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| LETTER TO EDITOR |
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| Ahead of print publication |
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Systemic lupus erythematosus with extensive brain calcification
Sham Santhanam
Department of Rheumatology, Consultant Rheumatologist, Gleneagles Global Health City, Chennai, Tamil Nadu, India
| Date of Submission | 17-May-2021 |
| Date of Acceptance | 15-Jun-2021 |
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Correspondence Address:
Sham Santhanam,
Gleneagles Global Health City, Chennai - 600 100, Tamil Nadu
India
 Source of Support: None, Conflict of Interest: None DOI: 10.4103/injr.injr_95_21
Dear Editor,
We present a 52-year-old female, diagnosed with systemic lupus erythematosus (SLE) for the past 10 years. At the time of diagnosis, she presented with fever, alopecia, skin rashes, oral ulcers, joint pains, and serositis. On evaluation, she tested positive for antinuclear antibodies, anti-ds DNA, anti-SSA, and anti-nucleosome antibodies and had low complement (C3 and C4) levels. With treatment, she improved and had been doing well. Currently, she is on low-dose steroids (5 mg/day of prednisolone), hydroxychloroquine (200 mg/day), and mycophenolate mofetil (1 g/day). Since the past 3 years, she had progressive extrapyramidal symptoms (dystonia and dysarthria) of the trunk and upper and lower limbs. She had severe postural imbalance and was not able to walk without support. The clinical features were suggestive of atypical Parkinsonism More Details with no features of Parkinson plus syndrome. All her baseline investigations (hemogram, erythrocyte sedimentation rate [ESR], renal function, liver function, thyroid profile, and serum electrolytes) including cerebrospinal fluid analysis were normal, and she tested negative for antiphospholipid antibodies. Magnetic resonance imaging of brain [Figure 1] was done which revealed extensive calcification of bilateral basal ganglia, periventricular white matter, centrum semiovale, pons, thalami, white matter of cerebellum, and gyriform calcification with diffuse cortical atrophy. The metabolic workup (normal serum parathormone, serum calcium, phosphorus, ferritin, and lactate levels) was normal except for Vitamin D (25-OH Vitamin D levels – 20 ng/ml) insufficiency. We made a diagnosis of SLE associated with Fahr's disease (Idiopathic Basal Ganglia Calcification [IBGC]) after ruling out all the possible secondary causes. We had a therapeutic dilemma on whether to step-up immunosuppression (rituximab infusion) or to give a trial of levodopa. We did not step-up immunosuppression as the patient had no other clinical features of SLE with normal ESR and complement levels. We discussed with the patient regarding this and started her on levodopa. Although it was informed to the patient that rituximab might be considered if clinical symptoms do not improve or if there is any deterioration. The patient improved symptomatically with levodopa supplementation with respect to her speech, movements, and balance and has been doing well for the past 1 year. | Figure 1: Magnetic resonance imaging brain showing dense calcification in basal ganglia (a) and cortex (b)
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IBGC or Fahr's disease is a rare neurodegenerative disorder named after “Theodore Fahr” in patients with calcification in basal ganglia and other parts of brain. It often presents in the middle age.[1] It can be either a primary form (Fahr's disease – genetic or sporadic) or a secondary form (Fahr's syndrome) associated with various endocrine syndromes, toxins, and infections.[2] We have discussed the differential diagnosis[1],[3] for brain calcification in [Table 1]. Patients with Fahr's disease/syndrome may be asymptomatic or may present with psychiatric manifestations, seizures, extrapyramidal symptoms, or other neurological deficits.[1],[4] Our patient had no similar family history, and all the possible secondary causes were excluded.
Neuropsychiatric lupus (NPL) is one of the important causes for mortality and morbidity in SLE patients. Extrapyramidal symptoms can be seen in NPL. The underlying pathogenesis is either due to immune-mediated small-vessel vasculitis or vasculopathy secondary to antiphospholipid antibodies.[4],[5] Cerebral calcifications have been rarely reported in SLE, except for few case reports.[4],[5],[6],[7] Most of these reports had asymptomatic patients with cerebral calcification being detected incidentally, though few of them had extrapyramidal symptoms.[8] The pattern of calcification in our patient was diffuse, extensive, coarse, and conglomerate suggestive of a pathological calcification unlike the small, faint physiological calcifications localized to globus pallidus.[1] The exact reason for cerebral calcification in SLE patients is not known though vascular mechanisms have been proposed. Our patient did not have evidence of the previous vascular injury except for diffuse cortical atrophy.
We report this case considering the rarity of symptomatic Fahr's disease in a patient with SLE and in view of the therapeutic dilemma.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | |  |
| 1. | Donzuso G, Mostile G, Nicoletti A, Zappia M. Basal ganglia calcifications (Fahr's syndrome): Related conditions and clinical features. Neurol Sci 2019;40:2251-63.  |
| 2. | Perugula ML, Lippmann S. Fahr's Disease or Fahr's Syndrome? Innov Clin Neurosci 2016;13:45-6. |
| 3. | Saade C, Najem E, Asmar K, Salman R, El Achkar B, Naffaa L. Intracranial calcifications on CT: An updated review. J Radiol Case Rep 2019;13:1-8. |
| 4. | Chang RS, Leung CY, Leong HS. Bilateral Striatopallidodentate Calcinosis associated with Systemic Lupus Erythematosus: Case report and review of literature. J Neurol Sci 2015;358:518-9. |
| 5. | Garcia Raya P, Gil Aguado A, Merlo MJ, Lavilla Uriol P, Astudillo AV, Garcia Puig J. Massive cerebral calcification in systemic lupus erythematosus: An report of an unusual case. Lupus 1994;3:133-5. |
| 6. | Filloux V, Marotte H, Miossec P. Cerebral calcifications in an elderly lupus patient. Ann Rheum Dis 2003;62:283-4. |
| 7. | Raymond A, Zariah A, Samad S, Chin C, Kong N. Brain calcification in patients with cerebral lupus. Lupus 1996;5:123-8. |
| 8. | Miyoshi Y, Atsumi T, Kitagawa H, Ogura N, Amasaki Y, Tsutsumi A, et al. Parkinson-like symptoms as a manifestation of systemic lupus erythematosus. Lupus 1993;2:199-201. |
[Figure 1]
[Table 1]
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