O10 A case of sarcoidosis mimicking Sjögren’s syndrome along with abnormal nailfold capillaroscopy

Abstract Case report - Introduction Sicca symptoms and Raynaud’s disease are amongst the common presentation of Sjögren’s syndrome (SS) which has an estimated prevalence of 1%. We are reporting a case that was initially considered as SS, but subsequent lip biopsy confirmed histological evidence of sarcoidosis in the presence of abnormal nailfold capillaroscopy and negative autoimmune profile. Sarcoidosis can be associated with various autoimmune diseases including systemic sclerosis, Hashimoto's thyroiditis, systemic lupus erythematosus, rheumatoid arthritis, Sjögren’s syndrome and spondyloarthropathy. Case report - Case description A 29-year-old Caucasian man was referred to rheumatology clinic, suffering with ongoing sicca symptoms including dry itchy gritty eyes, sore red eyelids, persistent mouth dryness and cracking ulcers. He has history of Raynaud’s disease, dysphagia for solid, dry cough and photosensitive rash on his chest and arms. His other symptoms were polyarthralgia, myalgia, headache, persistent fatigue, poor memory, dry skin over the hands and tingling sensation at the feet. There was no history of joint swelling, uveitis, chest pain or shortness of breath. He has hypothyroidism secondary to radioiodine treatment and is currently on levothyroxine. He works in a factory, drinks alcohol occasionally and is non-smoker. There was no family history of autoimmune conditions. General and systemic examination was unremarkable, and there were no stigmata of connective tissue disease except for tenderness and crepitus at the right knee joint. His saliva flow rate was 0.4ml/5 mins and Schirmer’s test was 20mm/5 mins (both were normal values). Autoimmune profile was negative; ANA: 0.2 units, RF factor: <10, CCP antibodies (IgG): 1.1U/mL. Apart from mild lymphopenia, the other laboratory tests were at normal range including CK, CRP, adjusted calcium, ACE, immunoglobulins, serum protein electrophoresis and urine test for protein. Chest X-ray and lung function test were both normal. Nailfold capillaroscopy shows grade1 enlarged capillaries, prominent capillary ramifications in the form of bushy capillaries and some microhaemorrhages, a pattern suggestive of mixed connective tissue disease. Lip biopsy showed small non-caseating epithelioid granuloma within the parenchyma. The initial working diagnosis was a possible serology-negative SS, but the later results of lip biopsy confirmed sarcoidosis. He was commenced on Hydroxychloroquine and advised about cognitive-behavioural therapy for generalised ache and fatigue. As he was not responding to pilocarpine, a referral for ophthalmology review was requested. Case report - Discussion Sarcoidosis is a multisystem disease of unknown aetiology, known to exist worldwide with a variable prevalence of around 20 per 100,000 in the UK. It is characterized by the presence of multiple non-caseating granulomas, inflammation which may occur in any tissue in the body manifested as local symptoms with or without systemic features. Involvement of the salivary and lacrimal glands can result in xerostomia and xerophthalmia, respectively. Chronic sarcoid has an often subtle, insidious, progressive, and highly variable clinical course; it can be asymptomatic in a significant percentage of the cases. In addition to the clinical and radiological findings, the diagnosis of sarcoidosis should be based on the histological proof of non-caseating granulomas and ruling out other diseases that shares similar features. SS has many features in common with sarcoidosis; these may include sicca symptoms, arthralgia, myalgia, arthritis, erythematous rash, lymphadenopathy, peripheral neuropathy, fatigue and raised inflammatory markers. Moreover, positive rheumatoid factor, defective T suppressor cell regulation and HLA-DR3 are linked to both diseases. Pulmonary involvement in either of them may have similar clinical and radiographic manifestations, making it difficult for the clinician to distinguish between these diseases. In terms of management, hydroxychloroquine may help the skin and joint manifestations in both diseases whereas more severe diseases may require treatment with glucocorticoids, methotrexate, and azathioprine. Nevertheless, prompt confirmation of the diagnosis is crucial provided the difference in response to medication, complications, outcome, and prognosis. Based on the clinical and lip biopsy findings, the above case was diagnosed with sarcoidosis. The case did not meet the criteria for the diagnosis of SS provided the negative results of Schirmer’s test, saliva flow test and autoimmune profile. Case report - Key learning points Raynaud’s phenomenon (secondary Raynaud’s) with positive capillaroscopy findings was an unusual early presenting feature of sarcoidosis in the above case. There was one case report of similar presentation published in 2011. However, literature did not reveal specific information about abnormal nailfold capillaroscopy results in sarcoidosis. Dry eyes and dry mouth may occur in sarcoidosis mimicking the presentation of primary SS. In addition, true coexistence of sarcoidosis and SS has also been defined based on the histopathologic examination of the exocrine glands. Diagnostic criteria need to be applied for patients with suspected overlap of the two diseases. Clinicians will need to be vigilant and perform appropriate investigations for overlapping rheumatic conditions with sarcoidosis. Lip biopsy remains a crucial investigation for cases referred with sicca features, not only to establish the diagnosis of SS but also to exclude associated lymphoma or sarcoidosis.


SJÖ GREN'S SYNDROME AND ITS MIMICS O10 A CASE OF SARCOIDOSIS MIMICKING SJÖ GREN'S SYNDROME ALONG WITH ABNORMAL NAILFOLD CAPILLAROSCOPY
Ziad Alkutobi, Arslan Sidhu and Anupama Nandagudi Basildon and Thurrock University Hospitals, Basildon, United Kingdom Case report -Introduction: Sicca symptoms and Raynaud's disease are amongst the common presentation of Sjö gren's syndrome (SS) which has an estimated prevalence of 1%. We are reporting a case that was initially considered as SS, but subsequent lip biopsy confirmed histological evidence of sarcoidosis in the presence of abnormal nailfold capillaroscopy and negative autoimmune profile. Sarcoidosis can be associated with various autoimmune diseases including systemic sclerosis, Hashimoto's thyroiditis, systemic lupus erythematosus, rheumatoid arthritis, Sjö gren's syndrome and spondyloarthropathy. Case report -Case description: A 29-year-old Caucasian man was referred to rheumatology clinic, suffering with ongoing sicca symptoms including dry itchy gritty eyes, sore red eyelids, persistent mouth dryness and cracking ulcers. He has history of Raynaud's disease, dysphagia for solid, dry cough and photosensitive rash on his chest and arms. His other symptoms were polyarthralgia, myalgia, headache, persistent fatigue, poor memory, dry skin over the hands and tingling sensation at the feet. There was no history of joint swelling, uveitis, chest pain or shortness of breath. He has hypothyroidism secondary to radioiodine treatment and is currently on levothyroxine. He works in a factory, drinks alcohol occasionally and is non-smoker. There was no family history of autoimmune conditions. General and systemic examination was unremarkable, and there were no stigmata of connective tissue disease except for tenderness and crepitus at the right knee joint. His saliva flow rate was 0.4ml/5 mins and Schirmer's test was 20mm/5 mins (both were normal values). Autoimmune profile was negative; ANA: 0.2 units, RF factor: <10, CCP antibodies (IgG): 1.1U/mL. Apart from mild lymphopenia, the other laboratory tests were at normal range including CK, CRP, adjusted calcium, ACE, immunoglobulins, serum protein electrophoresis and urine test for protein.Chest X-ray and lung function test were both normal. Nailfold capillaroscopy shows grade1 enlarged capillaries, prominent capillary ramifications in the form of bushy capillaries and some microhaemorrhages, a pattern suggestive of mixed connective tissue disease.
Lip biopsy showed small non-caseating epithelioid granuloma within the parenchyma. The initial working diagnosis was a possible serology-negative SS, but the later results of lip biopsy confirmed sarcoidosis. He was commenced on Hydroxychloroquine and advised about cognitive-behavioural therapy for generalised ache and fatigue. As he was not responding to pilocarpine, a referral for ophthalmology review was requested.
Case report -Discussion: Sarcoidosis is a multisystem disease of unknown aetiology, known to exist worldwide with a variable prevalence of around 20 per 100,000 in the UK. It is characterized by the presence of multiple non-caseating granulomas, inflammation which may occur in any tissue in the body manifested as local symptoms with or without systemic features. Involvement of the salivary and lacrimal glands can result in xerostomia and xerophthalmia, respectively. Chronic sarcoid has an often subtle, insidious, progressive, and highly variable clinical course; it can be asymptomatic in a significant percentage of the cases. In additionto the clinical and radiological findings, the diagnosis of sarcoidosis shouldbebasedonthehistologicalproofof non-caseatinggranulomas and ruling out other diseases that shares similar features. SS has many features in common with sarcoidosis; these may include sicca symptoms, arthralgia, myalgia, arthritis, erythematous rash, lymphadenopathy, peripheral neuropathy, fatigue and raised inflammatory markers. Moreover, positive rheumatoid factor, defective T suppressor cell regulation and HLA-DR3 are linked to both diseases. Pulmonary involvement in either of them may have similar clinical and radiographic manifestations, making it difficult for the clinician to distinguish between these diseases. In terms of management, hydroxychloroquine may help the skin and joint manifestations in both diseases whereas more severe diseases may require treatment with glucocorticoids, methotrexate, and azathioprine. Nevertheless, prompt confirmation of the diagnosis is crucial provided the difference in response to medication, complications, outcome, and prognosis. Based on the clinical and lip biopsy findings, the above case was diagnosed with sarcoidosis. The case did not meet the criteria for the diagnosis of SS provided the negative results of Schirmer's test, saliva flow test and autoimmune profile. Case report -Key learning points: Raynaud's phenomenon (secondary Raynaud's) with positive capillaroscopy findings was an unusual early presenting feature of sarcoidosis in the above case. There was one case report of similar presentation published in 2011. However, literature did not reveal specific information about abnormal nailfold capillaroscopy results in sarcoidosis. Dry eyes and dry mouth may occur in sarcoidosis mimicking the presentation of primary SS. In addition, true coexistence of sarcoidosis and SS has also been defined based on the histopathologic examination of the exocrine glands. Diagnostic criteria need to be applied for patients with suspected overlap of the two diseases.
Clinicians will need to be vigilant and perform appropriate investigations for overlapping rheumatic conditions with sarcoidosis. Lip biopsy remains a crucial investigation for cases referred with sicca features, not only to establish the diagnosis of SS but also to exclude associated lymphoma orsarcoidosis.

Alaeldin Mohamednour and Maumer Durrani
Leicester Royal infirmary, Leicester, United Kingdom Case report -Introduction: Primary Sjö gren's syndrome (PSS) is a systemic autoimmune disease that mainly affects exocrine glands.
Central nervous system (CNS) involvement in primary SS is extremely rare. In 10-20% of patients diagnosed with PSS, there are lesions in the central nervous system analogous to those presented in multiple sclerosis. We report a case of a 58-year-old female, diagnosed as PSS and multiple sclerosis (MS) (2007) Based on these MRI findings and the recent history of skin vasculitis, the deterioration in her neurological condition was put down to primary Sjö gren's. Therefore, her treatment was escalated to cyclophosphamide during the COVID-19 pandemic with a particularly good outcome. She was then switched to MMF and her condition remained stable. Case report -Discussion: Neurological disorders are one of the rare manifestations of primary Sjö gren's. The first reports regarding the involvement of the nervous system in PSS were published in 1980. Distinguishing betweenmultiple sclerosis and CNS-SS is not easy. Not only because of similarities of the MRI findings, but also the course of the disease can be like MS, either chronic or relapsing and remitting. This usuallyleads to missingor delaying in the diagnosis as shown in this case. However, Peripheral neuropathy is far much common in PSS rather MS which can help in differentiating these two conditions. Distal axonal sensory polyneuropathy is the most usual form of neuropathy in PSS as illustrated in this case. Furthermore, up to 75% of patients with SS and active CNS disease have been shown to have concomitant active peripheral vasculitis affecting the skin, muscles, and nerves. Our patient later developed skin vasculitis and peripheral neuropathy which made us think that all the neurological findings including the lesions on the brain are more likely to berelated to PSS rather MS. Cognitive disorders are common manifestations of CNS-SS such as attention disorder and memory deficit. Dementia-related to CNS-SS seems to be reversible after immunosuppressive treatment. A second MDT discussion took place and after considering the risk-benefit ratio, the decision was made to give cyclophosphamide. Patient was given all the information to make an informed decision. Patient asked for more time tothink and discuss with herpartner,but eventually, shehad decided to have cyclophosphamide despite all the risks and uncertainties around the COVID-19 pandemic. Our patient has noticed significant improvement regarding cognition after completing cyclophosphamide treatment and she was pleased with this outcome.
Case report -Key learning points: 1/ Distinguishing between multiple sclerosis and CNS-SS is difficult 2/ neurophysiological tests should be considered even in asymptomatic patients as they contribute to the detection of early and subtle damage to the nervous system. 3/ Successful outcome being achieved with intensive immunosuppression despite all the uncertainties around the COVID-19 -19pandemic. 4/ This case highlights the importance of communication and openness in shared decisions, especially while confronting uncertainties such as in COVID-19pandemic. Case report -Introduction: Distal renal tubular acidosis (RTA) type 1 is a rare condition in adults, which is characterised by impaired distal urine acidification. It is caused by failure of alpha intercalated cells and presents with alkaline urine (pH.5.5), metabolic acidosis, hypokalaemia, osteomalacia, nephrocalcinosis and hypercalciuria. Autoimmune diseases, such as Sjö gren's Syndrome (SS) and Rheumatoid Arthritis are among the most common causes. RTA can be the presenting feature of the rheumatic disease. SS is typically characterised by impaired lacrimal and salivary gland function but has a broad spectrum of symptoms and disease severity, whichincludes renal involvement in up to 40%. Case report -Case description: We present the case of a 29-year-old female, referred to rheumatology by endocrinology following a recent emergency admission. The patient, who had woken with severe muscle paralysis of sudden onset, presented at A&E and imminently suffered a Pulseless Electrical Activity arrest. Urgent investigations identified profound hypokalaemia (serum potassium <1.5mmol/l), creatinine 120umol/l,ametabolicacidosis(bicarbonate 5.8 mmol/l),hypercalcemia (2.96 mmol/l) and hypermagnesemia (1.87mmol/l). She was successfully resuscitatedand stabilised. During her admission, under the care of endocrine and renal teams, a diagnosis of Distal RTA was reached. A CT scan showed medullary nephrocalcinosis. Immunology results: ANA 1:2560 (speckled pattern) and strongly positive Ro antibodies; polyclonal IgG of 19g/l, normal complement. Renal function returned to normal and she had negligible albuminuria. The causation of RTA was not certain. She was discharged well and on magnesium, potassium, and bicarbonate supplements. No renal biopsy was performed. Past medical history included a pregnancy resulting in c-section (intrauterine growth retardation), six months previously. The baby was normal but post-partum the patient remained unwell with fatigue, nausea, and vomiting. She was diagnosed with postnatal depression. She had no other significant medical history but since the admission had suffered from a pneumonia and hearing loss due to recurrent otitis media. As SS was considered as a possible cause of RTA, she was referred for a rheumatological opinion. She was fatigued but denied sicca symptomatology (Schirmer's test was negative, and normal saliva production). She had a patch of alopecia, but no other classical features of SS. Salivary glands ultrasoundwereunremarkable. Her biochemistrywas nownormal except a low normal bicarbonate; inflammatory markers were within normal range.

O12 PULSELESS ELECTRICAL ACTIVITY ARREST IN
Case report -Discussion: This patient presents several challenges. She represents a common scenario of the balance between the high clinical suspicion and the failure to meet the ACR/EULAR diagnostic criteria for SS and the resultant management dilemma. She has no sicca symptoms, normal salivary gland US and hence biopsy was not sought. Anti-Ro/SSA and anti-La/SSB antibodies are detected on average 5 years before the appearance of an overt clinical phenotype of SS and thus serve also as predictive markers of the disease. In this patient with a strongly positive speckled ANA with anti RO along with RTA1(and renal microcalcification indicating long duration) without other explanation is almost certainly SS. The absence of renal biopsy in this patient also added to the challenge of clarification of her renal pathology. Renal involvement in SS is divided into: Overt glomerular disease which presents with haematuria, proteinuria or frank nephrotic syndrome; and Covert tubular disease whichmanifests with the metabolic abnormalities due to: proximal tubular injury; distal RTA or urinary concentrating defects. The prevalence of overt (glomerular) renal disease in SS is 4.3-5%. The prevalence of covert disease is 10-42% (Proximal tubular injury 10-42%; Distal RTA 5-24%; urinary concentrating defect 17-28%). Type1 RTA is confirmed by inappropriately alkaline urine (PH > 5.3) during systemic acidosis. The acidosis may occur spontaneously or be induced by an acid load test (NH4CL). Also, by stimulating intercalated