Thrombangiitis obliterans or endangitis obliterans is a chronic inflammatory disease of the smaller and medium-sized blood vessels that, if left untreated, can lead to necrosis in the surrounding tissue structures of the affected extremity. In particular, men between the ages of 20 and 40 who are high users of nicotine (98 percent) are affected by thrombangiitis obliterans.
What is thrombangiitis obliterans?
Thrombangiitis obliterans (also endangiitis obliterans, Buerger syndrome, Winiwarter-Buerger disease) is an inflammatory occlusive disease of the arterial and venous blood vessels that has a chronic and relapsing course. As a rule, the smaller and middle distal blood vessels of the upper and lower extremities (foot, lower leg, hands, forearm) are segmentally affected by this form of vasculitis (inflammation of the blood vessels). Due to the inflammatory processes, leukocytes (white blood cells) accumulate on the vessel walls, damage them and later cause thrombosis of the affected vessel, which leads to vessel occlusion and finally to necrotization of the adjacent tissue as a result of reduced blood flow (ischemia). Thrombangiitis obliterans is typically manifested by ischemic claudication (limping), pain at rest, paresthesias (sensory disturbances such as tingling, numbness, temperature perception disturbances), edema, cyanosis (blue coloration of fingers and teeth), and ulcers or gangrene (ischemic form of necrosis). In addition, thrombophlebitis (acute superficial phlebitis) and Raynaud’s syndrome (vasospasm) are characteristic concomitants of thrombangiitis obliterans.
Causes
The exact etiology of thrombangiitis obliterans has not been fully elucidated to date. Presumably, the disease is autoimmune or allergic-hyperergic due to an underlying genetic predisposition and is triggered in combination with certain noxious agents (exogenous toxins). In this regard, the antigens HLA-A9 and HLA-B5 are conspicuous, which in some studies could be detected in an increased number of affected persons. In particular, heavy nicotine consumption (smoking) is considered a trigger factor for thrombangiitis obliterans, which may be due to nicotine intolerance. Strikingly, the incidence is high among young men who are highly dependent on nicotine. Hyperhomocysteinemia is also controversially discussed as a potential triggering factor for the disease, although an association has so far only been demonstrated in isolated cases. In addition, whether the organism of smokers synthesizes autoantibodies against endogenous collagen is still being investigated in the context of thrombangiitis obliterans.
Symptoms, complaints, and signs
The disease thrombangiitis obliterans exclusively affects smokers. It usually begins before the age of 40. Women and men can suffer equally from the relapsing symptoms. In a chronic form, symptoms stop on their own for long periods of time. However, this by no means alleviates the causes. They start anew after a vacancy. Typical complaints are pain in the limbs. The hands are often affected. But the feet and calves can also hurt. The pain is not the only symptom. Patients complain of a permanent feeling of cold. This is indicated by bluish fingers and toes. In some cases, superficial skin ulcers are present. The nails not infrequently have necrosis. The dying off is favored by an insufficient blood circulation. Sometimes patients also describe sensory disturbances. Thrombangiitis obliterans can have serious consequences. Entire limbs or parts thereof may die. Individual fingers and toes or the entire hand may be affected. Patients can then no longer exercise a profession at a young age. The main symptoms of the hands and feet are not conclusive. The vascular inflammation can also spread to other organs. Thus, in consequence, diseases of the heart, brain and gastrointestinal tract are possible.
Diagnosis and course
Thrombangiitis obliterans can often be diagnosed on the basis of characteristic clinical symptoms. Color-coded duplex sonography allows visualization of blood flow velocity and adjacent tissue structures.Magnetic resonance angiography can detect vascular anomalies such as stenoses or occlusions, while phlebography (X-ray of the veins under contrast medium) can show outflow obstructions (e.g., thromboses). A biopsy is performed only in unclear cases due to the increased risk of wound healing disorders when thrombangiitis obliterans is suspected. In any case, the disease should be differentiated from other vasculitides, arterial embolisms, peripheral arterial occlusive disease, and venous insufficiency. Thrombangiitis obliterans has a favorable prognosis in terms of life expectancy. With regard to preservation of the affected extremities, the prognosis is considerably less favorable, especially in the absence of nicotine abstinence. Approximately 40 percent of those affected by thrombangiitis obliterans require subsequent amputation.
Complications
Thrombangiitis obliterans can result in severe complications. Sensory disturbances and pain are typical of the vascular disease and become permanent if treatment is not given or is too late. The typical blue coloration of the skin, cyanosis, can develop into so-called polyglobulia in a chronic course. Subsequently, iron deficiency anemia occurs, resulting in chronic fatigue. Cyanotic patients also have an increased bleeding tendency and frequently develop brain abscesses. If necrosis occurs, this can lead to organ damage. In about 40 percent of cases, one or more extremities have to be amputated in the course of the chronic inflammatory disease. A possible secondary disease of thrombangiitis obliterans is Raynaud’s syndrome, which is associated with further pain as well as skin redness and scleroderma. Therapy for the disease may be associated with side effects and interactions. For example, the analgesics typically prescribed cause gastrointestinal problems and skin irritation. Antirheumatic drugs, antibiotics, and antiplatelet agents also carry corresponding risks. Severe complications are only likely in complex surgical procedures such as bypass surgery or amputation. Despite all measures, there is always a risk that thrombangiitis obliterans will recur elsewhere.
When should you see a doctor?
The affected person is dependent on medical treatment for thrombangiitis obliterans. Only through proper and especially early treatment can further complications or discomfort be prevented and limited, since self-healing cannot occur in this disease. For this reason, a doctor should be consulted at the first signs and symptoms of the disease. In the case of thrombangiitis obliterans, very severe pain in the limbs usually indicates the disease. Especially the hands are affected by this pain. However, there is also decreased blood flow, which can also cause necrosis. Most patients also suffer from sensory disturbances, so there may be limitations in everyday life. In some cases, thrombangiitis obliterans can also cause inflammation of internal organs. Thrombangiitis obliterans can be detected and treated by a general practitioner or by an internist.
Treatment and therapy
As part of the treatment of thrombangiitis obliterans, strict nicotine abstinence has absolute priority, as this alone can halt the progression of the disease. Although impairments already present are usually irreversible, additional amputations can be avoided in the further course of the disease in about 94 percent of cases by abstaining from nicotine. Pain can be reduced with the aid of analgesics or NSAIDs (non-steroidal anti-inflammatory drugs), and in the case of severe pain, temporary analgesia by means of peridural anesthesia may be considered. In addition, improvement of blood flow in the affected extremities by pressure relief (immobilization), platelet aggregation inhibitors (including acetylsalicylic acid), and intravenously infused prostaglandin derivatives (mainly iloprost, alprostadil) are aimed at reducing the amputation rate. The latter additionally reduce pain at rest and significantly accelerate the healing of necrotic tissue. Necrotic structures and fibrin coatings should be removed, and open wounds should be treated prophylactically against infection by regular irrigation.If signs of infection can be detected, antibiotic therapy may be indicated. The long-term effect of sympathectomy (surgical nerve block) has not yet been proven, although it may be considered due to its spasm-relieving effect. In very rare cases, bypass surgery may be performed in cases of marked ischemia. In addition, those affected by thrombangiitis obliterans are advised to avoid cold exposure of the fingers and toes and heat baths.
Prevention
Because the exact cause of thrombangiitis obliterans is not clear, it cannot be prevented. However, strict nicotine abstinence can prevent the manifestation of the disease or halt the progression of thrombangiitis obliterans.
Follow-up
Various follow-up measures are usually necessary for thrombangiitis obliterans. These vary depending on the course of the disease as well as the therapy chosen. Often, immediate cessation of nicotine use leads to cure. In heavy smokers, however, the cessation process should be monitored medically. Therefore, regular check-ups with the family doctor or a specialist are advisable. The intervals between follow-up examinations can vary greatly. Often, a strong craving for nicotine persists for months and years after quitting smoking. Those affected are advised to consult a general practitioner in such cases. Psychotherapeutic care is sometimes necessary. In advanced thrombangiitis obliterans, infusion therapy is often necessary. This usually requires an inpatient hospital stay, during which some follow-up examinations already take place. In the case of a complication-free course, at least one further follow-up examination is usually necessary a few weeks later. In most cases, ultrasound and X-ray images of the affected area are taken. In a few cases, thrombangiitis obliterans requires amputation. In this case, the aftercare is much more intensive and protracted than in the case of a more favorable course of the disease. The healing of the surgical wound is initially monitored closely. A longer stay in hospital is therefore necessary. This is followed by further measures to regain mobility.
What you can do yourself
There are a number of things that affected individuals can do themselves. However, a doctor should be consulted before using them, if possible. Home remedies that can lead to cooling and regression from the inflammation are compresses with alcohol as well as apple cider vinegar. For this purpose, apple cider vinegar or alcohol from the pharmacy should be diluted with water. Cloths are soaked with it and wrapped around affected areas. This has a particularly cooling effect. Clay packs are also an old household remedy. For this, clay should be mixed with cold water to form a paste-like mixture and then applied to affected areas as thick as a finger. This has an anti-inflammatory effect in particular, as well as a decongestant effect. The disease is associated with smaller blood clots. To dissolve these, accelerated blood circulation often helps. Movement and support stockings or a compression bandage are particularly effective in reducing blood clots. The pulse rate increases with exercise. The heart then pumps more blood and accelerates blood circulation. Support stockings and compression bandages compress affected veins somewhat and thus reduce the cross-section of the veins. The same amount of blood must then flow in the narrowed vein cross-section. This increases the flow velocity. In addition, compression significantly improves the function of the venous valves and thus the function of the venous pump. Nevertheless, a physician should always be consulted, since in the worst case the condition can lead to a life-threatening pulmonary embolism.
