musculoskeletal and connective tissue disorders
Granulomatosis with Polyangiitis (GPA)
Symptoms and Signs of GPA
Onset of granulomatosis with polyangiitis may be insidious or acute; the full spectrum of the disease may take years to evolve. Some patients present initially with upper and lower respiratory tract symptoms; at some point later, the kidneys are affected. In other patients, onset of systemic manifestations is relatively acute; several organs and systems, such as the upper respiratory tract, peripheral nervous system (causing Multiple Mononeuropathy [mononeuritis multiplex]), kidneys (causing glomerulonephritis), and lower respiratory tract (causing hemorrhage, lung nodules, cavities, or a combination), are simultaneously affected. Upper respiratory tract:Sinus pain, serosanguineous or purulent discharge, and epistaxis may occur. The mucosa appears granular (like cobblestones) and is friable; ulcers, thick dark crusts, and septal perforation are common. Nasal chondritis can occur with swelling, pain, and collapse of the nasal bridge (saddle nose). Patients may report recurrent sinusitis that has responded inadequately to multiple antibiotic regimens and has required one or more sinus operations before diagnosis. Secondary infections (eg, due toStaphylococcus aureus) may develop. Subglottic stenosis may develop, causing symptoms such as pain in the larynx, hoarseness, dyspnea, wheezing, and stridor. Ears:Otitis, sensorineural hearing loss, vertigo, and chondritis may occur. The middle ear, inner ear, and mastoids are often affected. Eyes:Eyes may appear red and swollen. Nasolacrimal duct inflammation and obstruction, conjunctivitis, scleritis, uveitis, or retinal vasculitis may also occur. Inflammatory infiltrates in the retro-orbital space (orbital pseudotumor) can cause proptosis, compression of the optic nerve, and blindness. Extension into the extraocular muscles leads to diplopia. If serious eye symptoms develop, evaluation and treatment are required immediately to prevent permanent vision loss. Lower respiratory tract:Respiratory manifestations are common. Inflammation of the major bronchi and branches can cause localized wheezing, postobstructive pneumonia, and atelectasis. Single or multiple pulmonary nodules, with or without cavitation, and parenchymal infiltrates, sometimes cause symptoms, such as chest pain, shortness of breath, and productive cough. Dyspnea with bilateral infiltrates, with or without hemoptysis, may indicate alveolar hemorrhage and must be evaluated immediately. Heart:Coronary artery disease may occur but rarely. Musculoskeletal system:Patients frequently present with myalgias, arthralgias, or nonerosive inflammatory arthritis. Skin:Palpable purpura, tender subcutaneous nodules, papules, livedo reticularis, or ulcers may develop. Nervous system:Vasculitis may cause ischemic peripheral neuropathy, brain lesions, or extension of inflammation into neural tissue from contiguous sites. Lesions that originate in the sinuses or middle ear may extend directly to the retropharyngeal area and base of the skull, leading to cranial neuropathy, proptosis, diabetes insipidus, or meningitis. Symptoms and signs of glomerulonephritis develop. Urinary sediment is frequently abnormal, and serum creatinine may increase rapidly. Edema and hypertension may result. Rapidly Progressive Glomerulonephritis (RPGN) , which is life threatening, can develop. Venous system:Deep venous thrombosis can affect the lower extremities mostly when granulomatosis with polyangiitis is active. Other organs:Occasionally, an inflammatory mass occurs in the breasts, kidneys, prostate, or other organs. 