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CASE REPORT
Year : 2022 | Volume
: 8
| Issue : 3 | Page : 174-177
An unusual case of isolated symmetric bilateral peripheral artery disease of the radial artery of the index finger in systemic sclerosis: A case report and literature review
Debasish Das, Anindya Banerjee, Abhinav Kumar, Shashikant Singh, Subhas Pramanik
Department of Cardiology, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India
Correspondence Address:
Debasish Das
Department of Cardiology, All India Institute of Medical Sciences, Bhubaneswar - 751 019, Odisha
India
Source of Support: None, Conflict of Interest: None
CheckDOI: 10.4103/jpcs.jpcs_20_22
We report an extremely rare and peculiar case of symmetric peripheral small-vessel disease involving only the radial artery of the index finger sparing major vessels in an 80-year-old female with systemic sclerosis (SSc). Our case is the first literature description of bilateral small-vessel peripheral arterial disease only involving the radial artery of the index finger in an octogenarian with SSc. Symmetric small-vessel peripheral artery disease can also be a late manifestation of collagen vascular disease.
Keywords: Bilateral peripheral artery disease, radial artery of the index finger, symmetric, systemic sclerosis
Small-vessel peripheral arterial disease (PAD) commonly occurs as a part of syndromic association with large-vessel peripheral artery disease in atherosclerosis in diabetes mellitus. They commonly present as upper or lower limb claudication or digital gangrene. Isolated symmetric involvement of peripheral small vessels causing digital gangrene is not commonly encountered in diabetes mellitus. Collagen vascular disorders involve the small vessels besides large vessels producing medial fibrosis and constriction of peripheral vessels. Systemic sclerosis (SSc) is notorious to produce endarteritis obliterans in bilateral pulmonary beds causing irreversible pulmonary hypertension. Our case is an interesting, index, and late presentation of SSc presenting as bilateral occlusive symmetric small-vessel PAD of the radial artery of the index finger with bilateral digital gangrene.
Case ReportAn 82-year-old elderly nondiabetic, nonhypertensive female presented to the cardiology outpatient department with bilateral gangrene involving the index finger for the past 3 months [Figure 1]. The tip of the left index finger was amputated locally due to nonhealing dry gangrene 1 month back. For the past 1 month, she noticed blackening of the right index fingertip involving the distal nail bed with severe ischemic rest pain without any ulceration. During the presentation, she had a pulse rate of 82 beats/min with a blood pressure of 130/80 mmHg in the right arm supine position. Cardiovascular system examination was within normal limits without any presence of cardiomegaly and pulmonary arterial hypertension. She had typical scleroderma facies [Figure 2] with a pinched nose and small mouth. Her all serum chemistries were within normal limits including blood sugar and lipid profile. Erythrocyte sedimentation rate was quite high (i.e., 130 mm/h), Anti-nuclear antibody (ANA) was positive, and anti-SCL-70 antibody and anticentromere antibody were positive. Echocardiography was within normal limits with normal biventricular systolic function with grade 1 diastolic dysfunction without any pulmonary arterial hypertension (right ventricular systemic pressure [RVSP]: 18 mmHg). Linear Doppler interrogation of both upper limbs revealed isolated circumferential fibrotic narrowing of the index finger of both palms with turbulent color flow and monophasic Doppler pattern [Figure 3],[Figure 4],[Figure 5],[Figure 6],[Figure 7],[Figure 8]. Interestingly, except for the involvement of the radial artery of the index finger, all other arteries were normal in both the upper limb and lower limb. In view of expanding gangrene of the right index finger, we managed the patient with clopidogrel 75 mg daily with rosuvastatin 20 mg, cilostazol 100 mg twice daily, pentoxifylline 400 mg thrice daily, nitroglycerine 2.6 mg twice daily and injection fondaparinux 2.5 mg SC once daily for 5 days besides disease-modifying agents for SSc. The patient was followed up after 1 month in the cardiology outpatient department, her dry gangrene of the index finger had not progressed, the rest pain of the index finger significantly subsided, and she was quite happier than before. Our case is the first literature illustration of bilateral small-vessel PAD of the radial artery of the index finger in a case of SSc.
Figure 1: Bilateral digital gangrene of the only index finger in systemic sclerosis.
Figure 3: Linear Doppler showing narrowed radial artery of the index finger of the left hand.
Figure 4: Linear Doppler showing narrowed radial artery of the index finger of the right hand. Discussion SSc is characterized by complex fibrotic lesions of connective tissue and vascular bed. It produces dense intimal proliferation and fibrous thickening of the media. Raynaud's phenomenon is also a common problem. Vasospasm, endothelial damage, and abnormalities of hemostasis contribute to the development of ischemia in SSc. The association of the presence of anticentromere antibody with ischemic digit loss has been reported. The presence of anticentromere and antiphospholipid antibodies gives rise to CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia). Veale et al. found the prevalence of macrovascular disease in SSc to be 21.7%.[1] The prevalence of coexistent ischemic heart disease was 15%-20% and the prevalence of cerebrovascular disease was 5%-6% across the patients with SSc. In one out of nine patients with SSc, at least one risk factor for vascular diseases such as hypertension, history of smoking, and hypercholesterolemia was present. Ho et al. demonstrated that PAD is encountered in 17% of the patients with atherosclerosis,[2] and carotid artery involvement is encountered in 64% of the patients with atherosclerosis. They also demonstrated that no conventional cardiovascular risk factor operated behind those patients developing macrovascular disease in SSc. SSc patients experience twice more cardiovascular death as compared to SSc-related death.[3] The overall pattern of increased large-vessel disease associated with cardiovascular events mirrors that of atherosclerotic disease. Large studies have demonstrated a major decrease in cardiovascular death with the use of antiplatelet agents in SSc. Aspirin is poorly tolerated in SSc due to esophageal abnormalities[4],[5] for which clopidogrel serves as a better agent in SSc.[6] We treated the patient with clopidogrel and moderate-dose rosuvastatin in view of her elderly age. PAD in SSc responds well to the combination therapy of sildenafil and bosentan.[7] Stafford et al. described an interesting mode of vascular involvement in SSc. They observed ulnar arteries in patients with SSc were significantly narrower and smoothly thickened as compared to the controls.[8] In another series of brachial angiography, 12 out of 19 patients with SSc had ulnar artery occlusion and only two patients had radial artery occlusion.[9] Hasegawa et al. reported that seven out of eight cases of SSc with digital gangrene had upper extremity large-vessel PAD. Out of seven, three had occlusion limited to the digital arteries, three had occlusion in the ulnar artery, and one had occlusion of the radial artery.[10] Isolated involvement of a single digital artery in a symmetric manner in SSc has not been described so far, our case is an interesting and unique description of the same. The index patient had normal bilateral radial and ulnar arteries. Intravenous prostacyclin like epoprostenol or iloprost (0.5-2 ng/Kg/min) infusion for 1-3 days each infusion lasting 6 h remains an alternative option in acute limb ischemia.[11] Man et al. described the risk of developing PAD is four times higher in patients with SSc than the patients not having SSc.[12] Nordin et al. found the presence of anticentromere antibody in patients with SSc is associated with more incidence of PADs.[13] Atherothrombosis plays a critical role in patients with SSc with twofold more risk of having myocardial infarction and stroke.[14] SSc in toto is a multi-organ fibrotic autoimmune disease. Microvascular involvement in SSc is well known resulting in digital gangrene and tissue loss. Capillaroscopy well demonstrates the microvasculopathy in SSc.[15] Approximately 4.8% of patients with SSc require amputation of digits. Elderly age, long history of systemic sclerosis, presence of anticentromere antibody, coexistent PAD, and hypercholesterolemia are more associated with the development of digital gangrene and amputation.[16] The cause of peculiar involvement of the bilateral radial artery of the index finger in this index patient is unknown; PAD in systemic sclerosis can also be a bilateral mirror image.
ConclusionOur case is unique and the first literature description of bilateral small-vessel PAD only involves the radial artery of the index finger in an octogenarian with SSc. PAD in SSc can rarely be a mirror image even in the smaller vascular territory.
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