scholarly journals EP30 Sensory ganglionopathy as a possible presenting feature of Sjögren’s syndrome

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Preeya Ummur ◽  
Kirsty Levasseur ◽  
Yee Suh Tee ◽  
Benjamin Fisher
Keyword(s):  
Case Report ◽  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Neurological Symptoms ◽  
Lower Limbs ◽  
Sensory Axons ◽  
Sensory Ganglionopathy ◽  
Sjögren‘S Syndrome

Abstract Case report - Introduction Primary Sjögren’s syndrome is a systemic autoimmune disease affecting the exocrine glands, commonly resulting in dryness of mouth and eyes. It can also rarely present with neurological symptoms most commonly peripheral neuropathies. The case highlights a rare case of sensory and motor neuropathy with some features suggestive of Sjögren’s syndrome as an underlying diagnosis. Case report - Case description A 54-year-old Caucasian lady was transferred from a local hospital for specialist neurology care. She described widespread paraesthesia over two months progressing to lower limb weakness worsening over a two-day period. Her symptoms further progressed to involve her arms and new blurred vision. On further questioning she reported previous severe mouth ulcers, possible genital ulcers and painful jaw swelling with long standing history of dry mouth. On thorough neurological examination, the positive findings were mild ptosis of the right eye, paraesthesia on palpation of face, upper limbs had a flaccid tone with reduced power and pseudoathetosis of outstretched arms, reduced proprioception, and absent reflexes. Lower limbs were more affected with worse power and absent proprioception. Schirmer’s test showed normal tear production but saliva production was reduced. MRI brain and spine were reported as pathological enhancement in both optic nerves, no obvious brain parenchymal abnormality, no obvious spinal cord abnormality. Lumbar puncture CSF pro 0.38, white cells 12 (poly 50%, mono 50%), red cells 3700, no organisms on gram stain, opening pressure 21.5 cmH2O. Sural nerve biopsy demonstrated profound loss of sensory axons, severe loss of large myelinated fibres in all fascicles with motor nerve involvement. Muscle biopsy demonstrated chronic neurogenic atrophy. Rheumatology screen showed positive anti Ro 52 antibodies (on an extended myositis screen). EMG demonstrated neurophysiological evidence of widespread marked loss of sensory axons in the upper and lower limbs and partial loss of motor axons in several lower limb territories. USS parotid glands showed some possibly mildly abnormal areas, minor salivary gland biopsy was normal. She received methylprednisolone, IV immunoglobulin and two courses of plasma exchange. Given some improvement following this treatment, further immunosuppression was felt to be appropriate. Initially Mycofenolate mofetil was started and Rituximab is now being considered. Case report - Discussion This case demonstrates a mixed neuropathy picture. Following thorough investigations, many differential diagnoses of mixed neuropathy were considered and excluded (e.g. Guillain Barre, Syphilis, MS, HIV, Hepatitis). Neurological symptoms can develop before the onset of sicca symptoms in Sjögren’s syndrome. With a positive Anti Ro52, xerostomia and clinical improvement with methylprednisolone and plasmaphereses it seems likely there is an underlying autoimmune diagnosis in this case and despite a normal biopsy she would meet the 2016 ACR/EULAR classification criteria for Sjögren’s syndrome. Case report - Key learning points Sjögren’s syndrome should be considered as a potential underlying cause in patients with a sensory ganglionopathy. Neurological symptoms can develop before the onset of sicca symptoms in Sjögren’s syndrome.

scholarly journals Multiple dermatofibromas in a patient with lupus erythematosus and Sjögren’s syndrome: a new case report

2019 ◽  
Vol 3 (1) ◽  
pp. 13-14
Author(s):  
Mrabat Samia
Keyword(s):  
Case Report ◽  
Sjögren’S Syndrome ◽  
Lupus Erythematosus ◽  
Sjögren's Syndrome ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Lower Limbs ◽  
Skin Tumor ◽  
Solitary Lesion ◽  
Sjögren‘S Syndrome

Dermatofibroma (DF) is a common benign skin tumor. It is often a solitary lesion occurring on the lower limbs. Multiple DFs is rare. This is associated with auto-immune conditions and immunosuppression. We report a new case of multiple dermatofibromas in a patient with lupus erythematosus and Sjögren’s syndrome.

scholarly journals EP27 Sjögren’s or not: an anxious diagnosis

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Sharmin Nizam
Keyword(s):  
Case Report ◽  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Labial Gland ◽  
Schirmer’S Test ◽  
Sicca Symptoms ◽  
Sjögren‘S Syndrome

Abstract Case report - Introduction Sjögren’s syndrome is a chronic, autoimmune condition usually characterised by reduced function of exocrine glands (mainly lacrimal and salivary) resulting in sicca symptoms. Affected patients may also have extra-glandular features including arthritis, neuropathy, and interstitial nephritis. This is a case of possible Sjögren’s syndrome without classical features like positive serology or histology. This makes the patient feel anxious about his overall health. Diagnostic criteria have been debated over the years and whilst some clinical features may be suggestive, more objective evidence can help guide discussions on long term management and prognosis to allay anxiety. Case report - Case description A 63-year-old Asian gentleman has had 6 years of intermittent cervical lymphadenopathy, dry eye and mouth symptoms without weight loss or respiratory complaints. His background includes ulcerative colitis (relatively stable), angina, hypertension, degenerative back pain (confirmed on MRI), dental extraction and diabetes. Interval FNA sampling and excision biopsy of a prominent chain of right cervical nodes on separate occasions showed “reactive changes” with negative Mycobacterium TB screening (serology and lymph nodes). Blood tests show a normal CRP (<5 mg/L), ESR 36 mm/h, raised polyclonal IgG 28.6 g/L (IgG subclass 1, 20.40 g/L, subclass 2, 9.36g/L, subclass 3, 0.954g/L, subclass 4, 9.430g/L) , normal complement and negative results for ANA, HLA B27, Anti CCP and ANCA. Bilateral submandibular gland ultrasound showed hyperechoic lesions consistent with either chronic sialadenitis or Sjögren’s. FNA sampling of an intra-parotid lesion showed a “reactive” lymph node. A left lower lobe 5mm calcified granuloma seen on plain film was confirmed on CT chest imaging along with mild inflammatory changes (lingual area) and multiple soft tissue density nodules up to 1cm in the anterior mediastinum. Initially thought thymoma related, later it was agreed these were benign lymph nodes after noting bilateral, sub-centimetre axillary and pre-tracheal nodes of similar appearance. Following annual surveillance, a recent scan shows persistence of the lingular nodular focus, mediastinal lymphadenopathy and a 4mm ground glass nodule not thought suitable for PET CT or CT guided sampling. The previously seen parotid lymph node appears reduced and scattered low grade nodes are seen in the neck, chest, and porta hepatis. Ophthalmologists note a poor-quality tear film with an equivocal Schirmer’s test. He has been treated for blepharitis and diagnosed with macular oedema. He was due to have a labial gland (lip) biopsy but later declined the procedure. Case report - Discussion Sjögren’s syndrome has a female preponderance and is usually associated with sicca symptoms, a positive Schirmer’s test and autoantibodies (anti-Ro and anti-La). Extra-glandular features may exist, and secondary Sjögren’s features are seen in other autoimmune conditions. Various diagnostic criteria have been proposed using clinical, serological, and/or histological features. This patient has sicca symptoms, lymphadenopathy, and imaging findings suggestive of Sjögren’s. Though not routinely used, salivary gland imaging features include enlarged, hyperechoic lesions and later stage multi-cystic or reticular patterns within atrophic glands. Due to ethnicity, negative autoantibodies and imaging, the differential of tuberculosis (TB) was excluded. A labial gland biopsy was suggested as it may be a potentially sensitive and specific Sjögren’s biomarker. Presence of multiple, periductal, lymphocytic foci can help exclude alternative diagnoses like sarcoidosis, amyloidosis, or lymphoma. However, the patient declined the procedure due to concerns about possible post procedure hypersensitivity. This patient has mild fatigue and non-specific arthralgia but not typical of fibromyalgia which is known to mimic Sjögren’s. Reassuringly, he remains well but anxious about lymphadenopathy which he feels is unrelated to his mild ulcerative colitis managed with prednisolone enemas. In the absence of arthritis or significant organ involvement, he has only been given symptomatic treatment (e.g. eye drops). In Sjogren’s, any increased or persistent lymphadenopathy calls for further investigation. Other predictors include low complement and cryoglobulins which are absent in this patient. This case may add to the evidence of co-existence of secondary Sjögren’s or Sjogren’s like syndrome with IBD which seems uncommon and in other cases, appears to be in conjunction with immunosuppressive treatment and autoantibodies. Duration of follow up required remains uncertain and whilst the patient requires little ongoing monitoring, health anxieties can precipitate frequent contact. Case report - Key learning points  Sjögren’s syndrome (SS) can be variable in presentation but in most cases is mildUnlike other autoimmune disorders, in SS there is a lack of standardised criteria for diagnosis and classificationSome features can be non- specific and like features of fibromyalgia and sarcoidosisIn unclear cases, like this, objective markers like serology or histology (labial gland biopsy) may be more helpfulIn lymphadenopathy, depending on size and appearance, further investigations require multidisciplinary discussion to check if regular imaging is more appropriate compared to invasive tests. The frequency of imaging and potential radiation exposure needs careful consideration.In this case the patient is unwilling to undergo further invasive tests like a biopsy and the lymphadenopathy seen on imaging is thought relatively stable and not amendable to sampling.The ideal duration of follow up and need for ongoing investigations in this patient remains unclear – advice on monitoring and outcome of similar cases may help guide patient management and reduce anxiety

Coexistence of Sjögren's syndrome and bilateral ranulas: A case report and review of the literature

2021 ◽  
Author(s):  
Yoshiro Koma ◽  
Takehiro Fujimoto ◽  
Kiyoshi Sakai ◽  
Yukiko Sugimura ◽  
Satoshi Yamaguchi ◽  
...  
Keyword(s):  
Case Report ◽  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Review Of The Literature ◽  
Sjögren‘S Syndrome

scholarly journals Intravenous Immunoglobulin and Mycophenolate Mofetil for Long-Standing Sensory Neuronopathy in Sjögren's Syndrome

2012 ◽  
Vol 2012 ◽  
pp. 1-3 ◽  
Author(s):  
Maria Giovanna Danieli ◽  
Lucia Pettinari ◽  
Ramona Morariu ◽  
Fernando Monteforte ◽  
Francesco Logullo
Keyword(s):  
Mycophenolate Mofetil ◽  
Intravenous Immunoglobulin ◽  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Lower Limbs ◽  
High Dose ◽  
Sensory Neuronopathy ◽  
Sjögren‘S Syndrome

Sensory neuronopathy is described in association with the Sjögren's syndrome (SS). We studied a 55-year-old woman with a 4-year history of progressive asymmetric numbness, distal tingling, and burning sensation in upper and lower limbs. In a few months, she developed ataxia with increased hypoanaesthesia. Electrodiagnostic tests revealed undetectable distal and proximal sensory nerve action potential in upper and lower limbs. Cervical spine magnetic resonance showed a signal hyperintensity of posterior columns. Previous treatment with high-dose glucocorticoids and azathioprine was ineffective. A combined treatment with intravenous immunoglobulin and mycophenolate mofetil was followed by a progressive and persistent improvement. This case documented the efficacy and the safety of the coadministration of intravenous immunoglobulin and mycophenolate mofetil in sensory neuronopathy associated with SS refractory to conventional immunosuppressive therapy.

scholarly journals Sjögren’s syndrome with rapidly progressive motor neuron disease: a case report

2020 ◽  
Vol 48 (11) ◽  
pp. 030006052097446 ◽  
Author(s):  
Huijia Yang ◽  
Xiaozhong Jing ◽  
Jinhua Yan ◽  
Dihui Ma
Keyword(s):  
Motor Neuron ◽  
Motor Neuron Disease ◽  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Lower Limbs ◽  
Weekly Dose ◽  
Limb Weakness ◽  
Sjögren‘S Syndrome

Sjögren’s syndrome is an autoimmune disease that can affect multiple systems. Sjögren’s syndrome with motor neuron disease is rarely reported. Herein, we describe a patient with rapidly progressive motor neuron disease secondary to Sjögren’s syndrome. A 42-year-old woman was admitted to our hospital with a 2-month history of progressive limb weakness. Neurological assessment revealed fasciculation in the lower limbs and amyotrophy in the bilateral supraspinatus, interosseous, and thenar muscles. Serological examination and labial gland biopsy revealed Sjögren’s syndrome. In addition, electromyography demonstrated neurogenic damage to the upper and lower limbs. The patient received a short course of high-dose corticosteroids, intravenous immunoglobulins, and immunosuppressant treatment, including a weekly dose of 0.4 g cyclophosphamide and a daily dose of 0.2 g hydroxychloroquine. However, the patient’s limb weakness was further aggravated and her respiratory function was compromised. Electromyography re-examination demonstrated extensive neurogenic damage, and she was diagnosed with Sjögren’s syndrome with motor neuron disease. The patient died of respiratory failure after 2 months. We suggest that more effective maintenance treatments should be sought. Further investigation is required to elucidate the association between autoimmune motor neuron disease and Sjögren’s syndrome.

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