Chronic obstructive rare arterial disease

Vasculitis

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Inflammatory diseases with complex etiopathogenesis that are not fully known, probably immune-mediated, can affect both arteries and veins and evolve with signs of parietal necrosis and steno-occlusive phenomena. They can be framed in relation to the type of inflammatory infiltrate, which however can change during the evolution of the disease, especially in the transition from acute to chronic. The final outcome of parietal damage leads to the formation of aneurysms, hemorrhages and occlusions, acute (thrombosis) or chronic. The most well-known obstructive forms are Buerger's disease, Kawasaki's disease, and Behcet's syndrome; non-obstructive ones are panarteritis nodosa, allergic angiitis, giant cell arteritis and Takayasu's disease.

Buerger's disease

Buerger's disease is characterized by segmental stenoocclusions on the medium and small-caliber arteries, especially the most distal parts of the lower limbs.

Etiopathogenesis

With unknown etiopathogenesis, it predominantly affects males (70-80%) smokers and young adulthood. Some authors have advanced the hypothesis of the existence of an autoimmune mechanism, with cell-mediated hypersensitivity to human type I collagen. and III, constituents of the wall of the vessels. There would also be a genetic predisposition, with important variations in the incidence in the various populations (rather low in Europe and in the USA, very high in India, Korea and Japan and very high in Israel). In half of the cases it also causes superficial phlebitis and Raynaud's phenomenon. Histological lesions affect all vascular tunics (panarteritis) without necrosis and with preponderant endoluminal thrombosis.

Differential diagnosis

Unlike other arteriopathies that begin on atheroscleroric lesions, in the Buerger the atheroma is not detectable, generally there is integrity of the internal elastic lamina. In chronicity, the inflammatory infiltrate, which also affects the thrombus itself, turns towards a fibroblastic reaction with perivascular fibrosis and not infrequent recanalization of the thrombus. Characteristically, the adjacent veins and nerves also participate in the inflammatory insult.

Signs and symptoms

The pathology begins gradually, starting from the most distal vessels of the upper and lower limbs up to the most proximal areas. The risk of gangrene is high. Many subjects initially report feeling cold, tingling and burning; Raynaud's phenomenon is a common occurrence. The affected limb (generally the plantar arch or the leg, even if the disease can be manifested to the hands) is registered with intermittent claudication (intermittent lameness) (cf. see more >> chronic obstructive arterial disease). In the case of severe ischemia, as happens for example when you are at a pregangrenal stage or in the case of ulcers or gangrene, the pain becomes continuous. Frequent sensation of cold, sweating and cyanosis of the affected limb. The arterial pulses of one or more of the arteries of the foot and, frequently, of the radial artery are reduced or even absent. Often changes in the color of the skin are observed at the change of posture (for example, you can see pallor when the limb is raised and red when it is lowered). The disease presents with significant pain and paresthesia, both on ischemic and neurological, with areas of digital necrosis, even multiple and bilateral, inconstant peripheral asymmetry and femoral and popliteal polyps present.

Diagnosis

Arteriography demonstrates normal proximal arteries (iliac, femoral and poplitee) with abrupt transition to pathological, segmental and symmetrical tibial strokes, with diffused steno-occlusion of the plantar arch and fingers, and with collateral circles with a typical "corkscrew" appearance ".

Digital plethysmography shows the characteristic alteration of the obstruction wave, distributed to all the fingers. The prognosis is capricious, strongly influenced by the reduction of smoking, with a rate of amputation greater than about 10-35% at 5-10 years. However, unlike in atherosclerotics, survival does not differ much from the general population, as the vital arteries are spared. The therapy is pharmacological and provides, in addition to the abolition of smoking, antiplatelet, vasodilators, prostacyclins, none of which however of proven effectiveness. Lumbar sympathectomy, both surgical and percutaneous, is not very effective and even the distal by-passes are rarely feasible and offer disappointing results.

Kawasaki's disease

Kawasaki disease, also called muco-cutaneous lymph node syndrome, affects children with peak incidence 18-20 months and is rare after five years. It is an autoimmune disease characterized by prolonged fever, exanthema, conjunctivitis, mucositis, cervical lymphadenopathy and polyarthritis of varying severity.It is characteristic the presence of the "strawberry tongue", ie a child has the red tongue, of the color of ripe strawberries, with a series of pins that are the papillae, from which the appearance and, once overcome, the affected subjects present the nails with grooves called Beau lines. Special locations are the coronary, axillary and iliac areas. If not diagnosed in time it can lead to myocardial infarction. It manifests with acute systemic symptoms, such as pyrexia. It is characterized by an infantile vasculitis of the arteries of medium and small size that mainly affects the coronary arteries. It may present aneurysms, also at the coronary level, with overlapping thrombosis or embolism, and significant focal stenoses, outcome of the fibrotic healing processes.
The clinic is characterized by three main stages of the disease:

Acute febrile phase (7-14 days)
It can last up to 3-4 weeks in untreated cases and is characterized by high fever, suffering, septic, anorexia, irritability, an atypical presentation includes pleural effusion. After 8-10 days from the beginning of the fever appears a polyporphic maculopapulose rash morbilliforme or scarlattiniforme pruriginoso and extended to the whole body. Follows non-exudative conjunctival hyperemia, lesions of the oral mucosa (crusting lesions, strawberry tongue, erythema), cheilitis, presence of palpable or painful cervical lymph nodes, hard edema of the hands and feet, fusiform swelling of the fingers, palmar erythema and plantar purple red.
Subacute phase (2-4 weeks)
Characterized by desquamation with large lamellae and sometimes by platelets
Convalescence phase (3 months)
Resolution of the disease with disappearance of clinical signs, normalization of ESR and presence of characteristic transverse nail grooves (Beau lines). Beau's lines are horizontal grooves located on the nail plate, which typically affect most of the nails or all the nails. These alterations are attributable to the interruption of the mitosis of the nail bed caused by serious diseases, pemphigus, elevated fevers, and chemotherapy. Beau lines also develop in patients with Raynaud's disease. Treatment should be directed to the underlying pathology.

Behçet's disease

Behcet's disease is a vasculitis that affects the Mediterranean basin and more frequently affects the eye (iritis) and urogenital mucosa (ulcerations). The disease, also known as the "Silk Road" disease, from the international conference held in Germany in June 2002, has been definitively referred to as "Adamantiades-Behçet's disease", and is a chronic rheumatic disease that causes inflammation of blood vessels (vasculitis ) of unknown cause, although it has already been found in the serum of patients with this endothelial anti-endothelial cell antibody syndrome, type Ig M anti-enolase, and can affect almost any part of the organism (generalized or systemic distribution) and is classified as a rare disease.

It is an autoimmune disease in which the immune system attacks the capillaries producing inflammation (vasculitis). The origin is unknown even if it is believed to appear in genetically predisposed subjects exposed to an external agent, probably an infection. It consists of venous thrombosis and arterial lesions of aneurysmal and / or occlusive type, resulting in a mortality of 20% due to rupture of aortic aneurysms. In addition to the immuno-complex deposition and the subsequent complement activation reaction, the parietal lesions are due to occlusion of the vasa vasorum, with transmural necrosis and formation of pseudoaneurysms. More rarely, perivascular infiltrate is accompanied by endoluminal thrombosis.
Pathogenesis
- The pathogenesis is unknown, in most cases they are usually due to the maternal origin.
- The main anatomopathological lesion is generalized perivascititis with incipient neutrophil infiltration, endothelial edema and fibrinoid necrosis.
- Autoantibodies against endothelial cell alpha-enolase and antibodies against Saccharomyces cerevisiae (ASCA) are also found in advanced stages.
- There is a tendency to the formation of venous thrombi, due to the large endothelial lesion, since having damage in this activates the coagulation system in great power, causing a hypercoagulable state with a large increase in factors VII, XI, VIII, ristocetine and also a deficiency in the c-reactive protein (a potent coagulation inhibitor).
- There is an association with the human leukocyte antigen HLA-B51 and the family association.

Clinic

- recurrent aphthous ulcers deep mouth, central painful, superficial, or yellowish necrotic base, isolated or in outbreaks throughout the oral cavity, which persist for a week or two, and scars.
- Infrequent but more specific genital ulcers are not found in the glans or urethra and leave scars on the scrotum.
- The cutaneous manifestations are: folliculitis, erythema nodoso, rash correlated to the acne and rarely vasculitis.
- A frequent and specific manifestation is the non-specific inflammatory reactivity of the skin to any erosion or intradermal injection of saline (patergia test).
- The most feared injury is bilateral and cicatrizing panuveitis which sometimes degenerates into blindness quickly, usually appears at the beginning.
- There may be posterior uveitis, iritis, obstruction of the retina vessels and sometimes optic neuritis or spontaneous ropture of the retinal blood vessels.
- Non-deforming arthritis of knees and ankles, shoulders and wrists.
- Deep and superficial venous thrombosis in 25% of patients, but pulmonary embolism is rare.
- There may be a lung disease due to pulmonary artery vasculitis.
- The syndrome can influence the parenchyma of the CNS and its prognosis is bleak.
- Intestinal lesions are ulcers that resemble Crohn's disease.
- Laboratory: non-specific, ↑ erythrocyte sedimentation rate, ↑ PCR, leukocytosis, autoantibodies.

Treatment

The cure is absolutely specialized, prescribed by specialized centers
The drugs are aimed at reducing inflammation or trying to regulate the immune system. Some of the drugs used are:
Local corticosteroids, which can be applied directly to skin, mouth or eye injuries to reduce inflammation and pain.
NSAIDs and analgesics, such as aspirin, ibuprofen and paracetamol to relieve joint inflammation and pain.
Colchicine, which is used in the treatment of canker sores.
Oral corticosteroids, such as prednisone, to reduce inflammation.
Pentoxifylline and dapsone are sometimes used.
Immunosuppressants are used to slow down immune activity in severe disease manifestations and require close monitoring of treatment. Azathioprine, cyclosporine, tacrolimus, cyclophosphamide, methotrexate and interferon alpha were used.

Popliteal trapping

Normally the popliteal artery runs between the medial and lateral heads of the twin muscles. In 1-2% of the population (90% males and bilaterality in 1/5) it may be compressed due to congenital anomalies of its course or insertions of the muscular heads. The anomaly, which can also affect the vein, runs asymptomatic in the vast majority of cases, but occasionally can lead to obstructive symptoms in the third or fourth decade of life. The parietal lesions in the atherogenic sense are probably described for microtraumas of muscular contraction, possible aneurysmal evolution and thrombosis or distal embolisms. The diagnosis is not simple, but should be suspected when a claudication arises before the age of 40 in a non-smoker. The peripheral pulses may be normal, but decrease or disappear with the extension of the leg. Echo-doppler and arteriography can highlight the location and extent of the obstruction with changes in limb posture. The investigation of choice is the nuclear magnetic resonance, able to define anatomically the muscle-tendon area and the vascular structures. the treatment is surgical by partial or total section of the medial head of the twin when there is no major compromise of the vessel, but may require a bypass when the artery has already suffered significant damage.

Fibro-muscular dysplasia

It is an anomaly of arterial development of unknown etiology, prevalent in female sex (90% of cases), in which peculiar histological alterations of the wall in hyperplastic or dysplastic sense determine multiple stenoses, mostly eccentric, alternated with ectasies. The most involved arteries are renal (80-90%), external carotids and iliacs. Histologically, they are characterized in different types, in decreasing order of incidence: fibroplasia of the medium tunic (especially in the renal site), perimedial dysplasia, intimal fibroplasia and medium tunic hyperplasia. The symptomatology is indistinguishable from other atherosclerotic steno-occlusive forms. the treatment of choice, as in other localizations, is percutaneous angioplasty, which guarantees good results even at a distance. Adventitial cystic disease. Arterial steno-occlusion is rarely determined by the presence of one or more synovial cysts in the context of the vessel wall itself (sub-adventitial layer). It affects mainly males (80%) and manifests itself around 40 years. The most affected arteries are the popliteal (50-70%), the femoral and the iliac. The etiology is unknown, probably disembrionary due to the inclusion of synovial joint membrane residues. Clinically, at the level of the popliteal cable can be detected a thrill and a breath, with disappearance or weakening of the wrist itself and of the wrists distal to the flexion maneuver of the leg. Ecodoppler and arteriography show the location and length of the stenosis, with a characteristic "scimitar" appearance in the absence of signs of other obstructive pathology.

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