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Ann Saudi Med. 2010 May-Jun; 30(3): 245.
PMCID: PMC2886881

RE: Reactive macrophage activation syndrome (MAS) in a patient with parvovirus B19 infection, lymphocytic lichenoid vasculitis, urticaria and angioedema

To the Editor: I read with keen interest the article published by Soldo-Juresa et al in the last issue of the journal.1 I would like to make some comments: First, Still disease should be kept in mind whenever macrophage activation syndrome is considered in a patient with the clinical picture shown. In fact the patient's condition fulfills the criteria published by Yamagushi for Still disease.2 I would like to remark that the measurement of soluble IL-2R (also known as CD25s) could be helpful in distinguishing between Still disease and MAS.3 Furthermore, the development of MAS in Still disease is not infrequent and intravenous gamma globulins have shown promising results in treatment.4,5 Second, as the authors rule out a C1q-linked vasculitis, C4 and alternative complement pathway activity should be measured. It is well known that in esterase inhibitor type II levels of C1-inhibitor esterase are normal although C4 is decreased due to low functional C1-inhibitor levels.6 Hypocomplementemic vasculitis has been also described in patients with factor I deficiency.7 I have attended recently a patient with vasculitis secondary to factor I deficiency. This factor was not measured by the author in this patient. Third, the authors recognize that DNA of parvovirus B-19 can persist in bone marrow for years. This is why we should distinguish between MAS secondary to viral infection or underlying Still disease that needs long-term treatment and follow-up.


1. Soldo-Juresa D, Radman M, Pejsa V, Bozikov V. Reactive macrophage activation syndrome in a patient with parvovirus B19 infection, lymphocytic lichenoid vasculitis, urticaria and angioedema. Ann Saudi Med. 2010;30:88–9. [PMC free article] [PubMed]
2. Yamaguchi M, Ohta A, Tsunematsu T, Kasukawa R, Mizushima Y, Kashiwagi H, Kashiwazaki S, Tanimoto K, Matsumoto Y, Ota T. Preliminary criteria for classification of adult Stills disease. J Rheumatol. 1992;19:424–430. [PubMed]
3. Bleesing J, Prada A, Siegel DM, Villanueva J, Olson J, Ilowite NT, Brunner HI, Griffin T, Graham TB, Sherry DD, Passo MH, Ramanan AV, Filipovich A, Grom AA. The diagnostic significance of soluble CD163 and soluble interleukine-2 receptor a -chain in macrophage activation syndrome and untreated new-onset systemic juvenile idiopathic arthritis. Arthritis Rheum. 2007;56:965–71. [PubMed]
4. García-Consuegra Molina J, Merino Muñoz R, de Inocencio Arocena J. Grupo de Estudio del Síndrome de Activación Macrofágica y Artritis Idiopática Juvenil de la Sociedad Española de Reumatología Pediátrica. Macrophage activation syndrome and juvenile idiopathic arthritis. A multicenter study. An Pediatr (Barc) 2008;68:110–6. [PubMed]
5. Vignes S, Wechsler B, Amoura Z, Papo T, Francès C, Huong DL, Veyssier P, Godeau P, Piette JC. Intravenous immunoglobulin in adult Still's disease refractory to non-steroidal anti-inflammatory drugs. Clin Exp Rheumatol. 1998;16:295–8. [PubMed]
6. Zuraw BL, Christiansen SC. Pathogenesis and laboratory diagnosis of hereditary angioedema. Allergy Asthma Proc. 2009;30:487–92. [PubMed]
7. Sadallah S, Gudat F, Laissue JA, Spath PJ, Schifferli JA. Glomerulonephritis in a patient with complement factor I deficiency. Am J Kidney Dis. 1999 Jun;33(6):1153–7. [PubMed]

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