Abstract
Splenic involvement is rarely reported in patients with ANCA-associated vasculitides (AAVs), particularly in those with granulomatosis with polyangiitis (GPA) and is in fact considered to be underestimated. We aimed to investigate the frequency of splenic lesions—mainly infarction—and related factors in patients with AAVs. Patients with AAV whose abdominal or thoracic computed tomographies (CTs) were performed at or after diagnosis were included in the study. CT images were examined for splenic lesions. Overall, 69 patients (median age at diagnosis 54 [IQR 24] years; 55% with GPA, 29% with microscopic polyangiitis, and 16% with renal-limited disease) were included in the analysis. Splenic pathologies were detected in 19 (28%) patients; 12/19 (63%) splenomegaly and 7/19 (37%) splenic infarction. All patients with splenic infarction exhibited GPA with PR3-ANCA-positive serology. Three of these seven patients had autosplenectomy. Patients with splenic infarction were younger at diagnosis (p = 0.018) with also significantly higher ear-nose-throat (ENT) (%100 vs 37; p = 0.002) and eye involvement (%50 vs %12; p = 0.044) than patients without splenic infarction. Splenic pathologies are not rare in AAVs. Furthermore, infarction can help separate GPA from MPA. In young patients with GPA, particularly those with ENT and eye involvements, physicians should consider splenic infarction.
Key Points • Splenic infarction is more common than previously thought in ANCA-associated vasculitides, particularly in granulomatosis with polyangiitis. • Detecting splenic infarction can help differentiate granulomatosis with polyangiitis from other subgroups. |
References
Ghinoi A, Pipitone N, Cavazza A, Boiardi L, Salvarani C (2008) Wegener granulomatosis with spleen infarction: case report and review of the literature. Semin Arthritis Rheum 37:328–333
Gercik O, Solmaz D, Karasu S, Ekinci N, Akar S (2019) Cytomegalovirus disease in a patient with granulomatosis with polyangiitis who also has splenic necrosis. Arch Rheumatol 34:447–450
Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, Flores-Suarez LF, Gross WL, Guillevin L, Hagen EC, Hoffman GS, Jayne DR, Kallenberg CG, Lamprecht P, Langford CA, Luqmani RA, Mahr AD, Matteson EL, Merkel PA, Ozen S, Pusey CD, Rasmussen N, Rees AJ, Scott DG, Specks U, Stone JH, Takahashi K, Watts RA (2013) 2012 revised international Chapel Hill consensus conference nomenclature of vasculitides. Arthritis Rheum 65:1–11
Leavitt RY, Fauci AS, Bloch DA, Michel BA, Hunder GG, Arend WP, Calabrese LH, Fries JF, Lie JT, Lightfoot RW Jr (1990) The American College of Rheumatology 1990 criteria for the classification of Wegener’s granulomatosis. Arthritis Rheum 33:1101–1107
Kool DR, Berger FH, Vos PM (2012) Spleen. In: Scaglione M, Linsenmaier U, Schueller G (eds) Emergency radiology of the abdomen. Springer-Verlag, Berlin Heidelberg, pp 70–71
Johnson CD, Schmit GD (2005) Spleen. In: Stee LA (ed) Mayo Clinic gastrointestinal imaging review. Mayo Clinic Scientific Press and Informa Healthcare, USA, pp 649–662
Fishman D, Isenberg DA (1997) Splenic involvement in rheumatic diseases. Semin Arthritis Rheum 27:141–155
Walton EW (1958) Giant-cell granuloma of the respiratory tract (Wegener’s granulomatosis). Br Med J 2:265–270
Miyawaki Y, Katsuyama T, Sada KE, Taniguchi K, Kakio Y, Wada Y (2016) Development of intracerebral hemorrhage in the short-term clinical course of a patient with microscopic polyangiitis without neurological symptoms at diagnosis: an autopsy case. CEN Case Rep 5:173–178
Nakajima T, Yasuba H, Yamashita K, Kita H, Sumitomo S, Nakata K, Kato M (1999) An autopsy case of MPO-ANCA positive microscopic polyangiitis with manifestations of pulmonary hemorrhage and diffuse alveolar damage. [article in Japanese]. Nihon Kokyuki Gakkai Zasshi 37:807–811
Rentsch J, McColl G (2000) Splenic infarction in Wegener’s granulomatosis. J Rheumatol 27:1554–1555
Bottomley MJ, Gibson M, Alchi B (2019) PR3 vasculitis presenting with symptomatic splenic and renal infarction: a case report and literature review. BMC Nephrol 20:84
Roy DK, George A, Chattopadhyay C, Grennan DM (1999) Splenic infarction in a patient with Wegener’s granulomatosis. Rheumatology (Oxford) 38:1162–1163
Pinching AJ, Lockwood CM, Pussell BA, Rees AJ, Sweny P, Evans DJ, Bowley N, Peters DK (1983) Wegener’s granulomatosis: observations on 18 patients with several renal disease. Q J Med 52:435–460
Jonker ND, Peters AM, Gaskin G, Pusey CD, Lavender JP (1992) A retrospective study of radiolabeled granulocyte kinetics in patients with systemic vasculitis. J Nucl Med 33:491–497
Stone JH, Wegener’s granulomatosis etanercept trial research group (2003) Limited versus severe Wegener’s granulomatosis: baseline data on patients in the Wegener’s granulomatosis etanercept trial. Arthritis Rheum 48:2299–2309
Katikeneni VS, Kant S, Gapud EJ et al (2019) Uncommon presentations in ANCA vasculitis: clinical characteristics and outcomes. Clin Rheumatol 38:2195–2199
Lionaki S, Blyth ER, Hogan SL, Hu Y, Senior BA, Jennette CE, Nachman PH, Jennette JC, Falk RJ (2012) Classification of antineutrophil cytoplasmic autoantibody vasculitides: the role of antineutrophil cytoplasmic antibody specificity for myeloperoxidase or proteinase 3 in disease recognition and prognosis. Arthritis Rheum 64:3452–3462
Jennette JC, Falk RJ, Andrassy K, Bacon PA, Churg J, Gross WL, Hagen EC, Hoffman GS, Hunder GG, Kallenberg CG (1994) Nomenclature of systemic vasculitides: proposal of an international consensus conference. Arthritis Rheum 37:187–192
Watts R, Lane S, Hanslik T, Hauser T, Hellmich B, Koldingsnes W, Mahr A, Segelmark M, Cohen-Tervaert JW, Scott D (2007) Development and validation of a consensus methodology for the classification of ANCA-associated vasculitides and polyarteritis nodosa for epidemiological studies. Ann Rheum Dis 66:222–227
Venbrux AC, Dachman AH, Fishman EK (1993) Vascular disease. In: Dachman AH, Friedman AC (eds) Radiology of the spleen. Mosby Year Book, St. Louis, pp 171–205
Bisharat N, Omari H, Raz R (2001) Risk of infection and death among post-splenectomy patients. J Inf Secur 43:182–186
Waghorn DJ, Mayon-White RT (1997) A study of 42 episodes of overwhelming post-splenectomy infection: is current guidance for asplenic individuals being followed? J Inf Secur 35:289–294
Kanhatu K, Jones P, Cheng AC et al (2017) Spleen Australia guidelines for the prevention of sepsis in patients with asplenia and hyposplenism in Australia and New Zealand. Intern Med J 47:848–855
Davies JM, Lewis MP, Wimperis J, Rafi I, Ladhani S, Bolton-Maggs PH, British Committee for Standards in Haematology (2011) Review of guidelines for the prevention and treatment of infection in patients with an absent or dysfunctional spleen: prepared on behalf of the British Committee for Standards in Haematology by a working party of the Haemato-Oncology task force. Br J Haematol 155:308–317
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Gercik, O., Karasu, S., Solmaz, D. et al. Splenic infarction is not rare in granulomatosis with polyangiitis. Clin Rheumatol 39, 1929–1934 (2020). https://doi.org/10.1007/s10067-020-04993-w
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DOI: https://doi.org/10.1007/s10067-020-04993-w