The initial symptoms of RP may begin suddenly. The most common first symptom is pain and swelling of the ear. Both ears may turn red or purple and are tender to the touch. Swelling may extend into the ear canal causing loss of hearing, ear infections, balance disturbances with vertigo and vomiting, and eventually a droopy ear. Throat pain may occur leading to hoarseness and difficulty talking. The nose may be affected and deterioration may lead to a flattened nose bridge (sometimes known as a 'saddle nose'). Inflammation of the eye occurs causing impaired vision. Fatigue and weight loss are common symptoms in RP and fever frequently accompanies acute flares.
During the later stages, the symptoms of RP may become more debilitating and life-threatening. RP may cause deterioration of the cartilage holding the windpipe open and this may lead to difficulties breathing. Deterioration of the rib cartilage can lead to the collapse of the chest, again hindering breathing. Joints everywhere are involved in episodes of arthritis (see entry, Arthritis (Juvenile Idiopathic)), with pain and swelling. As the disease progresses over a period of years, the mortality rate increases. Kidney failure (see entry, Kidney disease) may lead to death.
The disease may occur episodically with complete remission between episodes, or it may continue over time, causing progressive destruction (atrophy) of organs. Individuals may have persistent symptoms between acute flares or the pattern of disease may be more limited. The severity and the frequency of symptoms associated with RP varies between people and with time. Furthermore, the areas affected by RP may either remain constant or be completely unpredictable for each person.