Though any cartilage in the body may be affected in persons with<unk> , in many cases the disease affects several areas while sparing others. The disease may be variable in its signs and symptoms, resulting in a difficult diagnosis which may leads to delayed recognition for several months, years or decades. Joint symptoms are often one of the first signs of the disease with cartilage inflammation initially absent in nearly half the cases. Associated diseases There are several other overlapping diseases associated with<unk> , that should also be taken into account. About one-third of people with<unk>  might be associated with other autoimmune diseases, vasculitides and hematologic disorders. Systemic vasculitis is the most common association with<unk> , followed by rheumatoid arthritis and systemic lupus erythematosus. The following table displays the main diseases in association with<unk> . Class of disease Specific type of disease Vasculitides Granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, polyarteritis nodosa, Behçet's disease, Takayasu's arteritis, leukocytoclastic vasculitis, temporal arteritis and systemic vasculitis. Autoimmune diseases Systemic lupus erythematosus, systemic scleroderma, mixed connective tissue disease, autoimmune thyroiditis, Sjögren's syndrome, dermatomyositis, antiphospholipid syndrome, autoimmune hemolytic anemia, Hashimoto's thyroiditis. Other Rheumatologic diseases Rheumatoid arthritis, spondyloarthropathy, reactive arthritis, polymyalgia rheumatica. Hematologic disorders Myelodysplasia, myeloproliferative neoplasm. Dermatologic diseases Psoriasis, atopic dermatitis, lichen ruber planus, vitiligo. Autoinflammatory diseases Familial Mediterranean fever. Others Primary biliary cirrhosis, ulcerative colitis, Crohn's disease, diabetes mellitus, pulmonary fibrosis. Cartilage inflammation Cartilage inflammation (technically known as chondritis) that is<unk>  is very characteristic of the disease and is required for the diagnosis of<unk> . These recurrent episodes of inflammation over the course of the disease may result in breakdown and loss of cartilage. The signs and symptoms of cartilage inflammation in various parts of the body will be described first. Ear A cauliflower ear deformity Inflammation of the cartilage of the ear is a specific symptom of the disease and affects most people. It is present in about 20% of persons with<unk>  at presentation and in 90% at some point. Both ears are often affected but the inflammation may alternate between either ear during a relapse. It is characteristic for the entire outer part of the ear except the earlobe to be swollen, red, or less often purplish, warm and painful to light touch. The inflammation of the ear usually lasts a few days or more, rarely a few weeks, and then resolves spontaneously and recurs at various intervals. Because of the loss of cartilage, after several flares cauliflower ear deformity may result. The outer part of the ear may be either floppy or hardened by calcifications of the scar tissue that replaces the cartilage. These cauliflower ear deformities occur in about 10% of persons with<unk> . Nose The inflammation of the cartilage of the nose involves the bridge of the nose and is often less marked than the ears. Statistics show that this clinical manifestation is present in 15% of persons with<unk>  and occurs at some point in 65% of persons with<unk> . Nasal obstruction is not a common feature. Atrophy may eventually develop secondarily during the disease, this appears gradual and is not easily noticed. This can result in collapse of the nasal septum with saddle-nose deformity, which is painless but irreversible. Respiratory tract Inflammation occurs in the laryngeal, tracheal and bronchial cartilages. Both of these sites are involved in 10% of persons with<unk>  at presentation and 50% over the course of this autoimmune disease, and is more common among females. The involvement of the laryngotracheobronchial cartilages may be severe and life-threatening; it causes one-third of all deaths among persons with<unk> . Laryngeal chondritis is manifested as pain above the thyroid gland and, more importantly, as dysphonia with a hoarse voice or transient aphonia. Because this disease is<unk> , recurrent laryngeal inflammation may result in laryngomalacia or permanent laryngeal stenosis with inspiratory dyspnea that may require emergency tracheotomy as a temporary or permanent measure. Tracheobronchial involvement may or may not be accompanied with laryngeal chondritis and is potentially the most severe manifestation of<unk> . The symptoms consist of dyspnea, wheezing, a nonproductive cough, and recurrent, sometimes severe, lower respiratory tract infections. Obstructive respiratory failure may develop as the result of either permanent tracheal or bronchial narrowing or chondromalacia with expiratory collapse of the tracheobronchial tree. Endoscopy, intubation, or tracheotomy has been shown to hasten death. Ribs Involvement of the rib cartilages results in costochondritis. Symptoms include chest wall pain or, less often, swelling of the involved cartilage. The involvement of the ribs is seen in 35% of persons with<unk>  but is rarely the first symptom. Other manifestations<unk>  may affect many different organ systems of the body. At first, some people with the disease may have only nonspecific symptoms such as fever, weight loss, and malaise. Joint The second most common clinical finding of this disease is joint pain with or without arthritis, after chondritis. All synovial joints may be affected. At presentation, around 33% of people have joint symptoms that involve Polyarthralgia and/or polyarthritis or oligoarthritis that affects various parts of the body and often appears to be episodic, asymmetric, migratory and non-deforming. The most common sites of involvement are the metacarpophalangeal joints, proximal interphalangeal joints and knees. After which is followed by the ankles, wrists, metatarsophalangeal joints and the elbows. Any involvement of the axial skeleton is considered to be very rare. Tests for rheumatoid factor are negative in affected persons with<unk> , unless there is a co-morbidity with RA. Less often it has been reported that persons may experience arthralgia, monoarthritis, or chronic polyarthritis that mimics rheumatoid arthritis, leading to a difficult diagnosis for this disease. The appearance of erosions and destruction, however, is exceedingly rare and this may point instead to rheumatoid arthritis as a cause. Diseases and inflammation of tendons have been reported in small numbers of people with<unk> . During the course of the disease, around 80% of people develop joint symptoms. Eye Involvement of the eye is rarely the initial symptom but develops in 60% of persons with<unk> . The most common forms of ocular involvement are usually mild and often consist of unilateral or bilateral episcleritis and/or scleritis, that is often anterior and could be lingering or<unk> . Scleritis that is necrotizing is found to be exceedingly rare. Less often, conjunctivitis occurs. There are also other ocular manifestations that occur in persons with<unk> , these include keratoconjunctivitis sicca, peripheral keratitis (rarely with ulcerations), anterior uveitis, retinal vasculitis, proptosis, lid edema, keratoconus, retinopathy, iridocyclitis and ischemic optic neuritis that can lead to blindness. Cataract also is reported in relation to either the disease or to glucocorticoid exposure. Neurological The involvement of the peripheral or central nervous system is relatively rare and only occurs in 3% of persons affected with<unk> , and is sometimes seen in a relation with concomitant vasculitis. The most common neurological manifestation are palsies of the cranial nerves V and VII. Also hemiplegia, ataxia, myelitis and polyneuropathy have been reported in scientific literature. Very rare neurological manifestations include aseptic meningitis, meningoencephalitis, stroke, focal or generalized seizures and intracranial aneurysm. Magnetic Resonance Imaging of the brain shows multifocal areas of enhancement consistent with cerebral vasculitis in some cases. Kidney The involvement of the kidney can be caused by primary renal parenchymal lesions, or an underlying vasculitis, or another associated autoimmune disease. Actual kidney involvement is quite rare, elevated creatinine levels are reported in approximately 10% of people with<unk> , and abnormalities in urinalysis in 26%. Involvement of the kidney often indicates a worse prognosis, with a 10-year survival rate of 30%. The most common histopathologic finding is mild mesangial proliferation, that is followed by focal and segmental necrotizing glomerulonephritis with crescents. Other abnormalities that are found include glomerulosclerosis, IgA nephropathy and interstitial nephritis. Immunofluorescence studies most often reveal faint deposits of C3, IgG or IgM in the primarily mesangium. Constitutional symptoms These symptoms could consist of asthenia, fever, anorexia, and weight loss. They mostly occur during a severe disease flare. Others * Skin and mucous membranes: 20 to 30% of people with<unk>  have skin involvement, including aphthous ulcers, genital ulcers, and a number of non-specific skin rashes including erythema nodosum, livedo reticularis, hives, and erythema multiforme. * Cardiovascular system:<unk>  may cause inflammation of the aorta. It can also cause leaky heart valves (aortic valve regurgitation in 4 to 10%, mitral valve regurgitation in 2%).
Relapsing polychondritis
Rheumatoid nodulosis
Reactive arthritis
Juvenile idiopathic arthritis
Psoriatic arthritis
Discoid lupus erythematosus
Polymyalgia rheumatica
Rheumatoid arthritis