Skip to main content

Advertisement

Log in

Headaches Related to Rheumatologic Disease

  • Secondary Headache (K Henry, Section Editor)
  • Published:
Current Pain and Headache Reports Aims and scope Submit manuscript

Abstract

Headaches are a common, but under-recognized and understudied symptom in the context of the rheumatic diseases. They can result from intracranial pathology, such as parenchymal and meningeal inflammation, thrombosis, space-occupying lesions, and more. Inflammation, irritation, or degeneration of anatomically related structures such as the eyes, neck, and sinuses can equally cause headaches. In addition, patients with rheumatologic disorders have the same tendencies as the general population to develop primary headaches. While the latter are benign in nature, and generally require only symptomatic relief, the former type of headaches may signal disease manifestation, progression, or complication. Thus, familiarity with common and uncommon headache syndromes related to rheumatologic disorders as well as with their possible underlying disease processes and mechanisms is important. This will help to successfully develop an effective approach toward the evaluation of patients presenting with headaches in a rheumatologic context, and, ultimately, diagnose and treat potentially severe underlying disease.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

References

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

  1. Hunder GG, Arend WP, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of vasculitis. Introduction. Arthritis Rheum. 1990;33:1065–7.

    Article  PubMed  CAS  Google Scholar 

  2. Jennette JC, Falk RJ, Andrassy K, et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum. 1994;37:187–92.

    Article  PubMed  CAS  Google Scholar 

  3. Birnbaum J, Hellmann DB. Primary angiitis of the central nervous system. Arch Neurol. 2009;66:704–9.

    Article  PubMed  Google Scholar 

  4. Gerretsen P, Kern RZ. Reversible cerebral vasoconstriction syndrome or primary angiitis of the central nervous system? Can J Neurol Sci. 2007;34:467–77.

    PubMed  Google Scholar 

  5. Lie JT. Primary (granulomatous) angiitis of the central nervous system: a clinicopathologic analysis of 15 new cases and a review of the literature. Hum Pathol. 1992;23:164–71.

    Article  PubMed  CAS  Google Scholar 

  6. • Ducros A. Reversible cerebral vasoconstriction syndrome. Lancet Neurol. 2012;11:906–17. A detailed review of reversible cerebral vasoconstriction from a “neurology point of view”.

    Article  PubMed  Google Scholar 

  7. Calabrese LH, Gragg LA, Furlan AJ. Benign angiopathy: a distinct subset of angiographically defined primary angiitis of the central nervous system. J Rheumatol. 1993;20:2046–50.

    PubMed  CAS  Google Scholar 

  8. Molloy ES, Hajj-Ali RA. Primary angiitis of the central nervous system. Curr Treat Options Neurol. 2007;9:169–75.

    Article  PubMed  Google Scholar 

  9. • Gonzalez-Gay MA, Martinez-Dubois C, Agudo M, et al. Giant cell arteritis: epidemiology, diagnosis, and management. Curr Rheumatol Rep. 2010;12:436–42. A review of temporal arteritis for the busy reader - concise and up-to-date.

    Article  PubMed  Google Scholar 

  10. Weyand CM, Goronzy JJ. Giant-cell arteritis and polymyalgia rheumatica. Ann Intern Med. 2003;139:505–15.

    Article  PubMed  Google Scholar 

  11. Weyand CM, Hicok KC, Hunder GG, Goronzy JJ. Tissue cytokine patterns in patients with polymyalgia rheumatica and giant cell arteritis. Ann Intern Med. 1994;121:484–91.

    Article  PubMed  CAS  Google Scholar 

  12. Cullen JF, Coleiro JA. Ophthalmic complications of giant cell arteritis. Surv Ophthalmol. 1976;20:247–60.

    Article  PubMed  CAS  Google Scholar 

  13. Niederkohr RD, Levin LA. A Bayesian analysis of the true sensitivity of a temporal artery biopsy. Invest Ophthalmol Vis Sci. 2007;48:675–80.

    Article  PubMed  Google Scholar 

  14. Younge BR, Cook Jr BE, Bartley GB, et al. Initiation of glucocorticoid therapy: before or after temporal artery biopsy? Mayo Clin Proc. 2004;79:483–91.

    Article  PubMed  Google Scholar 

  15. Achkar AA, Lie JT, Hunder GG, et al. How does previous corticosteroid treatment affect the biopsy findings in giant cell (temporal) arteritis? Ann Intern Med. 1994;120:987–92.

    Article  PubMed  CAS  Google Scholar 

  16. Nahas SJ. Headache and temporal arteritis: when to suspect and how to manage. Curr Pain Headache Rep. 2012;16:371–8.

    Article  PubMed  Google Scholar 

  17. Schmidt WA, Kraft HE, Vorpahl K, et al. Color duplex ultrasonography in the diagnosis of temporal arteritis. N Engl J Med. 1997;337:1336–42.

    Article  PubMed  CAS  Google Scholar 

  18. Karassa FB, Matsagas MI, Schmidt WA, Ioannidis JP. Meta-analysis: test performance of ultrasonography for giant-cell arteritis. Ann Intern Med. 2005;142:359–69.

    Article  PubMed  Google Scholar 

  19. Arida A, Kyprianou M, Kanakis M, Sfikakis PP. The diagnostic value of ultrasonography-derived edema of the temporal artery wall in giant cell arteritis: a second meta-analysis. BMC Musculoskelet Disord. 2010;11:44.

    Article  PubMed  Google Scholar 

  20. Black R, Roach D, Rischmueller M, et al. The use of temporal artery ultrasound in the diagnosis of giant cell arteritis in routine practice. Int J Rheum Dis. 2013;16:352–7.

    Article  PubMed  Google Scholar 

  21. Bley TA, Uhl M, Carew J, et al. Diagnostic value of high-resolution MR imaging in giant cell arteritis. Am J Neuroradiol. 2007;28:1722–7.

    Article  PubMed  CAS  Google Scholar 

  22. Schmidt WA. Imaging in vasculitis. Best Pract Res Clin Rheumatol. 2013;27:107–18.

    Article  PubMed  Google Scholar 

  23. Brodmann M, Lipp RW, Passath A, et al. The role of 2-18F-fluoro-2-deoxy-D-glucose positron emission tomography in the diagnosis of giant cell arteritis of the temporal arteries. Rheumatology. 2004;43:241–2.

    Article  PubMed  CAS  Google Scholar 

  24. Besson FL, Parienti JJ, Bienvenu B, et al. Diagnostic performance of (1)(8)F-fluorodeoxyglucose positron emission tomography in giant cell arteritis: a systematic review and meta-analysis. Eur J Nucl Med Mol Imaging. 2011;38:1764–72.

    Article  PubMed  Google Scholar 

  25. Gonzalez-Gay MA, Blanco R, Rodriguez-Valverde V, et al. Permanent visual loss and cerebrovascular accidents in giant cell arteritis: predictors and response to treatment. Arthritis Rheum. 1998;41:1497–504.

    Article  PubMed  CAS  Google Scholar 

  26. Hunder GG, Sheps SG, Allen GL, Joyce JW. Daily and alternate-day corticosteroid regimens in treatment of giant cell arteritis: comparison in a prospective study. Ann Intern Med. 1975;82:613–8.

    Article  PubMed  CAS  Google Scholar 

  27. Mazlumzadeh M, Hunder GG, Easley KA, et al. Treatment of giant cell arteritis using induction therapy with high-dose glucocorticoids: a double-blind, placebo-controlled, randomized prospective clinical trial. Arthritis Rheum. 2006;54:3310–8.

    Article  PubMed  CAS  Google Scholar 

  28. Villa-Forte A. Giant cell arteritis: suspect it, treat it promptly. Cleve Clin J Med. 2011;78:265–70.

    Article  PubMed  Google Scholar 

  29. Berger CT, Wolbers M, Meyer P, et al. High incidence of severe ischaemic complications in patients with giant cell arteritis irrespective of platelet count and size, and platelet inhibition. Rheumatology. 2009;48:258–61.

    Article  PubMed  CAS  Google Scholar 

  30. Hall S, Barr W, Lie JT, et al. Takayasu arteritis. A study of 32 North American patients. Medicine. 1985;64:89–99.

    Article  PubMed  CAS  Google Scholar 

  31. Brunner J, Feldman BM, Tyrrell PN, et al. Takayasu arteritis in children and adolescents. Rheumatology. 2010;49:1806–14.

    Article  PubMed  Google Scholar 

  32. Schmidt J, Kermani TA, Bacani AK, et al. Diagnostic features, treatment, and outcomes of takayasu arteritis in a US Cohort of 126 patients. Mayo Clin Proc. 2013;88:822–30.

    Article  PubMed  Google Scholar 

  33. Rossi CM, Di Comite G. The clinical spectrum of the neurological involvement in vasculitides. J Neurol Sci. 2009;285:13–21.

    Article  PubMed  Google Scholar 

  34. Rosenberg MR, Parshley M, Gibson S, Wernick R. Central nervous system polyarteritis nodosa. West J Med. 1990;153:553–6.

    PubMed  CAS  Google Scholar 

  35. Watts RA, Carruthers DM, Scott DG. Epidemiology of systemic vasculitis: changing incidence or definition? Semin Arthritis Rheum. 1995;25:28–34.

    Article  PubMed  CAS  Google Scholar 

  36. Seo P, Stone JH. The antineutrophil cytoplasmic antibody-associated vasculitides. Am J Med. 2004;117:39–50.

    Article  PubMed  CAS  Google Scholar 

  37. Chhetri MK, Bhattacharya AK, Kumar T. Headache in rheumatological disorders. J Indian Med Assoc. 2011;109(646):648–9.

    Google Scholar 

  38. Jones RB, Tervaert JW, Hauser T, et al. Rituximab vs cyclophosphamide in ANCA-associated renal vasculitis. N Engl J Med. 2010;363:211–20.

    Article  PubMed  CAS  Google Scholar 

  39. Stone JH, Merkel PA, Spiera R, et al. Rituximab vs cyclophosphamide for ANCA-associated vasculitis. N Engl J Med. 2010;363:221–32.

    Article  PubMed  CAS  Google Scholar 

  40. Idil A, Gurler A, Boyvat A, et al. The prevalence of Behcet’s disease above the age of 10 years. The results of a pilot study conducted at the Park Primary Health Care Center in Ankara, Turkey. Ophthalmic Epidemiol. 2002;9:325–31.

    Article  PubMed  CAS  Google Scholar 

  41. Azizlerli G, Kose AA, Sarica R, et al. Prevalence of Behcet’s disease in Istanbul, Turkey. Int J Dermatol. 2003;42:803–6.

    Article  PubMed  Google Scholar 

  42. Siva A, Saip S. The spectrum of nervous system involvement in Behcet’s syndrome and its differential diagnosis. J Neurol. 2009;256:513–29.

    Article  PubMed  Google Scholar 

  43. Saip S, Siva A, Altintas A, et al. Headache in Behcet’s syndrome. Headache. 2005;45:911–9.

    Article  PubMed  Google Scholar 

  44. Hatemi G, Silman A, Bang D, et al. EULAR recommendations for the management of Behcet disease. Ann Rheum Dis. 2008;67:1656–62.

    Article  PubMed  CAS  Google Scholar 

  45. Kotake S, Higashi K, Yoshikawa K, et al. Central nervous system symptoms in patients with Behcet disease receiving cyclosporine therapy. Ophthalmology. 1999;106:586–9.

    Article  PubMed  CAS  Google Scholar 

  46. Kotter I, Gunaydin I, Batra M, et al. CNS involvement occurs more frequently in patients with Behcet’s disease under cyclosporin A (CSA) than under other medications—results of a retrospective analysis of 117 cases. Clin Rheumatol. 2006;25:482–6.

    Article  PubMed  Google Scholar 

  47. Kato Y, Numaga J, Kato S, et al. Central nervous system symptoms in a population of Behcet’s disease patients with refractory uveitis treated with cyclosporine A. Clin Experiment Ophthalmol. 2001;29:335–6.

    Article  PubMed  CAS  Google Scholar 

  48. Cordato DJ, Djekic S, Taneja SR, et al. Prevalence of positive syphilis serology and meningovascular neurosyphilis in patients admitted with stroke and TIA from a culturally diverse population (2005-09). J Clin Neurosci. 2013;20:943–7.

    Article  PubMed  Google Scholar 

  49. Zhang HL, Lin LR, Liu GL, et al. Clinical spectrum of neurosyphilis among HIV-negative patients in the modern era. Dermatology. 2013;226:148–56.

    Article  PubMed  CAS  Google Scholar 

  50. Ahmad NM, Boruchoff SE. Multiple cerebral infarcts due to varicella-zoster virus large-vessel vasculopathy in an immunocompetent adult without skin involvement. Clin Infect Dis. 2003;37:e16–8.

    Article  PubMed  Google Scholar 

  51. Constantinescu CS. Association of varicella-zoster virus with cervical artery dissection in 2 cases. Arch Neurol. 2000;57:427.

    Article  PubMed  CAS  Google Scholar 

  52. Fukumoto S, Kinjo M, Hokamura K, Tanaka K. Subarachnoid hemorrhage and granulomatous angiitis of the basilar artery: demonstration of the varicella-zoster-virus in the basilar artery lesions. Stroke. 1986;17:1024–8.

    Article  PubMed  CAS  Google Scholar 

  53. Gilden D, Cohrs RJ, Mahalingam R, Nagel MA. Laboratory features, pathogenesis, and treatment. Lancet Neurol. 2009;8:731–40.

    Article  PubMed  Google Scholar 

  54. Kapoor S. Headache attributed to cranial or cervical vascular disorders. Curr Pain Headache Rep. 2013;17:334.

    Article  PubMed  Google Scholar 

  55. • Amaral TN, Peres FA, Lapa AT, et al. Neurologic involvement in scleroderma systematic review. Semin Arthritis Rheum. 2013. doi:10.1016/j.semarthrit.2013.05.002. Sheds new light on the probably underestimated prevalence of neurological involvement in scleroderma.

  56. Kupersmith MJ, Martin V, Heller G, et al. Idiopathic hypertrophic pachymeningitis. Neurology. 2004;62:686–94.

    Article  PubMed  CAS  Google Scholar 

  57. Chan SK, Cheuk W, Chan KT, Chan JK. IgG4-related sclerosing pachymeningitis: a previously unrecognized form of central nervous system involvement in IgG4-related sclerosing disease. Am J Surg Pathol. 2009;33:1249–52.

    Article  PubMed  Google Scholar 

  58. Wallace ZS, Carruthers MN, Khosroshahi A, et al. IgG4-related disease and hypertrophic pachymeningitis. Medicine. 2013;92:206–16.

    Article  PubMed  CAS  Google Scholar 

  59. Baughman R, Bresnitz E, Iannuzzi M, et al. Design of a case control etiologic study of sarcoidosis (ACCESS). ACCESS Research Group. J Clin Epidemiol. 1999;52:1173–86.

    Google Scholar 

  60. Judson MA. Extrapulmonary sarcoidosis. Semin Respir Crit Care Med. 2007;28:83–101.

    Article  PubMed  Google Scholar 

  61. Hoitsma E, Drent M, Sharma OP. A pragmatic approach to diagnosing and treating neurosarcoidosis in the 21st century. Curr Opin Pulm Med. 2010;16:472–9.

    Article  PubMed  Google Scholar 

  62. Stern BJ. Neurological complications of sarcoidosis. Curr Opin Neurol. 2004;17:311–6.

    Article  PubMed  Google Scholar 

  63. Vargas DL, Stern BJ. Neurosarcoidosis: diagnosis and management. Semin Respir Crit Care Med. 2010;31:419–27.

    Article  PubMed  Google Scholar 

  64. Pawate S, Moses H, Sriram S. Presentations and outcomes of neurosarcoidosis: a study of 54 cases. QJM. 2009;102:449–60.

    Article  PubMed  CAS  Google Scholar 

  65. Scott TF, Yandora K, Valeri A, et al. Aggressive therapy for neurosarcoidosis: long-term follow-up of 48 treated patients. Arch Neurol. 2007;64:691–6.

    Article  PubMed  Google Scholar 

  66. Marnane M, Lynch T, Scott J, et al. Steroid-unresponsive neurosarcoidosis successfully treated with adalimumab. J Neurol. 2009;256:139–40.

    Article  PubMed  Google Scholar 

  67. Pereira J, Anderson NE, McAuley D, et al. Medically refractory neurosarcoidosis treated with infliximab. Intern Med J. 2011;41:354–7.

    Article  PubMed  CAS  Google Scholar 

  68. Santos E, Shaunak S, Renowden S, Scolding NJ. Treatment of refractory neurosarcoidosis with Infliximab. J Neurol Neurosurg Psychiatry. 2010;81:241–6.

    Article  PubMed  Google Scholar 

  69. Sodhi M, Pearson K, White ES, Culver DA. Infliximab therapy rescues cyclophosphamide failure in severe central nervous system sarcoidosis. Respir Med. 2009;103:268–73.

    Article  PubMed  Google Scholar 

  70. •• Muscal E, Brey RL. Neurologic manifestations of systemic lupus erythematosus in children and adults. Neurol Clin. 2010;28:61–73. An excellent overview of neuro-psychiatric SLE, its prevalence, manifestations, and treatment.

    Article  PubMed  Google Scholar 

  71. Popescu A, Kao AH. Neuropsychiatric systemic lupus erythematosus. Curr Neuropharmacol. 2011;9:449–57.

    Article  PubMed  CAS  Google Scholar 

  72. Bicakci S, Ozbek S, Bicakci K, et al. Recurrent headache and MRI findings in systemic lupus erythematosus. J Natl Med Assoc. 2008;100:323–6.

    PubMed  Google Scholar 

  73. Barber CE, Leclerc R, Gladman DD, et al. Posterior reversible encephalopathy syndrome: an emerging disease manifestation in systemic lupus erythematosus. Semin Arthritis Rheum. 2011;41:353–63.

    Article  PubMed  Google Scholar 

  74. Gatla N, Annapureddy N, Sequeira W, Jolly M. Posterior reversible encephalopathy syndrome in systemic lupus erythematosus. J Clin Rheumatol. 2013;19:334–40.

    Article  PubMed  Google Scholar 

  75. Lai CC, Chen WS, Chang YS, et al. Clinical features and outcomes of posterior reversible Encephalopathy Syndrome in patients with Systemic Lupus Erythematosus. Arthritis Care Res. 2013 doi:10.1002/acr.22047.

  76. Liu B, Zhang X, Zhang FC, et al. Posterior reversible encephalopathy syndrome could be an underestimated variant of “reversible neurological deficits” in Systemic Lupus Erythematosus. BMC Neurol. 2012;12:152.

    Article  PubMed  Google Scholar 

  77. Kim JM, Kwok SK, Ju JH, et al. Idiopathic intracranial hypertension as a significant cause of intractable headache in patients with systemic lupus erythematosus: a 15-year experience. Lupus. 2012;21:542–7.

    Article  PubMed  Google Scholar 

  78. Abu-Shakra M, Gladman DD, Urowitz MB, Farewell V. Anticardiolipin antibodies in systemic lupus erythematosus: clinical and laboratory correlations. Am J Med. 1995;99:624–8.

    Article  PubMed  CAS  Google Scholar 

  79. Danowski A, de Azevedo MN, de Souza Papi JA, Petri M. Determinants of risk for venous and arterial thrombosis in primary antiphospholipid syndrome and in antiphospholipid syndrome with systemic lupus erythematosus. J Rheumatol. 2009;36:1195–9.

    Article  PubMed  CAS  Google Scholar 

  80. Widener HL, Littman BH. Ibuprofen-induced meningitis in systemic lupus erythematosus. JAMA. 1978;239:1062–4.

    Article  PubMed  CAS  Google Scholar 

  81. Ball J. Enthesopathy of rheumatoid and ankylosing spondylitis. Ann Rheum Dis. 1971;30:213–23.

    Article  PubMed  CAS  Google Scholar 

  82. Stevens JC, Cartlidge NE, Saunders M, et al. Atlanto-axial subluxation and cervical myelopathy in rheumatoid arthritis. QJM. 1971;40:391–408.

    PubMed  CAS  Google Scholar 

  83. Neva MH, Isomaki P, Hannonen P, et al. Early and extensive erosiveness in peripheral joints predicts atlantoaxial subluxations in patients with rheumatoid arthritis. Arthritis Rheum. 2003;48:1808–13.

    Article  PubMed  Google Scholar 

  84. Paimela L, Laasonen L, Kankaanpaa E, Leirisalo-Repo M. Progression of cervical spine changes in patients with early rheumatoid arthritis. J Rheumatol. 1997;24:1280–4.

    PubMed  CAS  Google Scholar 

  85. Winfield J, Young A, Williams P, Corbett M. Prospective study of the radiological changes in hands, feet, and cervical spine in adult rheumatoid disease. Ann Rheum Dis. 1983;42:613–8.

    Article  PubMed  CAS  Google Scholar 

  86. Neva MH, Kauppi MJ, Kautiainen H, et al. Combination drug therapy retards the development of rheumatoid atlantoaxial subluxations. Arthritis Rheum. 2000;43:2397–401.

    Article  PubMed  CAS  Google Scholar 

  87. Ramos-Remus C, Gomez-Vargas A, Guzman-Guzman JL, et al. Frequency of atlantoaxial subluxation and neurologic involvement in patients with ankylosing spondylitis. J Rheumatol. 1995;22:2120–5.

    PubMed  CAS  Google Scholar 

  88. Nowatzky J, Howard R, Pillinger MH, Krasnokutsky S. The role of uric acid and other crystals in osteoarthritis. Curr Rheumatol Rep. 2010;12:142–8.

    Article  PubMed  CAS  Google Scholar 

  89. Zhang W, Doherty M, Bardin T, et al. European League Against Rheumatism recommendations for calcium pyrophosphate deposition. Part I: terminology and diagnosis. Ann Rheum Dis. 2011;70:563–70.

    Article  PubMed  CAS  Google Scholar 

  90. Fye KH, Weinstein PR, Donald F. Compressive cervical myelopathy due to calcium pyrophosphate dihydrate deposition disease: report of a case and review of the literature. Arch Intern Med. 1999;159:189–93.

    Article  PubMed  CAS  Google Scholar 

  91. Lin SH, Hsieh ET, Wu TY, Chang CW. Cervical myelopathy induced by pseudogout in ligamentum flavum and retro-odontoid mass: a case report. Spinal Cord. 2006;44:692–4.

    Article  PubMed  Google Scholar 

  92. Salcman M, Khan A, Symonds DA. Calcium pyrophosphate arthropathy of the spine: case report and review of the literature. Neurosurgery. 1994;34:915–8. discussion 918.

    Article  PubMed  CAS  Google Scholar 

  93. Eross EJ, Dodick DW, Nelson KD, et al. Orthostatic headache syndrome with CSF leak secondary to bony pathology of the cervical spine. Cephalalgia. 2002;22:439–43.

    Article  PubMed  CAS  Google Scholar 

  94. Khung S, Budzik JF, Amzallag-Bellenger E, et al. Skeletal involvement in Langerhans cell histiocytosis. Insights Imaging. 2013;4:569–79.

    Article  PubMed  Google Scholar 

  95. Karmacharya P, Mainali NR, Aryal MR, Lloyd B. Recurrent case of ibuprofen-induced aseptic meningitis in mixed connective tissue disease. BMJ Case Rep. 2013;2013: doi:10.1136/bcr-2013-009571.

Download references

Acknowledgement

The authors thank Dr. H. Michael Belmont for his helpful remarks on the approach to headaches in SLE.

Compliance with Ethics Guidelines

Conflict of Interest

Noa Schwartz declares that she has no conflict of interest. Hal J. Mitnick declares that he has no conflict of interest. Johannes Nowatzky declares that he has no conflict of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Johannes Nowatzky.

Additional information

This article is part of the Topical Collection on Secondary Headache

Rights and permissions

Reprints and permissions

About this article

Cite this article

Schwartz, N., Mitnick, H.J. & Nowatzky, J. Headaches Related to Rheumatologic Disease. Curr Pain Headache Rep 17, 381 (2013). https://doi.org/10.1007/s11916-013-0381-4

Download citation

  • Published:

  • DOI: https://doi.org/10.1007/s11916-013-0381-4

Keywords

Navigation