<unk>  is a form of vasculitis that affects the lungs, kidneys and other organs. Due to its end-organ damage, it can be a serious disease that requires long-term immune suppression.<unk>  is part of a larger group of vasculitic syndromes, all of which feature the presence of an abnormal type of circulating antibody termed ANCAs (antineutrophil cytoplasmic antibodies) that affect small and medium-size blood vessels. Apart from<unk> , this category includes Churg-Strauss syndrome and microscopic<unk> . Although<unk>  affects small and medium-sized vessels, it is formally classified as one of the small vessel vasculitides in the Chapel Hill system.The pathogenesis of<unk>  is currently unknown. However, several hypothesizes have been made to identify possible links associated with this disease, such as bacterial infections, failure of B-cells to downregulate, and T cell dysfunction. The genetic component of<unk>  is not fully known. However, there seems to be a strong correlation between HLA-DPB1 and HLA-DPB2 with<unk> .<unk>  must be differentiated from other diseases that cause purpura, alveolar hemorrhage, fever, arthralgia, myalgia, necrotizing extra-capillary glomerulonephritis, such as microscopic<unk>  and eosinophilic<unk> .The incidence of<unk>  in children ranges form 0.03 to 3.2 per 100,000 per year. The prevalence in the United States is 3 per 100,000 persons. The mean age of diagnosis of<unk>  is 58 years. The disease most commonly presents in patients that are either middle aged or elderly. The mean age of diagnosis in children is between the ages of 4 to 17. Males are more commonly affected with<unk> . The overall male to female ratio is approximately 1.2 to 1. In children females are more commonly seen with the disease.<unk>  typically affects Caucasians. Common risk factors in the development of<unk>  <unk>  are age group of 40-65 years and presence of variant HLA-DPB1*0401 with ANCA. If left untreated,<unk>  may progress to morbidity and mortality. Complications of<unk>  include, vision loss, subglottic manifestations, hearing loss, renal failure and increased infections with prolonged immunosuppressant therapy. Determination of ANCAs can aid in the diagnosis, but positivity is not conclusive and negative ANCAs are not sufficient to reject the diagnosis. Cytoplasmic staining ANCAs that react with the enzyme proteinase 3 (cANCA) in neutrophils (a type of white blood cell) are associated with<unk> . If the patient has renal failure or cutaneous vasculitis, these are the most logical organs to obtain a biopsy from. Rarely, thoracoscopic lung biopsy is required. The symptomatology depends on the system involved. The disease involve ear, nose and throat and cause symptoms of sinusitis, nasal crusting, otitis media, otorrhea, epistaxis. Patients with history of lung involvement presents with hoarseness, Cough, Dyspnea, Stridor, Hemoptysis. Renal involvement presents with cloudy urine with hematuria, edema.The important physical exam findings are ulcerations, palpable purpura and subcutaneous nodules in the skin; conjunctivitis, uveitis, sinusitis, purulent exudate from nares, saddle nose deformity, reduced hearing in the head and neck; pleuritic chest pain and signs of atelectasis in lung examination; and signs of mononeuritis multiplex on neural exam. Laboratory findings consistent with the diagnosis of<unk>  include, anti-neutrophil cytoplasmic antibody, elevated blood urea nitrogen, elevated creatinine serum, erythrocyte sedimentation rate, C reactive protein, proteinuria, microscopic hematuria, and red blood casts. An x-ray may be helpful in the diagnosis of<unk> . Findings on an x-ray suggestive of<unk>  include 4 patterns as multiple nodules with or without cavitation; pulmonary hemorrhage as airspace opacities; reticulations and nodules and wedge-like consolidation in periphery. In most cases, treatment consists of a combination of a glucocorticoid (a steroid) and a cytotoxic medicine. It includes glucocorticoids, cyclophosphamide, Rituximab, Methotrexate, Azathioprine and when needed with plasmapheresis.
Granulomatosis with polyangiitis
Microscopic polyangiitis
Glomerular deposition disease
Polyarteritis nodosa
ANCA associated vasculitis
Thin basement membrane disease
Hypersensitivity vasculitis
Mesoamerican nephropathy