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 Table of Contents  
Year : 2016  |  Volume : 11  |  Issue : 4  |  Page : 222-225

Compressive myelopathy: A rare clinical presentation of IgG4-related disease

1 Institute of Rheumatology, Madras Medical College, Chennai; Department of Rheumatology, Saveetha Medical College Hospital, Kanchipuram, Tamil Nadu, India
2 Institute of Rheumatology, Madras Medical College, Chennai, Tamil Nadu, India

Date of Web Publication8-Nov-2016

Correspondence Address:
Dr. Kavitha Mohanasundaram
6, First Street, Shanthi Nagar, Adambakkam, Chennai - 600 088, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0973-3698.193587

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IgG4-related disease (IgG4RD) is a new entry into the field of rheumatology characterized by tumefactive lesions, storiform fibrosis, and involvement of more than one organ. The most common organ involved is the pancreas which often coexist with mass lesions in other organs. We report a case where IgG4RD presented with the involvement of aorta and meninges in the form of periaortitis and hypertrophic pachymeningitis respectively, a rare coexistence in IgG4RD. The patient responded well to rituximab in the initial visit, however during subsequent recurrence, the response was not significant. We also discuss the immunosuppressants used in IgG4 RD and the variability in clinical response during various stages of disease.

Keywords: Hypertrophic pachymeningitis, IgG4-related disease, periaortitis, rituximab

How to cite this article:
Mohanasundaram K, Mahendran B, Mani M, Saravanan M, Rajeswari S. Compressive myelopathy: A rare clinical presentation of IgG4-related disease. Indian J Rheumatol 2016;11:222-5

How to cite this URL:
Mohanasundaram K, Mahendran B, Mani M, Saravanan M, Rajeswari S. Compressive myelopathy: A rare clinical presentation of IgG4-related disease. Indian J Rheumatol [serial online] 2016 [cited 2022 Oct 2];11:222-5. Available from:

  Introduction Top

IgG4-related disease (IgG4RD) is one of the new entities of systemic fibrosing disorders, which can cause a wide array of clinical presentations depending on the site of fibrosis.[1] This disease has a tendency to involve almost all organs of the body often presenting as tumefactive lesions. Its involvement of central nervous system, though has been reported early, is very rare. An early clinical diagnosis and treatment result in good recovery. In the setting of extensive fibrosis, the response to treatment may be variable. We present a case of IgG4RD, which presented with paraparesis due to hypertrophic pachymeningitis. This is an emerging disease, and case reports of such diseases will help in studying the varied presentations of the same.

  Case Report Top

A 34-year-old female presented with slowly progressive paraperesis with motor, sensory, and autonomic disturbances with the level at D4 clinically, in the year of 2013. She was treated by a neurologist; magnetic resonance imaging (MRI) of the whole spine was done which revealed an epidural soft tissue mass causing spinal cord compression at multiple thoracic levels with maximum compression at D3–D4 level suggestive of pachymeningitis [Figure 1]. The lesion extended from D1 to D12 levels with eccentric diffuse circumferential enhancing aortic, adventitial, and periadventitial soft tissue mass without any aortic luminal compromise or dissection - suggestive of periaortitis. Differential diagnosis entertained was IgG4RD, tuberculosis, and sarcoidosis. She was referred to a rheumatologist with the above reports.
Figure 1: Magnetic resonance imaging - T1-weighted fat saturated, postcontrast imaging - periaortic cuffing with enhancing epidural lesion at D1–D12 levels, with maximal compression at D4 level

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Tuberculosis was ruled out since the patient had a normal cerebrospinal fluid cell count and polymerase chain reaction (PCR) for Mycobacterium tuberculosis was negative. Sarcoidosis presenting with hypertrophic pachymeningitis has been previously reported.[2] However, coexisting periaortic cuffing was rare, no skin lesions, arthritis, or hilar adenopathy was noted in the patient. Preliminary investigations like serum Vitamin D3 levels and angiotensin converting enzyme levels were normal. The patient was not willing for a biopsy at that point of time; hence, a positron emission tomography (PET) of the whole body was done; increased uptake at the above mentioned thoracic level with diffuse circumferential mural thickening and periaortic soft tissue thickening around ascending aorta, arch, descending aorta, and bilateral carotids was observed [Figure 2]. Such a PET imaging picture was also not consistent with sarcoidosis.
Figure 2: Positron emission tomography scan demonstrates increased uptake in the periaortic region involving the ascending, arch and descending thoracic aorta

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Following PET imaging a differential diagnosis of suspected large vessel vasculitis (LVV) was also entertained. Acute phase reactants were persistently normal, no pulse or blood pressure discrepancy was noted, and the presence of compressive myelopathy was not consistent with LVV. With two different organ involvement mass like presentation and periaortitis without origin or luminal occlusion, a diagnosis of IgG4RD was arrived at, even though her serum IgG4 levels were normal. She was treated with intravenous methyl prednisolone 1 g for 3 days, followed by oral steroids and 500 mg of Rituximab on day 0 and 14. The patient showed remarkable improvement, was able to lead a normal life as before and was lost to follow-up.

In mid of 2015, patient started developing paraperesis; it was progressively increasing in severity. MRI was done, and it showed similar findings as listed before with soft tissue dural lesion causing compression at multiple levels extending on to the neural foramen bilaterally. Cerebrospinal fluid was acellular with normal biochemical parameters; PCR done for M. tuberculosis was negative. Periaortic soft tissue mass was persistent [Figure 1]. A diagnosis of hypertrophic pachymeningitis resulting in cord compression was made. She was taken for a D3 D4 laminectomy and biopsy of the lesion was taken simultaneously. Biopsy showed dense hyalinized connective tissue with diffuse lymphocytic and macrophage infiltrate. One bit showed many scattered lymphoplasmacytic cells. No caseous necrosis or epithelioid granuloma was noted. No evidence of malignancy and special stains for acid-fast bacilli was negative. Serum IgG4 level was normal (0.47 [0.03–2 g/L]). The patient was treated with steroids and rituximab this visit; however, she failed to show a significant response.

  Discussion Top

IgG4RD is a recent inclusion to the spectrum of rheumatological diseases. It often presents with mass lesions in one or more organs and diagnosed by histopathology. The three salient features are infiltration with lymphoplasmacytic cells, storiform fibrosis, and obliterative phlebitis. Idiopathic hypertrophic pachymeningitis is now grouped under IgG4RD; it often presents with compressive myelopathy like symptoms.[3] It can cause cord compression at cervical, thoracic or lumbar levels, cervical being the most common.

Previous case reports of IgG4RD causing cord compression are few, and the major review on hypertrophic pachymeningitis published by Lu et al. (n = 33) had only 15% of people with motor weakness and the rest commonly with intracranial pachymeningitis.[4],[5] However, in our patient, spinal hypertrophic pachymeningitis coexist with periaortitis which to the best of our knowledge is very rare. A systematic review of head and neck involvement of IgG4RD published in 2015 does not have a single work from India.[6] The previous work published on hypertrophic pachymeningitis have the following organ involvement - lung kidney, bone, retroperitoneal fibrosis, and salivary glands.[5] [Table 1] presents the previous case reports of compressive myelopathy in IgG4RD; we have excluded intracranial hypertrophic pachymeningitis from the same. Large vessel involvement in IgG4RD reported previously by Perugino et al. had reported cases of aortitis and periaortitis being associated with retroperitoneal fibrosis.[14] Chronic periaortitis had been recognized as a common manifestation of IgG4RD, and it commonly affects abdominal aorta; it often coexists with autoimmune pancreatitis, another prototype manifestation of this disease.[15] In the diagnostic workup of any noninfectious aortitis, IgG4RD as a differential diagnosis has to be entertained.[16]
Table 1: Previous works on compressive myelopathy in immunoglobulin G4-related disease

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Another differential diagnosis entertained in our patient was tuberculosis and neurosarcoidosis; however, histopathology from the mass revealed no granulomas. The co-occurrence of periaortitis encasing the aorta in this patient makes the diagnosis of IgG4RD even more likely.

The treatment protocols of IgG4RD are not well defined; steroids are the drugs of choice followed by steroid-sparing immunosuppressants such as azathioprine, mycophenolate mofetil, and rituximab being tried for the same.[17] Rituximab had been tried in resistant IgG4RD previously and was found effective.[18] Serum IgG4 levels were found to reduce with rituximab suggesting B-cell depletion as a potential target for treatment of IgG4RD.

  Conclusion Top

In our country IgG4 immunohistochemistry in tissue biopsy is not routinely available even in the best of setups, establishing a tissue diagnosis becomes difficult. Combined with normal IgG4 serum levels, the process becomes tougher. In our patient, the presence of two diagnoses (idiopathic hypertrophic pachymeningitis and periaortitis) which are consistently grouped under IgG4RD along with the rapid and dramatic response to steroids and immunosuppression during the first visit makes it the most likely diagnosis. However, the progressive course of disease and extensive fibrosis resulted in a lack of response. This presentation describes a rare co-occurrence of hypertrophic pachymeningitis with periaortitis in IGG4RD and also underscores the need for maintenance steroids, immunosuppression, and regular follow-up in IgG4RD.


We acknowledge the help rendered by Dr. Srilekha Sairam, Consultant Rheumatologist, Apollo First Med Hospitals in providing the PET images of the patient

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Masaki Y, Dong L, Kurose N, Kitagawa K, Morikawa Y, Yamamoto M, et al. Proposal for a new clinical entity, IgG4-positive multiorgan lymphoproliferative syndrome: Analysis of 64 cases of IgG4-related disorders. Ann Rheum Dis 2009;68:1310-5.  Back to cited text no. 1
Subrati N, Vaqas B, Peterson D, Patel MC. Hypertrophic pachymeningitis with sarcoidosis: A rare cause of craniocervical compression. BMJ Case Rep 2015;2015. pii: Bcr2014208604.  Back to cited text no. 2
Stone JH, Khosroshahi A, Deshpande V, Chan JK, Heathcote JG, Aalberse R, et al. Recommendations for the nomenclature of IgG4-related disease and its individual organ system manifestations. Arthritis Rheum 2012;64:3061-7.  Back to cited text no. 3
Radotra BD, Aggarwal A, Kapoor A, Singla N, Chatterjee D. An orphan disease: IgG4-related spinal pachymeningitis: Report of 2 cases. J Neurosurg Spine 2016:1-5.  Back to cited text no. 4
Lu LX, Della-Torre E, Stone JH, Clark SW. IgG4-related hypertrophic pachymeningitis: Clinical features, diagnostic criteria, and treatment. JAMA Neurol 2014;71:785-93.  Back to cited text no. 5
Mulholland GB, Jeffery CC, Satija P, Côté DW. Immunoglobulin G4-related diseases in the head and neck: A systematic review. J Otolaryngol Head Neck Surg 2015;44:24.  Back to cited text no. 6
Kim SH, Kang Y, Oh SH, Paik S, Kim JS. Paraplegia in a patient with IgG4-related sclerosing disease: A Case Report. Ann Rehabil Med 2014;38:856-60.  Back to cited text no. 7
Wallace ZS, Carruthers MN, Khosroshahi A, Carruthers R, Shinagare S, Stemmer-Rachamimov A, et al. IgG4-related disease and hypertrophic pachymeningitis. Medicine (Baltimore) 2013;92:206-16.  Back to cited text no. 8
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.  Back to cited text no. 9
Choi SH, Lee SH, Khang SK, Jeon SR. IgG4-related sclerosing pachymeningitis causing spinal cord compression. Neurology 2010;75:1388-90.  Back to cited text no. 10
Lindstrom KM, Cousar JB, Lopes MB. IgG4-related meningeal disease: Clinico-pathological features and proposal for diagnostic criteria. Acta Neuropathol 2010;120:765-76.  Back to cited text no. 11
Tajima Y, Mito Y. Cranial neuropathy because of IgG4-related pachymeningitis; intracranial and spinal mass lesions. BMJ Case Rep 2012;2012. pii: Bcr2012006471.  Back to cited text no. 12
Della-Torre E, Passerini G, Furlan R, Roveri L, Chieffo R, Anzalone N, et al. Cerebrospinal fluid analysis in immunoglobulin G4-related hypertrophic pachymeningitis. J Rheumatol 2013;40:1927-9.  Back to cited text no. 13
Perugino CA, Wallace ZS, Meyersohn N, Oliveira G, Stone JR, Stone JH. Large vessel involvement by IgG4-related disease. Medicine (Baltimore) 2016;95:e3344.  Back to cited text no. 14
Stone JH, Khosroshahi A, Hilgenberg A, Spooner A, Isselbacher EM, Stone JR. IgG4-related systemic disease and lymphoplasmacytic aortitis. Arthritis Rheum 2009;60:3139-45.  Back to cited text no. 15
Stone JH, Khosroshahi A, Deshpande V, Stone JR. IgG4-related systemic disease accounts for a significant proportion of thoracic lymphoplasmacytic aortitis cases. Arthritis Care Res (Hoboken) 2010;62:316-22.  Back to cited text no. 16
Khosroshahi A, Stone JH. Treatment approaches to IgG4-related systemic disease. Curr Opin Rheumatol 2011;23:67-71.  Back to cited text no. 17
Khosroshahi A, Bloch DB, Deshpande V, Stone JH. Rituximab therapy leads to rapid decline of serum IgG4 levels and prompt clinical improvement in IgG4-related systemic disease. Arthritis Rheum 2010;62:1755-62.  Back to cited text no. 18


  [Figure 1], [Figure 2]

  [Table 1]

This article has been cited by
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