J Rheum Dis.  2021 Oct;28(4):242-246. 10.4078/jrd.2021.28.4.242.

A Case of Overlap Syndrome of Systemic Sclerosis and Cryoglobulinemic Vasculitis With Central Nervous System Involvement

• Jung Y
• Emily Lee E
• Yeo J
• Kim MH
• Choi SR
• Song YW

• KMID: 2520437
• DOI: http://doi.org/10.4078/jrd.2021.28.4.242

Abstract

Mixed cryoglobulinemic vasculitis (CV) is occasionally caused by autoimmune diseases including systemic sclerosis. Multiorgan involvement such as skin, kidney, and peripheral nerve involvement is common in mixed CV. However, central nervous system (CNS) involvement is extremely rare. Here, we report a case of overlap syndrome of limited cutaneous systemic sclerosis and mixed cryoglobulinemic vasculitis with CNS involvement. The neurologic deficits and systemic symptoms improved promptly after steroid and cyclophosphamide therapy.

Keyword

Cryoglobulinemia; Vasculitis; Central nervous system; Scleroderma; systemic

Figure

• 

  Figure 1 Brain MRI shows a number of multifocal patchy T2 high signal intensity areas in the bilateral cerebral hemisphere (A) and a 4.5-cm-sized lesion with inner hemorrhage with subtle enhancement on the right posterior lobe in the T2 FLAIR (B) and DWI images (C). MRI: magnetic resonance imaging, FLAIR: fluid attenuated inversion recovery, DWI: diffusion-weighted imaging.

• 

  Figure 2 Lateral view (A) and anterior-posterior view (B) of the conventional cerebral angiography shows diffuse stenosis and beaded pattern (white arrows) of the anterior and middle cerebral arteries and their branches. The right distal vertebral artery and basilar artery trunk show diffuse luminal narrowing with dysplastic changes (white arrows) (C) compared to the left side (D).

Reference

1. Brouet JC, Clauvel JP, Danon F, Klein M, Seligmann M. 1974; Biologic and clinical significance of cryoglobulins. A report of 86 cases. Am J Med. 57:775–88. DOI: 10.1016/0002-9343(74)90852-3. PMID: 4216269.

2. Trejo O, Ramos-Casals M, García-Carrasco M, Yagüe J, Jiménez S, de la Red G, et al. 2001; Cryoglobulinemia: study of etiologic factors and clinical and immunologic features in 443 patients from a single center. Medicine (Baltimore). 80:252–62. DOI: 10.1097/00005792-200107000-00004. PMID: 11470986.

3. Terrier B, Karras A, Kahn JE, Le Guenno G, Marie I, Benarous L, et al. 2013; The spectrum of type I cryoglobulinemia vasculitis: new insights based on 64 cases. Medicine (Baltimore). 92:61–8. DOI: 10.1097/MD.0b013e318288925c. PMID: 23429354. PMCID: PMC4553985.

4. Giuggioli D, Manfredi A, Colaci M, Manzini CU, Antonelli A, Ferri C. 2013; Systemic sclerosis and cryoglobulinemia: our experience with overlapping syndrome of scleroderma and severe cryoglobulinemic vasculitis and review of the literature. Autoimmun Rev. 12:1058–63. DOI: 10.1016/j.autrev.2013.06.013. PMID: 23806565.

5. Ramos-Casals M, Trejo O, García-Carrasco M, Cervera R, Font J. 2000; Mixed cryoglobulinemia: new concepts. Lupus. 9:83–91. DOI: 10.1191/096120300678828127. PMID: 10787003.

6. Terrier B, Krastinova E, Marie I, Launay D, Lacraz A, Belenotti P, et al. 2012; Management of noninfectious mixed cryoglobulinemia vasculitis: data from 242 cases included in the CryoVas survey. Blood. 119:5996–6004. DOI: 10.1182/blood-2011-12-396028. PMID: 22474249.

7. Quéméneur T, Mouthon L, Cacoub P, Meyer O, Michon-Pasturel U, Vanhille P, et al. 2013; Systemic vasculitis during the course of systemic sclerosis: report of 12 cases and review of the literature. Medicine (Baltimore). 92:1–9. DOI: 10.1097/MD.0b013e31827781fd. PMID: 23263715. PMCID: PMC5370746.

8. Silva F, Pinto C, Barbosa A, Borges T, Dias C, Almeida J. 2019; New insights in cryoglobulinemic vasculitis. J Autoimmun. 105:102313. DOI: 10.1016/j.jaut.2019.102313. PMID: 31383568.

9. Desbois AC, Cacoub P, Saadoun D. 2019; Cryoglobulinemia: an update in 2019. Joint Bone Spine. 86:707–13. DOI: 10.1016/j.jbspin.2019.01.016. PMID: 30731128.

10. Petty GW, Duffy J, Houston J 3rd. 1996; Cerebral ischemia in patients with hepatitis C virus infection and mixed cryoglo-bulinemia. Mayo Clin Proc. 71:671–8. DOI: 10.1016/S0025-6196(11)63005-X. PMID: 8656709.

11. Feldman L, Dhamne M, Li Y. 2019; Neurologic manifestations associated with cryoglobulinemia: a single center experience. J Neurol Sci. 398:121–7. DOI: 10.1016/j.jns.2019.01.041. PMID: 30708209.

12. Ristow SC, Griner PF, Abraham GN, Shoulson I. 1976; Reversal of systemic manifestations of cryoglobulinemia. Treatment with melphalan and prednisone. Arch Intern Med. 136:467–70. DOI: 10.1001/archinte.1976.03630040069014. PMID: 1267555.

13. Byram K, Hajj-Ali RA, Calabrese L. 2018; CNS vasculitis: an approach to differential diagnosis and management. Curr Rheumatol Rep. 20:37. DOI: 10.1007/s11926-018-0747-z. PMID: 29846828.

14. Fugate JE, Rabinstein AA. 2015; Posterior reversible encephalopathy syndrome: clinical and radiological manifestations, pathophysiology, and outstanding questions. Lancet Neurol. 14:914–25. DOI: 10.1016/S1474-4422(15)00111-8. PMID: 26184985.

15. Stott VL, Hurrell MA, Anderson TJ. 2005; Reversible posterior leukoencephalopathy syndrome: a misnomer reviewed. Intern Med J. 35:83–90. DOI: 10.1111/j.1445-5994.2004.00750.x. PMID: 15705136.