Fibromuscular dysplasia: unusual cause of abdominal pain. M Alonso-Alcañiz, JM Antolín, JR Rebolledo, C Muñoz-Montano. Healthcare Department of Ocaña I. Se procede al diagnóstico de angiográfico de displasia fibromuscular y ante la imposibilidad de angioplasia se procede a autotrasplante renal con injerto de. Fibromuscular dysplasia: unusual cause of abdominal pain. Displasia fibromuscular: causa infrecuente de dolor abdominal. M. Alonso-Alcañiz, J.M. Antolín, J.R.
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Fibromuscular dysplasia FMD involves small- and medium-sized arteries, being a well-known cause of hypertension in young Caucasian women, when renal arteries are involved. The etiology of FMD remains unknown, despite many theories. A genetic component is suspected to exist, because the pathology affects primarily Caucasians. The major sites affected are renal, cerebral, carotid, visceral, iliac, subclavian, brachial and popliteal arteries.
FMD can manifest as a systemic vascular disease, mimicking vasculitis. This understanding is important because vasculitis and FMD can both dsiplasia a severe clinical course, but require distinct treatments.
Fibromuscular dysplasia – Wikipedia
The differential diagnosis can be difficult in face of an atypical clinical presentation or lack of histopathologic confirmation. Isolated cases of FMD have been reported mimicking the following conditions: Rheumatologists should be aware of this differential diagnosis. Treatment of FMD is recommended only in symptomatic cases, and consists in revascularization, which may be either surgical or via percutaneous transluminal angioplasty.
In FMD, the effects of corticotherapy can directly and rapidly harm the vascular wall, aggravating the lesions.
Fibromuscular dysplasia FMD is a non-inflammatory, non-atherosclerotic vascular disease that involves small- and medium-sized arteries. A genetic component is believed to exist, because the disease affects mainly Caucasians and is associated with the HLA-DRw6 histocompatibility antigen. It is worth noting that FMD was first described in pairs of cousins and monozygous twins.
The female predominance and frequent discovery during pregnancy suggest that estrogen plays a role in the pathogenesis of FMD. In addition to the findings suggesting genetic and hormonal etiologies, FMD has been reported in association with coagulation disorders, such as mutation of factor V Leiden, presence of antiphospholipid antibodies, mechanical stress, 5 and smoking. FMD is classified according to the arterial wall layer primarily affected as follows: Dysplasia of the media is subdivided into medial, perimedial, and hyperplastic medial FMD.
Arterial occlusion and simultaneous involvement of several mediumsized arteries, such as renal, carotid, and mesenteric, can occur mimicking necrotizing vasculitis. Injury of the adventitia is far less frequent. The role of mechanical stress as the etiological factor is corroborated by the predominance of lesions in the right side, because the mobility of the right kidney is greater than that of the left kidney.
The clinical manifestations of FMD are determined by the artery affected and the degree of impairment of arterial blood flow. Cerebrovascular symptoms, resulting from FMD of the carotid artery, and arterial hypertension secondary to FMD of the renal artery are the most common manifestations. The involvement of other arteries can remain asymptomatic.
In patients with FMD of renal artery, because of arterial obstruction, ischemia and progressive loss of the renal parenchyma occur, in addition to the clinical findings of arterial hypertension. The neurological manifestations comprise transient ischemic attacks, cerebral vascular accident, subarachnoid hemorrhage, and unspecific findings, such as headache, vertigo, tinnitus, hemianopsia, 9 ataxic paraparesis, 14 and mental alterations, depending on the brain area affected.
The following have been reported in association with involvement of the nucleus caudatus, 8 due to arterial occlusion: In the presence of stenosis of the coronary arteries, the patient might not have symptoms such as angina, but usually has electrocardiographic alterations, such as ventricular fibrillation, presence of Q wave, and ST-segment elevation.
In case of renal involvement, the only finding on physical examination that might suggest FMD is a dibromuscular and diastolic murmur in the abdomen or flanks. The diagnosis of FMD fibromuscuular established by histopathology or angiography. Involvement of the media is seen as the classical pattern of “pearl necklace”, in which sequential thickening and thinning of the affected arterial segment occurs.
It is worth noting that, when stenosis occurs, occlusion is rarely complete.
FMD is a progressive disease, requiring periodical angiography for patients’ follow-up. Its treatment is still object of discussion, 10 being recommended only in symptomatic cases. The treatment of FMD consists in revascularization, 5 which can be either surgical or via percutaneous transluminal angioplasty PTA.
DFM can manifest as a systemic vascular disease, mimicking polyarteritis nodosa PAN and being called pseudovasculitis. Visceral angiography is an important diagnostic tool, but it lacks specificity.
Both vasculitis and FMD can have a severe clinical course, but require different treatments; thus, it is important fibromuzcular recognize the limitations of angiography in the diagnosis displsaia such diseases.
However, the biological signs of inflammation are absent in about onethird of cases of vasculitis.
In vasculitis, vascular stenosis might occur, causing ischemia of the organ or blood vessel injury, and resulting in aneurysm formation or hemorrhage. The following laboratory findings can occur in some patients: Angiography and biopsy should be performed whenever possible. One characteristic histological image can confirm a diagnosis of vasculitis and exclude other diseases, such as neoplasia and infection.
It can also play a role in establishing the cause of the patient’s deterioration, especially when the kidneys are affected. The classification criteria for vasculitis were established in ; however, when applied to clinical practice, several initial presentations remain difficult to classify. In such situations, the differential diagnosis should always include pseudovasculitis.
This established diagnostic criterion raises the question that the arteriographic profile of those diseases are truly neither pathognomonic nor characteristic. Finding a visceral aneurysm can cause confusion between both diseases. The clinical characteristics of PAN comprise constitutional symptoms, such as fever and weight loss. The involvement of organs in PAN is represented by arterial hypertension, renal failure, peripheral neuropathy, abdominal pain, and impairment of the musculoskeletal system.
Of the later, the discovery of several aneurysmatic dilations up to 1 cm on visceral angiography is considered sufficient for the diagnosis of PAN, even in the absence of histological evidence of the disease, which leads to confusion with FMD.
TA and FMD are vaso-occlusive diseases, and the signs and symptoms of both conditions reflect some degree of damage to the extremity of the organ. The presence of inflammation should suggest the diagnosis of TA; however, in the chronic vaso-occlusive phase of the disease, most patients have no inflammatory signs. It is worth noting that the classification criteria include no inflammation signs, lacking, thus, specificity. Although it does not occur in clinical practice, performing three consecutive biopsies increases the diagnostic probability, because the histopathological aspect of both diseases progresses over time; thus, one single assessment can lead to misdiagnosis.
Marfan’s syndrome is often the first diagnostic consideration in young patients with ascending aorta aneurysms. Although this is not a frequent site of FMD, in the presence of aneurysms it is always a differential diagnosis.
Marfan’s syndrome is an hereditary disorder of the connective tissue due to a mutation in the fibrillin-1 gene in chromosome The typical patient is tall and thin, has arachnodactyly and long limbs. The upper segment of the body is smaller than the lower segment.
In addition, chest deformities, scoliosis, or kyphosis can occur.
The implications of this diagnosis are relevant, since the potentially curative treatment can be not performed, while treatment regimens, such as those with displasoa and cytotoxic agents, can be directly and quickly deleterious to the vascular wall, aggravating the lesions.
Another important differential diagnosis of FMD is atherosclerosis. FMD of the renal arteries is a known cause of secondary hypertension, usually easily differentiated from atherosclerosis of the renal artery, since FMD tends to occur in younger women under the age of 35 yearsat low risk for atherosclerotic cardiovascular disease. FMD of the tibial and fibular arteries has similar symptoms to those of atherosclerosis in the lower limbs. The patients can have intermittent claudication, critical ischemia of the limb, or peripheral microembolism.
In the legs, symptomatic FMD can be treated with peripheral angioplasty. The association of FMD and carotid artery occlusion has been reported.
Clinically, the patient can dosplasia features of displaxia antiphospholipid syndrome APSsuch as visual loss, optic atrophy, angina, and recurring strokes. Anticardiolipin antibodies ACA are a class of acquired immunoglobulins that bind to a disolasia of anionic phospholipids and represent a subset of antiendothelial autoantibodies. The association with arterial or venous occlusions is fibrlmuscular to their thrombogenic nature. Two pathogenic mechanisms have been considered: The presence of vaso-occlusive conditions in different arterial beds should draw our attention not only to true vasculites, but also to other non-inflammatory conditions that mimic vasculites.
Of such conditions, FMD stands out, because displasis can be a diagnostic challenge, and, when missed, determines inadequate management. J Clin Hypertens ;8 Systemic fibromuscular dysplasia masquerading as polyarteritis nodosa. Nephrol Dial Transplant ;11 7: Rev Neurol ; S3: Brachial artery fibromuscular dysplasia.
Ann Vasc Surg ;24 2: Presse Med ;36 6 Pt 2: Curr Opin Rheumatol ;12 1: Isolated intracranial fibromus-cular dysplasia presents as stroke in a year-old female. Arq Neuropsiquiatr ;63 2A: Fibromuscular dysplasia with carotid artery dissection presenting as an isolated hemianopsia. J Stroke Cerebrovasc Dis ;16 fibromusculat Curr Rheumatol Rep ;4 1: Fibrodysplastic popliteal aneurysm and dilatation of pedal artery.
Ann Vasc Surg ;23 6: Dispasia atrophy outcome after revascularization in fibromuscular dysplasia disease. J Endovasc Ther ;9 5: J Neurol Sci ; Ropponen KM, Alafuzoff I. A case of sudden death caused by fibromuscular dysplasia. J Clin Pathol ;52 7: Fibromuscular dysplasia of the carotid artery.
Cerebral infarction attributable to cerebrovascular fibromuscular dysplasia. Brain Nerve ;60 Asymptomatic dissection and occlusion of the basilar artery.