Incidentally detected Monckeberg's sclerosis in a diabetic with coronary artery disease
- 1Department of Medicine, Kasturba Medical College, Manipal, Karnataka, India
- 2Department of Cardiology, Kasturba Medical College, Manipal, Karnataka, India
- Correspondence to Dr G Vivek
We report the case of a 62-year-old diabetic man, who was incidentally detected to have extensive calcification in his upper limb arteries, consistent with Monckeberg's sclerosis. The condition was identified when routine radial puncture attempted in the course of coronary angiography was repeatedly unsuccessful. Coronary angiography and angioplasty was subsequently performed through the femoral route. Monckeberg's sclerosis is a poorly understood condition associated with generalised atherosclerosis and chronic kidney disease. The pathogenesis and natural history of Monckeberg's sclerosis are briefly discussed.
Monckeberg's sclerosis is a disease of unknown aetiology characterised by calcification of the media of small-sized and medium-sized arteries1 first described by Johann Georg Monckeberg in 19032 for whom it is eponymously named. The result is progressive stiffening of the elastic layer of the arterial wall. This loss of elasticity can subsequently lead to systolic hypertension, left ventricular hypertrophy and impaired myocardial perfusion and ultimately increased cardiovascular risk.3
At first believed to be a benign condition exclusively involving small-sized and medium-sized arteries, without thrombosis and luminal obstruction characteristic of atherosclerotic disease4 this condition has now been associated with a multitude of systemic conditions, including atherosclerosis, chronic kidney disease and hyperparathyroidism.5
The aetiology and pathogenesis of Monckeberg's sclerosis remain unclear. Proposed mediators of calcification include abnormal vascular smooth muscle cells with osteoblastic properties, and cytokine modulators of bone mineralisation such as osteoprotegerin, tumour necrosis factor (TNF)-related apoptosis-inducing ligand and receptor activator of nuclear factor kappa B ligand. Monckeberg's sclerosis has also been associated with autonomic neuropathy in diabetics,6 ,7 suggesting that autonomic dysfunction might play an important role in medial calcification. This association is further supported by the increased incidence of vascular calcification following lumbar sympathectomy.8
This case highlights the bidirectional relationship between Monckeberg's sclerosis and coronary artery disease. While ischaemic heart disease is usually looked for in individuals with peripheral arterial calcification, it is equally important to rule out arterial calcification in patients with proven coronary artery disease. Monckeberg's sclerosis can be an unexpected contraindication to arterial puncture in patients planned for coronary angiography and percutaneous intervention.
A 62-year-old man presented with angina at rest since the past 6 days. He was a known diabetic on oral antidiabetic drugs since the past 8 years. There were no other risk factors for ischaemic heart disease. There were also no symptoms suggestive of peripheral arterial insufficiency including paraesthesias and non-healing ulcers in the extremities.
At admission, the patient was haemodynamically stable. All peripheral pulses were palpable; arterial wall thickening was noted in both radial arteries. His hands and feet were warm with no evidence of limb ischaemia. Cardiovascular examination was essentially normal.
Routine laboratory tests including blood glucose, renal (serum creatinine 1.0 mg/dl) and liver function tests and blood counts were all normal. Cardiac enzymes were elevated and suggestive of recent myocardial infarction (creatinine phosphokinase (CPK): 239 U/l, CPK-MB: 7.8 ng/ml, troponin-T: 0.118 ng/ml). ECG showed q waves in leads III and augmented foot lead (aVF), compatible with inferior wall involvement.
Coronary angiography was attempted through a radial route after confirming patency of the palmar arch with modified Allen's test. After repeated failures at negotiating a hydrophilic wire after successful sheath insertion; fluoroscopy revealed extensive calcification of radial and ulnar arteries extending up to the brachial artery. Angiography was subsequently performed via the femoral approach. Coronary angiogram revealed triple vessel disease. The left main coronary artery was normal. The left anterior descending artery had extensive proximal adventitial calcification with a mid 95% discrete stenosis. The left circumflex coronary artery had proximal 90% stenosis with total distal occlusion (Video 1). His right coronary artery had proximal extensive calcification and a pre-crux 95% tubular thrombus-laden stenosis (video 2). x-Rays of hands and forearms performed after unsuccessful radial puncture, showed extensive calcification of radial, ulnar and digital arteries bilaterally (figure 1), consistent with Monckeberg's sclerosis. His lower limb vessels were screened during fluoroscopy and revealed no calcification (figure 2).
Monckeberg's sclerosis involving bilateral radial and ulnar arteries
Calcification secondary to atherosclerosis and plaque formation
Percutaneous angioplasty was performed with drug eluting stents to the right coronary artery and the left anterior descending artery in a staged approach. Medical management with statin and antiplatelets was also initiated. No intervention was attempted for peripheral arterial calcification as there was no evidence of circulatory insufficiency.
Outcome and follow-up
The patient tolerated the procedure well with no postprocedural complications. At 3 months after the event, he continues to be on follow-up at our hospital. There have been no episodes of peripheral ischaemia during this period.
Monckeberg's sclerosis has been described in case reports,3 ,9–12 as well as several case series.1 ,8 ,13–15 Many of these cases required limb and/or penile amputation1 ,12 calling into question the benign prognosis of this condition. One large case series reported intimal calcification on histopathology, 15a feature strongly inconsistent with the conventional definition of Monckeberg's sclerosis.
Our patient showed no symptoms or signs of arterial insufficiency, presenting a clinical picture similar to the classical description of Monckeberg's sclerosis. The coexistence of ischaemic heart disease and diabetes—a known risk factor of atherosclerosis, however, raised important questions about the aetiology of calcification. Although the entire clinical picture could be explained by atherosclerosis alone, extensive nature of the calcification with disproportionate involvement of selected arteries and complete absence of ischaemic symptoms in the affected limbs strongly support a diagnosis of coexistent Monckeberg's sclerosis. The unusual pattern of calcification with isolated involvement of upper limb arteries and sparing of lower limb arteries is not typical of Monckeberg's sclerosis, where lower limb arteries show early calcification. Although a large case series by Chowdhury et al16 showed radial involvement in up to 6.3% of cases the incidence of associated lower limb arterial calcification was not studied. The frequency of upper limb predominant Monckeberg's sclerosis is therefore unclear.
Our case also highlights the difficulty of arterial access for diagnostic or interventional procedures in patients with Monckeberg's sclerosis. In our case, the femoral arteries were uninvolved, affording a second route. However, it is likely that a secondary approach will have to be formulated for patients with widespread arterial calcification preventing arterial access altogether.
We conclude that Monckeberg's sclerosis can coexist with coronary artery disease, and can result in unanticipated difficulty in arterial catheterisation. Although it is important to rule out atherosclerotic disease in all patients with Monckeberg's sclerosis, it is equally important to rule out medial calcification in patients planned for percutaneous angiography and intervention. Although universal screening for Monckeberg's sclerosis is not recommended in patients scheduled for angiography because of unnecessary radiation exposure, we do suggest screening in patients with palpable arterial wall thickening and patients in whom arterial puncture is repeatedly unsuccessful, in order to determine the most suitable site for arterial puncture. Simple fluoroscopy would suffice for both the radial and lower limb arteries.
Monckeberg's sclerosis can coexist with atherosclerotic disease.
Monckeberg's sclerosis can prevent arterial catheterisation, hampering or even prevent percutaneous intervention.
Absence of evidence of arterial insufficiency and disproportionate involvement of selected arteries are useful clues to a diagnosis of Monckeberg's sclerosis in the absence of corroboratory histopathology.