Temporal arteritis, the second type of giant cell arteritis, is also a chronic, inflammatory disease involving mid- to large-sized arteries. Temporal arteritis has a higher incidence in people of Scandinavian descent. However, the incidence rate differs based on population, region and races. Temporal arteritis is not uncommon in North America. The incidence rate is around 0.017% for individuals over 50 years of age.

Symptoms of temporal arteritis are classified as specific and nonspecific.

Nonspecific symptoms:

- Headache

- Low grade fever

- Sweating

- Anorexia (loss of appetite)

- Weight loss

- General malaise

Specific symptoms:

- Claudication of the jaw

- Engorged, tender vessels

Specific symptoms usually develop in the advanced stages of temporal arteritis.

Polyarteritis nodosa of unknown mechanism can cause testiscular pain. It is often associated with aneurysms and Hepatitis B.

Possible symptoms include:

- General symptoms: Fever, weight loss

- Skin: Palpable purpura, livedo reticularis

- Muscles and joints: Myalgia or myositis, arthralgia or arthritis

- Nervous system: Mononeuritis multiplex, headache, stroke, tinnitus, reduced visual acuity, acute visual loss

- Heart and arteries: Myocardial infarction, hypertension, gangrene

- Respiratory tract: Nose bleeds, bloody cough, lung infiltrates

- GI tract: Abdominal pain, bloody stool, perforations

- Kidneys: Glomerulonephritis

These conditions are sometimes considered together with the small vessel vasculitides.

Polyarteritis nodosa (PAN). Systemic necrotizing vasculitis and aneurysm formation affecting both medium and small arteries. If only small vessels are affected, it is called microscopic polyangiitis, although it is more associated with granulomatosis with polyangiitis than to classic PAN. At least 3 out of 10 criteria yields sensitivity and specificity of 82 and 87%:

- unexplained weight loss > 4 kg

- livedo reticularis

- testicular pain

- myalgias, weakness

- Abdominal pain, diarrhea, and GI bleeding

- mononeuropathy or polyneuropathy

- new onset diastolic blood pressure > 90 mmHg

- elevated serum BUN (> 40 mg/dL) or serum creatinine (> 1.5 mg/dL)

- hepatitis B infection

- arteriographic abnormalities

- arterial biopsy showing polymorphonuclear cells

Kawasaki disease. Usually in children(age<4), it affects large, medium, and small vessels, prominently the coronary arteries. Associated with a mucocutaneous lymph node syndrome. Diagnosis requires fever lasting five days or more with at least 4 out of 5 criteria:

- bilateral conjunctival injection

- injected or fissured lips, injected pharynx, or strawberry tongue

- erythema of palms/soles, edema of hands/feet, periungual desquamation

- polymorphous rash

- cervical lymphadenopathy (at least one node > 1.5 cm)

Isolated cerebral vasculitis. Affects medium and small arteries over a diffuse CNS area, without symptomatic extracranial vessel involvement. Patients have CNS symptoms as well as cerebral vasculitis by angiography and leptomeningeal biopsy.

Arteritis may be primary or secondary to some other disease process. The primary types are:

An example of a secondary arteritis is arteritis caused by infection with the fungal pathogen "Candida albicans".

Giant-cell arteritis and Takayasu's arteritis have much in common, but usually affect patients of different ages, with Takayasu's arteritis affecting younger people, and giant-cell arteritis having a later age of onset.

Aortitis can also be considered a large-vessel disease.

Takayasu arteritis. Primarily affects the aorta and its main branches. At least 3 out of 6 criteria yields sensitivity and specificity of 90.5 and 97.8%:

- onset < 40 years affects young and middle -aged women (ages 15–45)

- claudication of extremities

- decreased pulsation of one or both brachial arteries

- at least 10 mmHg systolic difference in both arms

- bruit over one or both carotid arteries or abdominal aorta

- arteriographic narrowing of aorta, its primary branches, or large arteries in upper or lower extremities

- Ocular manifestation

- visual loss or field defects

- Retinal hemorrhages

- Neurological abnormalitis

- Treatment: steroids

Giant cell (temporal) arteritis. Chronic vasculitis of both large and medium vessels, primarily affecting cranial branches of the arteries arising from the aortic arch. At least 3 out of 5 criteria yields sensitivity and specificity of 95 and 91%:

- Age at onset ≥ 50 years

- New onset headache with localized tenderness

- Temporal artery tenderness or decreased pulsation

- Elevated ESR ≥ 50 mm/hour Westergren

- Temporal artery biopsy showing vasculitis with mononuclear cell infiltrate or granulomatous inflammation, usually with multinucleated giant cells

Vasculitis is a group of disorders that destroy blood vessels by inflammation. Both arteries and veins are affected. Lymphangitis is sometimes considered a type of vasculitis. Vasculitis is primarily caused by leukocyte migration and resultant damage.

Although both occur in vasculitis, inflammation of veins (phlebitis) or arteries (arteritis) are their own are separate entities.

The major symptom and signs include an acute onset of right upper quadrant (RUQ) abdominal pain aggravated by breathing, coughing or laughing, which may be referred to the right shoulder. There is usually also tenderness on palpation of the right upper abdomen and tenderness to percussion of the lower ribs which protect the liver. Surprisingly there is often no or only minimal pelvic pain, vaginal discharge or cervical motion tenderness, which may lead to the diagnosis being missed. This may be due to infectious bacteria bypassing pelvic structures on the way to the liver capsule.

Pain or tenderness to palpation usually occurs on the sides of the sternum, affects multiple ribs, and is often worsened with coughing, deep breathing, or physical activity. On physical examination, a physician inspects and feels the patient for swollen or tender areas, and can often produce the pain of costochondritis by moving the patient's rib cage or arms.

A factor that may aid in the differentiation of costochondritis from Tietze syndrome is the location of the pain on the sternum. Costochondritis typically affects the third, fourth, and fifth costosternal joints in contrast to Tietze's syndrome, which usually affects the second or third costosternal joint. Pain from costochondritis typically resolves within one year.

Fitz-Hugh–Curtis syndrome is a rare complication of pelvic inflammatory disease (PID) involving liver capsule inflammation leading to the creation of adhesions. The condition is named after the two physicians, Thomas Fitz-Hugh, Jr and Arthur Hale Curtis who first reported this condition in 1934 and 1930 respectively.

Lung involvement is typically in the form of hemoptysis, pleuritis, cough, or fever, and in severe cases can be life-threatening if the outlet pulmonary artery develops an aneurysm which ruptures causing severe vascular collapse and death from bleeding in the lungs. Nodules, consolidations, cavities and ground glass lesions are common in patients with pulmonary involvement. Pulmonary artery thrombosis may occur.

Patients with trochleitis typically experience a dull fluctuating aching over the trochlear region developing over a few days. Some may also feel occasional sharp pains punctuating the ache. In patients with migraines, trochleitis may occur simultaneously with headache. Presentation is usually unilateral with palpable swelling over the affected area supranasal to the eye. The trochlear region is extremely tender to touch. Pain is exacerbated by eye movements looking down and inwards, and especially in supraduction (looking up) and looking outwards, which stretches the superior oblique muscle tendon. Notably, there is no restriction of extraocular movements, no diplopia, and often no apparent ocular signs such as proptosis. However, occasionally mild ptosis is found. The absence of generalized signs of orbital involvement is helpful in eliminating other more common causes of periorbital pain.

CNS involvement most often occurs as a chronic meningoencephalitis. Lesions tend to occur in the brainstem, the basal ganglia and deep hemispheric white matter and may resemble those of MS. Brainstem atrophy is seen in chronic cases.

Neurological involvements range from aseptic meningitis to vascular thrombosis such as dural sinus thrombosis and organic brain syndrome manifesting with confusion, seizures, and memory loss. Sudden hearing loss (Sensorineural) is often associated with it. They often appear late in the progression of the disease but are associated with a poor prognosis.

DIRA displays a constellation of serious symptoms which include respiratory distress, as well as the following:

Affected individuals typically present with sudden painful proptosis, redness, and edema. Proptosis will vary according to the degree of inflammation, fibrosis, and mass effect. Occasionally, ptosis, chemosis, motility dysfunction (ophthalmoplegia), and optic neuropathy are seen. In the setting of extensive sclerosis there may be restriction, compression, and destruction of orbital tissue. Symptoms usually develop acutely (hours to days), but have also been seen to develop over several weeks or even months.Malaise, headaches, and nausea may accompany these symptoms. Other unusual presentations described include cystoid macular edema, temporal arteritis, and cluster headaches.

Pediatric IOI accounts for about 17% of cases idiopathic orbital inflammation. The most common sign is proptosis, but redness and pain are also experienced. Presentation varies slightly compared to adults with bilateral involvement, uveitis, disc edema and tissue eosinophilia being more common in this population. The presence of uveitis generally implies a poor outcome for pediatric IOI. Bilateral presentation may have a higher incidence of systemic disease.

Costochondritis, also known as chest wall pain, costosternal syndrome, or costosternal chondrodynia is an acute and often temporary inflammation of the costal cartilage, the structure that connects each rib to the sternum at the costosternal joint. The condition is a common cause of chest pain. Though costochondritis often resolves on its own, it can be a recurring condition that has little or no signs of onset.

Costochondritis symptoms can be similar to the chest pain associated with a heart attack. Chest pain is considered a medical emergency until life-threatening cardiac issues can be ruled out. Severe cases of costal cartilage inflammation that also involve painful swelling are sometimes referred to as Tietze's syndrome, a term sometimes used interchangeably with costochondritis. However, some physicians view costochondritis and Tietze's syndrome as separate disease states due to the absence of costal cartilage swelling in costochondritis.

Treatment options are quite limited and usually involve a combination of rest, analgesics, or anti-inflammatory medications. Cases with persistent discomfort may be managed with cortisone injections or surgery may be indicated if the condition is severe. Individuals with costochondritis are typically instructed to avoid strenuous physical activity to prevent the onset of an attack.

Trochleitis is inflammation of the superior oblique tendon trochlea apparatus characterized by localized swelling, tenderness, and severe pain. This condition is an uncommon but treatable cause of periorbital pain. The trochlea is a ring-like apparatus of cartilage through which passes the tendon of the superior oblique muscle. It is located in the superior nasal orbit and functions as a pulley for the superior oblique muscle. Inflammation of the trochlear region leads to a painful syndrome with swelling and exquisite point tenderness in the upper medial rim of the orbit. A vicious cycle may ensue such that inflammation causes swelling and fraying of the tendon which then increases the friction of passing through the trochlea which in turn adds to the inflammation. Trochleitis has also been associated with triggering or worsening of migraine attacks in patients with pre-existing migraines (Yanguela, 2002).

Pathophysiology of this disease consists of relaxation of the transverse ligament of the atlanto-axial joint.

Idiopathic orbital inflammatory (IOI) disease, or orbital pseudotumor, refers to a marginated mass-like enhancing soft tissue involving any area of the orbit. It is the most common painful orbital mass in the adult population, and is associated with proptosis, cranial nerve (Tolosa–Hunt syndrome), uveitis, and retinal detachment. Idiopathic orbital inflammatory syndrome, also known as orbital pseudotumor, was first described by Gleason in 1903 and by Busse and Hochhmein. It was then characterized as a distinct entity in 1905 by Birch-Hirschfeld. It is a benign, nongranulomatous orbital inflammatory process characterized by extraocular orbital and adnexal inflammation with no known local or systemic cause. Its diagnosis is of exclusion once neoplasm, primary infection and systemic disorders have been ruled-out. Once diagnosed, it is characterized by its chronicity, anatomic location or histologic subtype.

Idiopathic orbital inflammation has a varied clinical presentation depending on the involved tissue. It can range from a diffuse inflammatory process to a more localized inflammation of muscle, lacrimal gland or orbital fat. Its former name, orbital pseudotumor, is derived due to resemblance to a neoplasm. However, histologically it is characterized by inflammation. Although a benign condition, it may present with an aggressive clinical course with severe vision loss and oculomotor dysfunction.

The age of onset is almost always before 3 months of age. Many infants are born preterm (1/3 cases) and dysmature. The babies are frequently small for dates. The placenta may be abnormal with non-specific inflammation on histology. Umbilical cord anomalies have occasionally been reported. In severe cases, signs in the brain may be detected on prenatal ultrasound.

The presentation is pleiomorphic, making the diagnosis difficult, but the most common features of this disease involve the skin, joints, and central nervous system.

All have a maculopapular urticarial skin rash that is often present at birth (75% cases). It is probably more correctly described as an urticarial-like rash. The presence of the rash varies with time, and biopsy of these skin lesions shows a perivascular inflammatory infiltrate including granulocytes.

In about 35-65% of cases, arthritis occurs. Joint signs are variably expressed and can lead to transient swelling without sequelae between crises, or to unpredictable anomalies of growth cartilage and long bones epiphyses suggestive of a pseudo-tumour. Biopsies reveal hypertrophic cartilage without inflammatory cells. This most commonly affects the large joints (knees, ankles, elbows, and wrists) but may also involve the small joints of the hands and feet. It is usually bilateral and painful. A common and characteristic feature is giant kneecaps. Severe cases may result in contractures (joint deformities).

Most patients eventually have neurological problems. These manifest themselves in three principal ways: chronic meningitis, involvement of both the optic tract and eye, and sensorineural hearing loss. The chronic meningitis presents with the features of chronically raised intracranial pressure: headaches, vomiting, ventriculomegaly, hydrocephalus, macromegaly, cerebral atrophy, and optic atrophy. Some of these features may be evidenced on prenatal ultrasound. In 50% of cases, intellectual deficit occurs. Seizures occur in 25% of cases, but other manifestations are rare. Histological examination shows infiltration of the meninges with polymorphs.

Ocular manifestations occur in 80% of cases and include uveitis (70%), papillary involvement, conjunctivitis, and optical neuritis. If untreated, these may result in blindness (25%). The sensorineural hearing loss occurs in 75%, and tends to be progressive leading to deafness in 20% of cases.

Almost all children are remarkably short and have growth delay. Fever is extremely common but inconstant and is most often mild. Anemia is frequent. Other findings that have been reported include macrocephaly (95%), large fontanelle, prominent forehead, flattening of the nasal bridge (saddleback nose), short and thick extremities, and finger clubbing. The liver and/or spleen may be enlarged. Lymph node enlargement may also be present.

Later in life, secondary amyloidosis may occur. Delayed puberty and secondary amenorrhoea are not uncommon. Hoarseness due to inflammation of the laryngeal cartilage has also been reported.

The second stage is characterized by an abnormally high level of eosinophils (a type of white blood cell) in the blood and tissues. The symptoms of hypereosinophilia depend on which part of the body is affected, but most often it affects the lungs and digestive tract. The signs and symptoms of hypereosinophilia may include weight loss, night sweats, asthma, cough, abdominal pain, and gastrointestinal bleeding. Fever and malaise are often present.

The eosinophilic stage can last months or years, and its symptoms can disappear, only to return later. Patients may experience the third stage simultaneously.

Deficiency of the interleukin-1–receptor antagonist (DIRA) is a autosomal recessive, genetic autoinflammatory syndrome resulting from mutations in "IL1RN", the gene encoding the interleukin 1 receptor antagonist. The mutations result in an abnormal protein that is not secreted, exposing the cells to unopposed interleukin 1 activity. This results in sterile multifocal osteomyelitis, periostitis (inflammation of the membrane surrounding the bones), and pustulosis due to skin inflammation from birth.

Syphilitic aortitis (SA) is inflammation of the aorta associated with the tertiary stage of syphilis infection. SA begins as inflammation of the outermost layer of the blood vessel, including the blood vessels that supply the aorta itself with blood, the vasa vasorum. As SA worsens, the vasa vasorum undergo hyperplastic thickening of their walls thereby restricting blood flow and causing ischemia of the outer two-thirds of the aortic wall. Starved for oxygen and nutrients, elastic fibers become patchy and smooth muscle cells die. If the disease progresses, syphilitic aortitis leads to an aortic aneurysm. Unlike atherosclerosis, which typically manifests in older people, syphilitic aortitis typically affects those under the age of 50. It has become rare in the developed world with the advent of penicillin treatments after World War II.

Grisel’s syndrome is a non-traumatic subluxation of the atlanto-axial joint caused by inflammation of the adjacent tissues. This is a rare disease that usually affects children. Progressive throat and neck pain and neck stiffness can be followed by neurologic symptoms such as pain or numbness radiating to arms (radiculopathies). In extreme cases, the condition can lead to quadriplegia and even death from acute respiratory failure. The condition often follows soft tissue inflammation in the neck such as in cases of upper respiratory tract infections, peritonsillar or retropharyngeal abscesses. Post-operative inflammation after certain procedures such as adenoidectomy can also lead to this condition in susceptible individuals such as those with Down's syndrome.

Inflammatory involvement of tertiary syphilis begins at the adventitia of the aortic arch which progressively causes obliterative endarteritis of the vasa vasorum. This leads to narrowing of the lumen of the vasa vasorum, causing ischemic injury of the medial aortic arch and then finally loss of elastic support and dilation of the vessel. Dissection of the aortic arch is rare due to medial scarring. As a result of this advanced disease process, standard methods of angiography/angioplasty may be impossible for those with suspected coronary heart disease. However, these patients may be candidates for diagnostic CT as a less invasive modality. This disorder is also known eponymously as Heller-Döhle syndrome.

The third and final stage, and hallmark of EGPA, is inflammation of the blood vessels, and the consequent reduction of blood flow to various organs and tissues. Local and systemic symptoms become more widespread and are compounded by new symptoms from the vasculitis.

Severe complications may arise. Blood clots may develop within the damaged arteries in severe cases, particularly in arteries of the abdominal region, which is followed by infarction and cell death, or slow atrophy. Many patients experience severe abdominal complaints; these are most often due to peritonitis and/or ulcerations and perforations of the gastrointestinal tract, but occasionally due to acalculous cholecystitis or granulomatous appendicitis.

The most serious complication of the vasculitic stage is heart disease, which is the cause of nearly one-half of all deaths in patients with EGPA. Among heart disease-related deaths, the most usual cause is inflammation of the heart muscle caused by the high level of eosinophils, although some are deaths due to inflammation of the arteries that supply blood to the heart or pericardial tamponade. Kidney complications have been reported as being less common.