Neuromyelitis optica spectrum disorders associated with systemic sclerosis: a case report and literature review

Bollo, Luca; Santoro, Carlo; Libro, Giuseppe; Pellicciari, Roberta; Paolicelli, Damiano; Iaffaldano, Pietro; Trojano, María

doi:10.1007/s10072-022-05984-2

Cited by 1 publication

(3 citation statements)

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“…Systemic sclerosis is rarely reported in association with NMO. According to the study by Bollo et al, till now, there has been only seven cases reported to be suffering from concomitant NMO and SSC in which all of them were treated by glucocorticoids [ 14 ]. The time of onset of SSC and NMO differed among cases as in the case reported by Hernandez et al NMO was developed years after the diagnosis of SSC and in the case studied by Moriguchi et al NMO was diagnosed one year before the symptoms of SSC.…”

Section: Discussionmentioning

confidence: 99%

“…Another interesting finding from the studies is that some cases did not respond well to the treatment with steroids and the patients were subsequently treated with other immunosuppressive agents such as cyclophosphamide [ 14 ]. This might highlight the importance of choosing proper treatment for patients with such complicated condition.…”

Section: Discussionmentioning

confidence: 99%

“…Although the condition satisfactorily responds to treatment with angiotensin converting enzyme inhibitors (ACEI), severe complications such as malignant hypertension, intracranial hemorrhage, and permanent renal impairment could still ensue [9]. In the case reported by Deeb et al [17], the patient developed systemic sclerosis renal crisis following the administration of glucocorticoid; however, other cases of which all were treated with high dose methylprednisolone did not develop renal crisis [12,14,[18][19][20][21]. Accordingly, in the present case, intracerebral and subarachnoid hemorrhage after administration of glucocorticoids was notable in her medical history.…”

Section: Discussionmentioning

confidence: 99%

See 2 more Smart Citations

Systemic Sclerosis, Reversible Cerebral Vasoconstriction Syndrome, and NeuroMyelitis Optica in a Patient

Etemadifar

Shafiei

Salari

et al. 2022

Case Reports in Immunology

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Systemic sclerosis (SSC) is an autoimmune disease of connective tissue and microvasculature mostly caused by autoantibodies. Likewise, neuromyelitis optica (NMO) is a demyelinating disease of the central nervous system correlating with autoantibodies against aquapourin-4. Reversible cerebral vasoconstriction syndrome (RCVS) is a disorder of brain vasculature resembling Raynaud phenomena in SSC. Despite co-occurrence is not rare in autoimmune disorders, the co-occurrence of NMO and SSC is extremely rare. In this case, we report a 35-year-old female presenting with paraplegia one day after discharge from hospital following surgical carnioplasty. She had a history of scleroderma and optic neuritis for which she was treated with high dose glucocorticoids causing renal crisis and RCVS causing intracranial and intracerebral hemorrhage which required a craniotomy to be performed in February 2020. In her recent admission, magnetic resonance imaging of the spinal cord indicated longitudinally extensive transverse myelitis (LETM) and blood tests revealed a highly positive titer of NMO-IgG. Daily plasmapheresis resulted in satisfactory improvement in her condition. This case highlights the importance of evaluating neurologic manifestations in systemic sclerosis patients considering the NMO and RCVS occurrence. Additionally, in concomitant cases, the treatment strategy should be modified regarding the risk of scleroderma renal crisis.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%