis, and an incompletely defined form of non-Hodgkin's lymphoma.

Plasmapheresis

plasma exchangeplasmaplasmapharesis
Plasmapheresis is sometimes recommended for very severe manifestations of GPA, such as diffuse alveolar hemorrhage and rapidly progressive glomerulonephritis (as seen in pulmonary-renal syndrome). Plasma exchange is also used in severe cases with damage to the lungs, kidneys, or intestines.

Anti-neutrophil cytoplasmic antibody

ANCAanti-neutrophil cytoplasmic antibodiesantineutrophil cytoplasmic antibodies
Granulomatosis with polyangiitis is part of a larger group of vasculitic syndromes called systemic vasculitides or necrotizing vasculopathies, all of which feature an autoimmune attack by an abnormal type of circulating antibody termed ANCAs (antineutrophil cytoplasmic antibodies) against small and medium-size blood vessels. It is now widely presumed that the anti-neutrophil cytoplasmic antibodies (ANCAs) are responsible for the inflammation in GPA.
It has also been shown that up to 70% of patients with granulomatosis with polyangiitis (previously known as Wegener's granulomatosis) are chronic nasal carriers of Staphylococcus aureus, with carriers having an eight times increased risk of relapse.

Urinary cast

urinary castscastsRBC casts
The presence of red blood cells within the cast is always pathological, and is strongly indicative of granulomatosis with polyangiitis, systemic lupus erythematosus, post-streptococcal glomerulonephritis or Goodpasture's syndrome.

Granuloma

granulomatousgranulomasgranulomatous inflammation
Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis (WG), is a long-term systemic disorder that involves the formation of granulomas and inflammation of blood vessels (vasculitis).
For example, granulomas with numerous eosinophils may be a clue to coccidioidomycosis or allergic bronchopulmonary fungal disease, and granulomas with numerous neutrophils suggest blastomycosis, granulomatosis with polyangiitis, aspiration pneumonia or cat-scratch disease.

Hearing loss

deafdeafnesshearing impairment
Long-term complications are common (86%), mainly chronic kidney failure, hearing loss, and deafness.

Friedrich Wegener

The full clinical picture was first presented by Friedrich Wegener (1907–1990), a German pathologist, in two reports in 1936 and 1939, leading to the eponymous name Wegener's granulomatosis or Wegener granulomatosis.
Friedrich Wegener (April 7, 1907, Varel – July 9, 1990, Lübeck) was a German pathologist who is notable for his description of a rare disease now referred to as granulomatosis with polyangiitis.

Microscopic polyangiitis

Apart from GPA, this category includes eosinophilic granulomatosis with polyangiitis (EGPA) and microscopic polyangiitis.
The signs and symptoms of microscopic polyangiitis may resemble those of granulomatosis with polyangiitis (GPA) (another form of small-vessel vasculitis) but typically lacks the significant upper respiratory tract involvement (e.g., sinusitis) frequently seen in people affected by GPA.

White blood cell

leukocyteleukocyteswhite blood cells
Cytoplasmic-staining ANCAs that react with the enzyme proteinase 3 (cANCA) in neutrophils (a type of white blood cell) are associated with GPA.