Young Woman With Calf Pain and Fever of Unknown Origin

An 18-year-old Hispanic female with no significant medical history presented to our hospital with severe, debilitating pain and weakness in both calves, intermittent fevers over the past three months, drenching night sweats, and new-onset upper extremity bilateral weakness and pain during the previous three weeks.  Her fevers, which had increased from once weekly to twice daily over the past month, usually arrived in the afternoon, with associated chills, frontal headache, nausea, photophobia, phonophobia, and dizziness.  She also reported decreased appetite and a 5 kg weight loss over the prior six months.

The calf pain and weakness had begun four months earlier, concurrent with an upper respiratory infection that resolved within two weeks. This episode was not associated with cough, fever, chills, or night sweats. She had no prior history of respiratory problems. 

An MRI image of the right calf appears above. What do you think it shows? What other symptoms would you expect to see, and which further tests would you order?

Click here to see the diagnosis and complete case study.

References:

REFERENCES

1. Watts R, Scott DGI. Polyarteritis nodosa and microscopic polyangiitis. In Rheumatology, 5th edn. Edited by: Hochberg MC, Silman AJ, Smolen JS, Weinblatt ME, Weisman MH. Mosby Elselvier, Philadelphia. (2011) 1523-1534.
2. Kamimura T, Hatakeyama M, Torigoe K, et al. Muscular polyarteritis nodosa as a cause of fever of undetermined origin: a case report and review of the literature.Rheumatol Int (2005) 25:394-397.
3. Khellaf M, Hamidou M, Pagnoux C, et al. Vasculitis restricted to the lower limbs: a clinical and histopathological study.Ann Rheum Dis (2007) 66: 554-556.
4. Ahmed S, Kitchen J, Hamilton S, et al. A case of polyarteritis nodosa limited to the right calf muscles, fascia, and skin: a case report.Journal of Medical Case Reports (2011) 5:450.
5. Nakamura T, Tomoda K, Yamamura Y, et al. Polyarteritis nodosa limited to calf muscles: a case report and review of literature.Clin Rheumatol (2003) 22:149-153.
6. Jennette JC, Falk RJ, Bacon PA, et al. 2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides.Arthritis Rheum (2013) 64:1-11.
7. Pagnoux C, Seror R, Henegar C, et al. Clinical features and outcomes in 348 patients with polyarteritis nodosa: a systematic retrospective study of patients diagnosed between 1963 and 2005 and entered into the French Vasculitis Study Group Database.Arthritis Rheum (2010) 62:616-626.
8. Gallien S, Mahr A, Rety F, et al. Magnetic resonance imaging of skeletal muscle involvement in limb restricted vasculitis.Ann Rheum Dis (2002) 61:1107-1109.
9. Balbir-Gurman A, Nahir AM, Braun-Moscovici Y. Intravenous immunoglobulins in polyarteritis nodosa restricted to the limbs: case reports and review of the literature.Clin Exp Rheumatol (2007) 25 (1 suppl 44):S28-30.
10. Carron P, Hoffman IE, De Rycke L, et al. Case number 34: relapse of polyarteritis nodosa presenting as isolated and localized lower limb periostitis.Ann Rheum Dis (2005) 64:1118-1119.
11. Garcia-Porrua C, Mate A, Duran-Marino JL, et al. Localized vasculitis in the calf mimicking deep venous thrombosis.Rheumatology (Oxford) (2002) 41:944-945.
12. Hall C, Mongey AB. Unusual presentation of polyarteritis nodosa.J Rheumatol (2001) 28:871-873.
13. Iwamasa K, Komori H, Niiya Y, et al. A case of polyarteritis nodosa limited to both calves with a low titre of MPO-ANCA.Riyumachi (2001) 41:876-879.
14. Soubrier M, Bangil M, Franc S, et al. Vasculitis confined to the calves. Report of a case. Rev Rheum Engl Ed (1997) 64:414-416.
15. Esteva-Lorenzo FJ, Ferreiro JL, Tardaguila F, et al. Case report 866: pseudotumor of the muscle associated with necrotizing vasculitis of medium and small-sized arteries and chronic myositis.Skeletal Radiol (1994) 23:572-576.
16. Gardner GC, Lawrence MK. Polyarteritis nodosa confined to calf muscles. J Rheumatol (1993) 20:908-909.
17. Garcia F, Pedrol E, Casademont J, et al. Polyarteritis nodosa confined to calf muscles.J Rheumatol (1992) 19:303-305.
18. Hofman DM, Lems WF, Witkamp TB, et al.Demonstration of calf abnormalities by magnetic resonance imaging in polyarteritis nodosa.Clin Rheumatol (1992) 11:402-404.
19. Nash P, Fryer J, Webb J. Vasculitis presenting as chronic unilateral painful leg swelling.J Rheumatol (1988) 15:1022-1025.
20. Ferreiro JE, Saldana MJ, Azevedo SJ. Polyarteritis manifesting as calf myositis and fever.Am J Med (1986) 80:312-315.
21. Laitinen O, Haltia M, Lahdevirta J. Polyarteritis confined to lower extremities.Scand J Rheumatol (1982) 11:71-74.
22. Golding DN. Polyartertitis presenting with leg pains. Br Med J (1970) 1:277-278.23. Eckel CG, Sibbitt RR, Sibbitt WL Jr, et al. A possible role for MRI in polyarteritis nodosa: the “creeping fat” sign.Magn Reson Imaging (1988) 6:713-715.
24. Watts RA, Scott DGI: Epidemiology of vasculitis. In: Vasculitis, (2008) 2nd edition. Edited by: Ball GV, Bridges, SL. Oxford: Oxford University Press; 7-22.
25. Mahr A, Guillevin L, Poissonnet M, Aymé S. Prevalences of polyarteritis nodosa, microscopic polyangiitis, Wegener's granulomatosis, and Churg-Strauss syndrome in a French urban multiethnic population in 2000: a capture-recapture estimate.Arthritis Rheum (2004) 51:92-99.