RP quite often involves joints usually independently of other manifestations. The clinical features show episodic asymmetrical inflammatory involvement of large and small joints, including parasternal articulations and sacroiliac joints, that lasts for several days or weeks. In general, it manifests itself as a nondeforming, nonerosive, and seronegative (negative RF) polyarthritis. Radiographic examination of joints as a rule reveals joint space narrowing without erosion, which is given by the fact that pathological process causes only loss of the hyaline cartilage. A typical histopathological finding shows a lighter space with multinucleated bodies around chondrocytes and unspecific proliferation of epithelial cells in the middle and deep layers of the synovial membrane. The clinical course as well as radiographic and histopathological findings serves as the basis for distinguishing pure RP polyarthritis from polyarthritis of the rheumatoid type.

In a half of the patients, RP affects the respiratory system which may result in a breakdown of the architecture of bronchi and trachea and air collapse during the respiratory cycle. The first involved are usually the larynx and the upper part of trachea, with subglottic inflammation. The process may progress and involve also the lower part of the trachea and main bronchi. Dominant clinical symptoms include dysphonia, cough, stridor, and dyspnea. During the active phase of the disease, there may occur increased tenderness over the thyroid cartilage and the trachea. Neilly et al. [5] have found out that young patients with upper airway compromise, developed as early as at the onset of the disease, are often resistant to the treatment and have a poor prognosis. Involvement of the respiratory system may be the only dominant symptom of the disease and may be mistaken for a banal chronic bronchitis. In such case, it is beneficial to establish the diagnosis on the basis of flow-volume curve that will reveal an abrupt onset of acute obstruction of the upper airways.

On the contrary, the bronchitis loop shows a chronic obstructive disorder with a peak in the periphery (Fig. 10.2).

Involvement of cardiovascular system occurs in about 10 % of patients, particularly in the form of thoracic and abdominal aortic aneurysms. Another disorder may be aortitis causing thinning of the media and aortic root dilatation with ruptures of the aortic valve. Systemic polyarteritis nodosa was identified in 9 % of cases. The range of effects of inflammation on the blood vessels is quite broad. If it affects small blood vessels, it has a form of cutaneous leukocytoclastic vasculitis, while in large blood vessels that of the Takayasu’s arteritis. Inflammation may affect also the aortic and mitral valves and cause their functional insufficiency due to aortic root dilatation, valvulitis, or papillary muscle dysfunction. In addition to these symptoms and findings, there may occur several abnormalities, such as arrhythmia, heart block, and supraventricular tachycardia caused by myocarditis of the conductive system.

Renal involvement was detected in 20 % of patients with RP. It manifests itself predominantly in the form of segmental proliferative necrotizing glomerulonephritis. Immunofluorescence and electron microscopy examination showed a small amount of sediments of IgG and IgM immunoglobulins and of C3 component of the granular complement deposited in the subendothelial and mesangial areas. The course of the disease in patients with renal involvement is usually quite severe and may be associated with manifestations of extrarenal vasculitis and unfavorable prognosis.

A severe disorder in RP is inflammation of the eyeball, most often episcleritis, scleritis, and corneal thinning, that may cause perforations associated with other complications, leading ultimately to the loss of vision. Other ocular manifestations in RP include uveitis, retinal vasculitis, and optic neuritis that may also ultimately result in loss of vision. In addition, this disease may be associated with paralysis of eye muscles, inflammation of the orbit, and papilla swelling.

Skin symptoms in RP include purpura, rash, and angioedema, less frequently livedo reticularis, migratory superficial thrombophlebitis, erythema nodosum, erythema multiforme, and panniculitis.

Neurological complications in RP include cranial neuropathy, headache, encephalopathy, hemiplegia, and ataxia, sometimes also transverse myelitis, mononeuritis multiplex, and temporal non-granulomatous vasculitis. In up to 22 % of patients, RP is accompanied by quite a high fever.

Bellamy and Dewar [6] described a case of a 25-year-old woman who presented with chondritis at 14 weeks of pregnancy. The patient was receiving high doses of glucocorticoids. Pregnancy and delivery of the child were physiological. The child developed normally after the birth, and the disease did not reactivate in the postpartum period. Later the patient gave birth to another healthy child, again without reactivation of the disease.

Sallam et al. [7] described an unusual manifestation of relapsing polychondritis presenting initially with isolated ocular signs, mimicking infective keratitis. It was a case report of a 75-year-old man who presented with marked left ocular irritation and photophobia. Ophthalmological examination disclosed corneal intrastromal infiltrate and hypopyon which failed to respond to intensive antimicrobial drops. Later he developed bilateral auricular chondritis. Relapsing polychondritis was diagnosed. Treatment with topical and oral corticosteroids resulted in marked improvement of the corneal infiltrate and resolution of the auricular inflammation. The authors highlighted the importance of considering connective tissue inflammatory conditions in any stromal keratitis unresponsive to antimicrobial treatment, in the context of a developing relapsing polychondritis or another connective tissue inflammatory condition. In 2010, Starr et al. [8] presented a case report of a 70-year-old man with an unusual clinical manifestation of the relapsing polychondritis in the form of alopecia areata.

Erten-Lyons et al. [9] detected in a 50-year-old lawyer with the diagnosis of relapsing polychondritis the presence of subacute dementia associated with the disease. Another patient, a 68-year-old man, with the diagnosis of relapsing polychondritis presented with myalgia, headache, fever, and bilateral swelling of auricles. For 8 months, the patient’s condition was gradually aggravating, with typical weight loss, conjunctiva involvement, and loss of cognitive functions. The patient was unable to perform activities of daily living without assistance and had speech difficulties lasting for several hours. Psychological examination revealed impaired verbal and visual memory indicating an early stage of dementia.

Relapsing polychondritis may occur simultaneously with ulcerative colitis, Behçet’s syndrome, Wegener’s granulomatosis, Sweet’s syndrome, systemic lupus erythematosus, and other inflammatory diseases of the connective tissue (rheumatoid arthritis, Sjögren’s syndrome, systemic scleroderma, psoriatic arthritis, polyarteritis nodosa), but also in association with tumorous diseases, such as chronic lymphocytic leukemia [10].

The “MAGIC syndrome” (mouth and genital ulcers with inflamed cartilage syndrome) is a combination of the Behçet’s disease and relapsing polychondritis [11].

Duda and Botka [12] described a rare association of RP in a 64-year-old female patient with Sjögren’s syndrome, tubulointerstitial nephritis, and autoimmune thyropathy. During the first year, tubulointerstitial nephritis became aggravated in the form of renal failure with hypokalemia and hyperuricemia. Later it was followed by a stroke and development of pancytopenia even after elimination of cyclophosphamide from the treatment. After 1 year and 7 months, the patient died of catheter-related sepsis and bronchopneumonia.

The presented case reports indicate that a severe relapsing polychondritis may occur even in an older age category.

The common feature of laboratory parameters in RP is increase in acute inflammatory phase reactants and the presence of anemia and thrombocytosis, sometimes also a mild leukocytosis. Serological tests have shown that the serum in 50 % of patients was positive for antibodies against type II collagen. In most cases, the finding includes circulating immune complexes and antibodies against intracellular antigens (approximately in 20 % of cases). A finding of circulating anticoagulants was also described, which explains the clinical finding of severe thromboses.