polyarteritis nodosa scleritis
Introduction
Introduction to nodular polyarteritis scleritis Nodular polyarteritis (PAN) was first reported by Kussmaul and Maier in 1866 as a necrotizing vasculitis characterized by small blood vessels in multiple systems. The lesion is segmental and occurs at the bifurcation of the vessel. It can invade adjacent veins and may involve the arteries or venules at the distal end of the vessel. basic knowledge Sickness ratio: 0.0001% Susceptible people: no special people Mode of infection: non-infectious Complications: hypertension, heart failure, pericarditis, myocarditis, endocarditis, pleurisy
Cause
Causes of nodular polyarteritis scleritis
(1) Causes of the disease
In 1970, Gocke et al reported that PAN is positively related to HBsAg, and immune complexes containing HBsAg are visible on the vessel wall. This provides strong support for the hypothesis that PAN is usually secondary to the precipitation of soluble immune complexes, but there are many domestic HBsAg carriers. The disease is rare, PAN can be spontaneous or secondary to the application of certain drugs, most of the reasons are still unknown.
(two) pathogenesis
The specific pathogenesis is unclear, and it is speculated that it is related to immune system dysfunction.
Prevention
Nodular polyarteritis scleritis prevention
1, should promote the wash, avoid blinking. It is recommended to wash your face, towels, handkerchiefs and other items to be separated from others, and often cleaned and disinfected.
2, for patients with infectious scleritis should take some isolation measures, but also not allowed to go to the public swimming because of swimming, medical staff must also wash hands and disinfect after contact with patients to prevent cross-infection.
3. If you have scleritis at a glance, you must tell the patient to protect your eyes from infection.
4, where the working environment is windy, dusty smoke and other irritants, should improve the environment and wear protective glasses to prevent conjunctivitis.
5. Health promotion, public inspection, and regular management of public places such as bathrooms, restaurants, and swimming pools.
Complication
Nodular polyarteritis scleritis complications Complications hypertension heart failure pericarditis myocarditis endocarditis pleurisy
Other clinical manifestations include cardiac involvement including heart enlargement and heart failure due to hypertension or coronary arteritis (myocardial ischemia, infarction), coronary artery damage is more common, second only to renal vascular damage, rare non- Exudative pericarditis, myocarditis and endocarditis can also occur in pulmonary involvement such as pleurisy or transient or progressive pulmonary infiltration, but mainly in patients with granulomatous vasculitis.
Symptom
Nodular polyarteritis scleritis symptoms Common symptoms Nausea severe pain Lack of hemianopia nodules Inability tinnitus edema Appetite decline convulsions
Eye performance
Depending on the extent of vascular damage, almost every part of the eye can be involved. Choroidal vasculitis is the most common histological abnormality, and clinically, subretinal yellow plaques due to choroidal vascular inflammation have not caused enough for ophthalmologists. Remarks, retinal vasculitis can cause retinal hemorrhage, edema, cotton-like exudation, abnormal retinal vessel diameter and vascular occlusion, retinopathy can also be secondary to hypertension, optic vasculitis can lead to optic disc edema or optic discitis, orbital vasculitis Can cause eyeball protrusion, central and peripheral nervous system vasculitis leads to III, V, VI and VII to cranial nerve palsy, homonymous hemianopia, nystagmus, transient erythema and Horner syndrome, rare uveal vasculature Inflammation can cause anterior uveitis, anterior chamber protein exudation, conjunctival infarction can appear pale yellow uplift and brittle damage, conjunctival edema and subconjunctival hemorrhage, superficial sclera, sclera and scleral vasculitis can produce scleral inflammation , scleritis and keratitis, ocular symptoms can be the first performance of PAN.
(1) Scleritis: It is reported that the incidence of PAN in patients with scleritis is 0.68% to 6.45%. The most common type of PAN scleritis is necrotizing anterior scleritis, often accompanied by marginal ulcerative keratitis, unless the diagnosis is correct. And systemic control medication, otherwise, scleritis will become severely painful and highly destructive, corneal ulcers gradually progress to the surrounding or central, forming a corneal edge of the cornea to the centrally eroded ulcer surface, whether clinically accompanied Scleritis is used to identify corneal ulcers in Mooren ulcers and vasculitis-related diseases (such as RA, WG, PAN). In most cases, corneal scleritis occurs after PAN diagnosis, and occasionally, it can be used as a clinical manifestation of PAN.
Among the 172 cases of scleritis observed by Foster et al, 2 cases of PAN (1.16%), 1 case of male and female, mean age of 58 years, 1 case of diffuse anterior scleritis, and 1 case of necrotizing anterior scleritis with marginal ulcer In keratitis, the first case developed diffuse anterior scleritis after 1 year of diagnosis of PAN; the other case first developed necrotizing anterior scleritis with marginal ulcerative keratitis, which was diagnosed as PAN after further examination, and the latter case There are general systemic symptoms of PAN such as dizziness, deep quadriceps weakness, tinnitus, skin damage and epileptic seizures. Biopsy found pathological findings of elbow nodules consistent with PAN, and found a capsular aneurysm of superior mesenteric artery. Combining these clinical manifestations and findings to confirm PAN, timely and correct treatment improves eye and systemic symptoms and prognosis. Therefore, ophthalmologists can play an important role in the diagnosis and mastering of these fatal vasculitis diseases. The role.
(2) Scleral outer inflammation: Although histological findings have superficial scleral vasculitis, clinical scleral inflammation is very rare, can be simple or nodular scleral inflammation, PAN patients with scleral inflammation than scleritis Rarely, 94 cases of scleral inflammation in Foster et al., no PAN patients.
2. Non-eye performance
Clinical manifestations can be mild, but also mild, fulminant, fever, fatigue, weight loss and loss of appetite and other systemic symptoms can occur simultaneously with skin, joint or nervous system symptoms, visceral lesions such as gastrointestinal or kidney damage Accompanying or following these characteristic manifestations, skin lesions include the most characteristic tender purple nodules (Osler nodules), purpura, ulcers, reticular bluish and gangrene, joint involvement is asymmetry, migratory Polyarthritis that does not cause deformity, occurs mostly in the lower extremity joints, abnormalities of the nervous system include peripheral nerves (sensory or sensory-motor neuropathy) and rare, about 30% of patients with central nervous system manifestations in the late stages of the disease ( Convulsions, hemiplegia, encephalopathy and psychiatric symptoms), due to the involvement of nutrient nerve vessels, can cause mononeuritis or multiple mononeuritis, asymmetry, pain or pain loss along the nerve, this lesion is more complicated than RA Sensory motor neuropathy has a poor prognosis. 30% to 50% of arterial lesions are common in one or more abdominal organs. Patients have gastrointestinal symptoms, which are abdominal pain and nausea. Vomiting, liver enlargement, intestinal obstruction and bleeding of internal organs, infarction and perforation, appendix, gallbladder, pancreas and spleen can also be affected, 80% of patients have renal lesions, manifested as focal or diffuse glomerulonephritis, Renal ischemia, renal involvement includes intermittent proteinuria, microscopic or gross hematuria, cell casts and progressive renal failure, renal polyarteritis can cause hypertension, uremia, congestive heart failure and death, ovary Testis and epididymis are commonly affected, but the symptoms are not obvious. If bleeding, there is pain during infarction, hemorrhagic cystitis can cause obvious hematuria and dysuria.
Examine
Examination of nodular polyarteritis scleritis
To date, no specific laboratory tests have confirmed PAN, more than 75% of PAN leukocytes, eosinophils increased, ESR increased, if hematuria, proteinuria suggests kidney involvement, HBsAg-positive patients may have abnormal liver function.
Angiography has shown that small hemangiomas in the liver, kidney and gastrointestinal tract contribute to the diagnosis of PAN. Small hemangiomas are not limited to this disease, but can also occur in SLE and fibromuscular dysplasia.
Diagnosis
Diagnosis and diagnosis of nodular polyarteritis scleritis
Tissue biopsy is the most powerful diagnostic tool for PAN. It is found to have necrotizing vasculitis of small and medium arteries and can be diagnosed when it is consistent with the clinical manifestations of multiple systems. In symptomatic areas such as skin, testis, epididymis, skeletal muscle and peripheral nerves. Biopsy is the most valuable, but biopsy of non-lesional sites is generally difficult to diagnose.
