The early symptoms of Sjogren's syndrome can be easily overlooked. The main clinical presentation in adults is xerophthalmia and xerostomia. In children, bilateral swelling of the parotid glands is a common sign. Xerophthalmia and xerostomia are often described in the following way by affected individuals:

• Unable to eat dry foods
• Tongue sticks on the roof of the mouth
• Unable to speak for long period
• Increased cases of dental caries
• Change in tastes
• Painful mouth
• Sandy sensation in the eyes
• Persistent, dry cough

In addition to these sicca symptoms, various extraglandular manifestations may develop, including arthralgia, arthritis, myalgia, Raynaud phenomenon, pulmonary disease, gastrointestinal disease, pericarditis, pulmonary hypertension, lymphadenopathy, neuropathy, vasculitis, renal tubular acidosis, xeroderma, purpura, and urticaria. Late complications such as blindness, dental destruction, oral candidiasis, and non-Hodgkin lymphoma may occur [6].

• Splenomegaly

  On admission, she presented with anemia, elevated C-reactive protein levels, anasarca, and hepato-splenomegaly. [ncbi.nlm.nih.gov]

  Rarely splenomegaly or myositis may be seen. If we make blood tests, we can see leukopenia, anemia, an increased Erythrocyte sedimentation rate, and some autoantibodies. [youtube.com]

  The rating of this domain should take in account the following comments: Lymphadenopathy or splenomegaly: If there is no significant lymph node or splenomegaly at the clinical examination, no other examination is requested. [rmdopen.bmj.com]

  Clinical risk factors include persistent, unilateral salivary gland enlargement, lymphadenopathy, splenomegaly, skin vasculitis, cryoglobulinemia and the development of glomerulonephritis [54, 55]. [medsci.org]

  […] syndrome26 Malaise Peripheral neuropathy Primary biliary cirrhosis Fatigue Autoimmune thyroiditis GI symptoms Fibromyalgia Renal tubular acidosis Respiratory diseases Fever Myositis Psychosis Arthralgia Chronic hepatitis Lymphadenopathy Synovitis Purpura Splenomegaly [onlinelibrary.wiley.com]

• Fatigue

  BMI, body mass index; VAS, Visual Analogue Scale (0–100, with 0 = no fatigue/no pain at all); ProF, Profile of Fatigue (0–112, somatic fatigue 0–7, mental fatigue 0–7, with 0 = no problem at all); VO 2max, maximal oxygen consumption; RPE, ratings of perceived [doi.org]

  Fatigue was evaluated in the patients using the Fatigue Severity Scale (FSS) and a fatigue visual analogue scale (VAS); mood was evaluated using the Beck Depression Inventory (BDI). [ncbi.nlm.nih.gov]

  Balance of rest and exercise Guided exercise programs can help patients overcome fatigue, maintain flexibility, and overcome joint and muscle pain. Good sleep hygiene is helpful for improving fatigue and body pain. [clevelandclinic.org]

• Anemia

  On admission, she presented with anemia, elevated C-reactive protein levels, anasarca, and hepato-splenomegaly. [ncbi.nlm.nih.gov]

  Blood tests may reveal elevated erythrocyte sedimentation rate and transaminase levels, anemia, leukopenia, eosinophilia, hypergammaglobulinemia, presence of antinuclear antibodies, anti–alpha-fodrin antibody and rheumatoid factor. [symptoma.com]

  The primary feature of this condition is a megaloblastic anemia. [enerca.org]

  anemia. A??long-term follow-up study. Acta Paediatr Scand 1984;73(2):248-53. [Pubmed] [revistanefrologia.com]

  The primary feature of this condition is a blood disorder called megaloblastic anemia. In this form of anemia, which is a disorder characterized by the shortage of red blood cells, the red cells that are present are abnormally large. [ghr.nlm.nih.gov]

• Fever

  The patient was admitted due to dyspnea, fever, polyarthralgia, and generalized edema. [ncbi.nlm.nih.gov]

  […] something is in the eye Mouth and throat symptoms: Difficulty swallowing or eating dry foods Loss of sense of taste Problems speaking Thick or stringy saliva Mouth sores or pain Teeth decay and gum inflammation Hoarseness Other symptoms may include: Fatigue Fever [mountsinai.org]

  Fever: axillary temperature >37.5 °C, not associated with infectious processes. Lymphadenopathy: enlarged nodes (>1 cm) in the cervical, axillary, or inguinal regions. Cutaneous vasculitis demonstrated by cutaneous purpura and/ or rash. [doi.org]

• Weight Loss

  A 65-year-old woman with Sjögren's syndrome presented with recurrent abdominal distension, constipation, weight loss, orthostatic dizziness, loss of sweating and incomplete emptying of the bladder. [ncbi.nlm.nih.gov]

  The following can all be symptoms of lymphoma: night sweats, fever fatigue unexplained weight loss Call your doctor if you have any of these symptoms. [healthline.com]

  The criteria for qualifying for disability benefits based on Sjogren’s syndrome are: Two or more body systems or organs affected At least one system/function/organ affected to a moderate or severe level At least two of the following symptoms: weight loss [disability-benefits-help.org]

  Some patients experience constitutional symptoms like fever, involuntary weight loss or night sweats. Since dental health requires saliva, Ms. [nytimes.com]

• Chronic Fatigue Syndrome

  Komaroff, The Frequency of HLA Class II Antigens in Chronic Fatigue Syndrome, Journal of Chronic Fatigue Syndrome, 11, 4, (33), (2003). Juan-Manuel Anaya, Paula A. Correa, Ruben D. [doi.org]

  These dryness symptoms can occur in other conditions — including chronic fatigue syndrome and fibromyalgia — and can be side effects of a variety of commonly used medications. [hopkinsmedicine.org]

  Myalgic encephalomyelitis, commonly known as chronic fatigue syndrome, can affect people of any age, including children. Type 1 diabetes can affect anyone of any age, but is more common in people under 30 years. [betterhealth.vic.gov.au]

  That's because the symptoms can overlap or look like those of other conditions, including chronic fatigue syndrome, fibromyalgia, lupus, rheumatoid arthritis, or even multiple sclerosis. [stlouischildrens.org]

  That’s because the symptoms can overlap or look like those of other conditions, including chronic fatigue syndrome, fibromyalgia, lupus, rheumatoid arthritis, or even multiple sclerosis. [urmc.rochester.edu]

• Cough

  Unexplained cough was defined as chronic cough of unknown etiology despite algorithm-based evaluation and treatment. [ncbi.nlm.nih.gov]

  […] in the following way by affected individuals: Unable to eat dry foods Tongue sticks on the roof of the mouth Unable to speak for long period Increased cases of dental caries Change in tastes Painful mouth Sandy sensation in the eyes Persistent, dry cough [symptoma.com]

  It’s also quite common for those with the condition to have a cough. However, in instances where shortness of breath, wheezing or a having a cough for a prolonged period is experienced, then it is recommended that further medical help is sought. [news-medical.net]

• Dry Cough

  Sandy sensation in the eyes Persistent, dry cough In addition to these sicca symptoms, various extraglandular manifestations may develop, including arthralgia, arthritis, myalgia, Raynaud phenomenon, pulmonary disease, gastrointestinal disease, pericarditis [symptoma.com]

  (The most frequent reason, in 80%, was persistent, dry cough periodically waking up patients at night). The chest HRCT scan revealed lung tissue changes in the course of 29% of all examined patients (of 68). [ncbi.nlm.nih.gov]

  Skin rashes Chronic dry cough Vaginal dryness Problems urinating, including pain, urinating more than usual, getting up at night often to urinate and needing to urinate suddenly Numbness or tingling in your fingers and toes Prolonged fatigue and/or a [hopkinsmedicine.org]

  Other symptoms can include joint swelling and pain, vaginal dryness, persistent dry cough and dry skin. It may also spread to other parts of the body such as blood vessels, nerves, lungs, kidneys and digestive system. [coloradoarthritis.com]

  […] such as crackers decreased or altered sense of taste difficulty speaking increase in dental cavities and even tooth loss at early age dry cough or hoarseness Enlarged parotid glands (located at the angle of jaw), looking like an infection Excessive fatigue [my.clevelandclinic.org]

• Hoarseness

  Others are forced to cope with blurred vision, constant eye discomfort, recurrent mouth infections, swollen parotid glands, hoarseness, and difficulty in swallowing and eating. [ninds.nih.gov]

  Burning sensation in eyes Feeling that something is in the eye Mouth and throat symptoms: Difficulty swallowing or eating dry foods Loss of sense of taste Problems speaking Thick or stringy saliva Mouth sores or pain Teeth decay and gum inflammation Hoarseness [mountsinai.org]

  You may be hoarse if your vocal cords are swollen or get irritated from a dry throat or coughing. Do not clear your throat before you talk. Drink some water or use gum or candy to prevent hoarseness. [drugs.com]

  Eye symptoms: Itching eyes Feeling that something is in the eye Mouth and throat symptoms: Difficulty swallowing or eating dry foods Loss of sense of taste Problems speaking Thick or stringy saliva Mouth sores or pain Teeth decay and gum inflammation Hoarseness [nlm.nih.gov]

  […] symptom Extremely dry mouth and throat, causing: difficulty chewing and swallowing, especially dry food such as crackers decreased or altered sense of taste difficulty speaking increase in dental cavities and even tooth loss at early age dry cough or hoarseness [my.clevelandclinic.org]

• Chronic Cough

  Unexplained cough was defined as chronic cough of unknown etiology despite algorithm-based evaluation and treatment. [ncbi.nlm.nih.gov]

  In Sjögren syndrome, extraglandular involvement may result in painful inflammation of the joints and muscles; dry, itchy skin and skin rashes; chronic cough; a hoarse voice; kidney and liver problems; numbness or tingling in the hands and feet; and, in [medlineplus.gov]

  The patient may be seeing various health professionals, perhaps a dentist for dry mouth and tooth decay, a gynecologist for vaginal dryness, and an ophthalmologist for dry eyes, a pulmonologist for shortness of breath and chronic cough. [medicalnewstoday.com]

  cough, recurrent bronchitis with chronic diffuse interstitial infiltrates on radiography, abnormal spirometry, pulmonary alveolitis or fibrosis on computed tomography) 29 Raynaud’s phenomenon 16 to 28 Cutaneous vasculitis 12 Peripheral neuropathy 7 Lymphadenopathy [aafp.org]

• Dyspnea

  This paper examines a 58-year-old man with SS, severe dyspnea and recurrent exacerbations associated with bronchiolitis-bronchiectasis. [ncbi.nlm.nih.gov]

  Lung involvement: persistent cough and/or dyspnea, with chronic diffuse interstitial infiltrates on X-ray, altered pattern on pulmonary function studies, and/or evidence of pulmonary alveolitis/fibrosis in computed tomography scan. [doi.org]

  ILD is difficult to treat and results in an increase of dry cough and dyspnea, leading to decreased quality of life. [medsci.org]

• Dysphagia

  As secretion of saliva decreases, patients with SS experience rampant caries, pain in the oral mucosa, inflammation and hardening of the salivary glands, abnormal taste, dysphagia, and loss of teeth earlier than healthy individuals. [ncbi.nlm.nih.gov]

  This is accompanied by a reduction in oral phosphate levels and xerostomia due to reduced salivary flow, which can lead to infections, progressive caries, dysphagia and oral pain. [genome.jp]

  Gastrointestinal manifestations mainly include dysphagia due to lack of saliva, but also due to esophageal dysmotility. [rheumatologyadvisor.com]

  Dryness and gastrointestinal mucosal or submucosal atrophy and diffuse infiltration by plasma cells and lymphocytes may cause symptoms (eg, dysphagia). [msdmanuals.com]

  Dysphagia might also occur, which can lead to aspiration pneumonia. The systemic autoimmunity associated with Sjögren's syndrome can also result in dry skin, fatigue, low grade fever, constipation, myalgia, and joint pain. [nature.com]

• Constipation

  A 65-year-old woman with Sjögren's syndrome presented with recurrent abdominal distension, constipation, weight loss, orthostatic dizziness, loss of sweating and incomplete emptying of the bladder. [ncbi.nlm.nih.gov]

  CASE REPORT A 22-year-old woman had been referred to our pediatric outpatient clinic with the complaints of pale skin, loss of appetite, ataxia and diarrhea-constipation periods when she was 2-year-old. [revistanefrologia.com]

  Case report A 22-year-old woman had been referred to our pediatric outpatient clinic with the complaints of pale skin, loss of appetite, ataxia and diarrhea-constipation periods when she was 2-year-old. [scielo.isciii.es]

  The systemic autoimmunity associated with Sjögren's syndrome can also result in dry skin, fatigue, low grade fever, constipation, myalgia, and joint pain. [nature.com]

• Abdominal Pain

  […] of numbness or tingling Thyroid gland abnormalities Skin rashes Memory loss, difficulty concentrating or confusion Gastrointestinal problems, such as acid reflex, bloating, abdominal pain, or diarrhea Inflammation of the lungs, kidneys (unlike lupus [my.clevelandclinic.org]

  Skin rashes Memory loss, difficulty concentrating or confusion Gastrointestinal problems, such as acid reflex, bloating, abdominal pain, or diarrhea Inflammation of the lungs, kidneys (unlike lupus nephritis), liver, or pancreas Cancer of the lymphatic [clevelandclinic.org]

  This can cause lower abdominal pain and alteration in bowel habit. Treatment of this tends to consist of the increase of fibre (bran) within the diet and occasionally the use of antispasmodic drugs. [sruk.co.uk]

  Side effects can include sweating, abdominal pain, flushing and increased urination. Address specific complications. [mayoclinic.org]

  Anemia and proteinuria were determined in our patient from the clinical findings of pallor, weakness, weight loss, abdominal pain, glossitis and vomiting together with the laboratory results. [oatext.com]

• Xerostomia

  Eye dryness and xerostomia were assessed by the Schirmer's test, the Fox test and the visual analogue scale (VAS). [ncbi.nlm.nih.gov]

  The main clinical presentation in adults is xerophthalmia and xerostomia. In children, bilateral swelling of the parotid glands is a common sign. [symptoma.com]

  Additionally, xerostomia symptoms are common to many conditions and are, in part, subjective. [jamanetwork.com]

• Dental Caries

  Dental treatments including scaling, tooth extraction, root canal treatment, periodontal therapy and dental prosthesis were provided if indicated. Restorations were provided except for initial caries lesion and arrested non-cavitated caries lesions. [web.archive.org]

  The International Caries Detection and Assessment System (ICDAS): an integrated system for measuring dental caries. [ncbi.nlm.nih.gov]

  Xerophthalmia and xerostomia are often described in the following way by affected individuals: Unable to eat dry foods Tongue sticks on the roof of the mouth Unable to speak for long period Increased cases of dental caries Change in tastes Painful mouth [symptoma.com]

• Cheilitis

  Other frequent complaints are angular cheilitis, simple cheilitis ( Figures 1 and 2 ), exfoliative cheilitis, lip cracking and dryness ( Table 2 ). [ncbi.nlm.nih.gov]

  Oral-related complications include sialoliths (salivary stones), salivary gland swelling, high caries risk, particularly at the cusp tips and cervical sites, periodontal disease, and soft tissue infections such as candidiasis, and angular cheilitis. [nature.com]

  Oral candidiasis and angular cheilitis. Dry eyes tend to cause a gritty sensation. There is a predisposition to blepharitis and the eyes may be sticky in the morning. [patient.info]

  On examination had grade 2 PEM as per IAP classification with pallor, cheilitis and glossitis (Figure 2). Multiple ecchymotic patches were present in the knuckles and periumbilical area (Figure 1). [oatext.com]

  In addition to the appearance of dental caries, angular cheilitis associated with candidiasis may exist.1 The taste buds also may be abnormal, resembling those of patients with idiopathic hypogeusia, and their number decrease.53 Gross accumulation of [jamanetwork.com]

• Parotid Swelling

  Corticosteroids (eg, oral prednisone 1 mg/kg once a day), or rituximab may be needed in severe disease with cryoglobulinemic vasculitis, peripheral neuropathy/multiple mononeuropathy, severe parotid swelling, severe lung involvement, or inflammatory arthritis [msdmanuals.com]

  Corticosteroids (eg, oral prednisone 1 mg/kg once a day), or rituximab may be needed in severe disease with cryoglobulinemic vasculitis, peripheral neuropathy/mononeuritis multiplex, severe parotid swelling, severe lung involvement, or inflammatory arthritis [merckmanuals.com]

  […] keratomileusis Up to 34% of patients with Sjögren’s syndrome report episodic or chronic, typically bilateral swelling of the parotid glands (9). [ncbi.nlm.nih.gov]

  Clinical trials of tocilizumab in this setting are ongoing.4 Treatment Recommendations for Specific Systemic Manifestations15 Parotid swelling: NSAIDS and short term oral corticosteroids with dosages less than 20 mg/day for a maximum of 1 month. [rheumatologyadvisor.com]

• Cracked Lips

  Using oil or petroleum-based lip balm or lipstick to help dry, cracked lips feel better. Using a saliva substitute prescribed by a doctor to make the mouth feel wet. Using medicine to help your mouth make more saliva. [web.archive.org]

  […] including: Chronic inflammation Corneal ulcerations Eye infections Dry nose Nose bleeds Chronic or recurrent sinusitis Mouth sores/ulcerations Swollen salivary glands Salivary gland stones Dental decay, dentures Problems with speech Problems with taste Dry, cracked [migraine.com]

  […] may be generalized mucosal soreness and ulceration of the areas covered by the denture.[3] Mouth soreness and oral mucositis.[1][3] Lipstick or food may stick to the teeth.[1] A need to sip drinks frequently while talking or eating.[3] Dry, sore, and cracked [en.wikipedia.org]

• Arthralgia

  The most difficult challenge in diagnosis and therapy is patients with symptoms of fibromyalgia (arthralgia, myalgia, fatigue) and oral and ocular dryness in the presence of circulating antinuclear antibodies. [ncbi.nlm.nih.gov]

  Arthralgias occur in about 50% of patients. Arthritis occurs in about 33% of patients and is similar in distribution to rheumatoid arthritis but is not erosive. [msdmanuals.com]

  […] mouth Unable to speak for long period Increased cases of dental caries Change in tastes Painful mouth Sandy sensation in the eyes Persistent, dry cough In addition to these sicca symptoms, various extraglandular manifestations may develop, including arthralgia [symptoma.com]

  The clinical manifestations evaluated were defined as follows: Articular involvement: arthralgia and/or nonerosive arthritis characterized by tenderness, swelling, or effusion involving 2 or more peripheral joints. [doi.org]

  Included classifications: — Adult Still's disease [Cush] — Adult Still's disease [Yamaguchi] — Antiphospholipid syndrome — Arthralgia suspicious for progression to RA [2016 EULAR] — Axial spondyloarthritis — Behçet’s Disease (ICBD) — Fibromyalgia [2016 [play.google.com]

• Dry Skin

  Females are affected more than males Symptoms: Alopecia (hairloss), Dry: skin, eyes, nose, mouth, respiratory tract, vagina. The dry skin can be treated with lubricants. The dry vagina can cause dyspareunia, which is a painful sexual intercourse. [youtube.com]

  […] eyes Dry mouth Difficulty swallowing Loss of taste and smell Dry skin, nose, throat, and/or lungs Dental problems Swollen salivary glands Vaginal dryness Skin rashes Joint pain, swelling, and stiffness Muscle pain Fatigue In some cases, other parts of [winchesterhospital.org]

  […] eyes Dry mouth Difficulty swallowing Loss of taste and smell Dry skin, nose, throat, and/or lungs Swollen salivary glands Severe dental cavities caused by dry mouth Oral yeast infections Vaginal dryness Skin rashes Joint pain, swelling and stiffness [uvahealth.com]

  Treating other symptoms of Sjogren's syndrome Dry skin Several soaps and creams are especially designed for people with dry skin. Your pharmacist or GP can advise you. [hse.ie]

  […] of the lacrimal and salivary glands.1, 3 In a cohort from the NIDCR/NIH Sjögren's Syndrome Clinic, 87.4% of people with confirmed Sjögren disease reported dry mouth.8 Other organ systems can be affected as well (e.g., nasal passages, vagina, skin).3 Skin [ada.org]

• Urticaria

  […] manifestations may develop, including arthralgia, arthritis, myalgia, Raynaud phenomenon, pulmonary disease, gastrointestinal disease, pericarditis, pulmonary hypertension, lymphadenopathy, neuropathy, vasculitis, renal tubular acidosis, xeroderma, purpura, and urticaria [symptoma.com]

  “Inhibition of BTK has emerged as a potential therapeutic option for a number of autoimmune diseases,” including chronic urticaria, presenter Thomas Dörner (Charité – Universitätsmedizin Berlin, Germany) told delegates at ACR Convergence 2022 in Philadelphia [rheumatology.medicinematters.com]

  They may present as red flat patches, raised lumps, blisters, ulcers or a raised urticaria-like weals. [dermnetnz.org]

  DH in pediatric patients presents as urticaria, and more commonly purpura involving the soles, palms, and sides of the fingers. [doi.org]

• Chronic Urticaria

  “Inhibition of BTK has emerged as a potential therapeutic option for a number of autoimmune diseases,” including chronic urticaria, presenter Thomas Dörner (Charité – Universitätsmedizin Berlin, Germany) told delegates at ACR Convergence 2022 in Philadelphia [rheumatology.medicinematters.com]

• Dry Eyes

  Dry Eye. American Academy of Ophthalmology. EyeSmart® Eye health. https://www.aao.org/eye-health/diseases/dry-eye-list. Accessed March 08, 2019. Boyd K, Devin A Harrison. Sjögren's Syndrome. American Academy of Ophthalmology. [eyewiki.aao.org]

  Primary Sjögren syndrome is defined as dry eyes and dry mouth without another autoimmune disorder. [mountsinai.org]

  Diagnosis Ophthalmologists at The Ocular Surface Diseases and Dry Eye Clinic are leading experts in the study, diagnosis and treatment of dry eye syndrome. [hopkinsmedicine.org]

  Dry Eye. American Academy of Ophthalmology. EyeSmart ® Eye health. https://www.aao.org/eye-health/diseases/dry-eye-list. Accessed March 08, 2019. Boyd K, Devin A Harrison. Sjögren's Syndrome. American Academy of Ophthalmology. [eyewiki.org]

  Dry eyes can be diagnosed by an ophthalmologist (eye doctor) by measuring tear production or carefully examining the cornea (clear part of the eye). Certain laboratory tests also suggest that dry eyes and mouth are caused by autoimmune mechanisms. [clevelandclinic.org]

• Lacrimation

  EBV genomes can be detected in 35% of normal lacrimal glands. EBV genotype analysis by PCR revealed that only type I EBV genomes could be detected in 35% of normal lacrimal glands. [eyewiki.aao.org]

  Efficacy of treatment will be assessed by monitoring salivary and lacrimal function, serological markers of autoimmune activity, and subjective reports of local and systemic symptoms. [clinicaltrials.gov]

  We further hypothesize that androgen treatment to the ocular surface will promote both lacrimal and meibomian gland function and alleviate both "aqueous-deficient" and "evaporative" dry eye. [ncbi.nlm.nih.gov]

  Rose Bengal test measures the function of the lacrimal glands and Schirmer's test the production of tears. [symptoma.com]

• Blurred Vision

  Others are forced to cope with blurred vision, constant eye discomfort, recurrent mouth infections, swollen parotid glands, hoarseness, and difficulty in swallowing and eating. [ninds.nih.gov]

  Besides being painful, having dry eyes makes it harder to perform everyday tasks, like working on a computer, and many patients have blurred vision. Dry mouth makes it very difficult to eat and causes burning of the throat. [youtube.com]

  This change of the tear film results in irritation and changes on the eye surface that lead to characteristic symptoms, such as a sandy or burning sensation, discomfort, blurred vision, and redness of the eye that progresses as the day goes on. [hopkinsmedicine.org]

  Blurred vision and dizziness can affect your ability to do skilled tasks such as driving or operating heavy machinery. [hse.ie]

  Some individuals also experience blurred vision and heightened light sensitivity. [health.facty.com]

• Blepharitis

  Private practitioners were more likely to use symptom questionnaires and grading scales and to describe anterior blepharitis. Academic settings were more likely to record TBUT and tear meniscus height. [ncbi.nlm.nih.gov]

  Symptoms of Sjögren's syndrome Eye-related symptoms of Sjögren's syndrome include: Very dry eyes Blurry vision Burning or stinging eyes A gritty feeling in the eye Being very sensitive to light Discomfort wearing contact lenses Blepharitis (inflamed eyelid [aao.org]

  Evaporation of the water from the tear film can increase due to blepharitis (inflammation of the eyelid), decreased production by the oil glands of the eyelids, or thyroid disease. [hopkinsmedicine.org]

  The GP can check for some of the more common causes, such as swelling of the eyelids (blepharitis), diabetes or side effects from medicine. [nhs.uk]

• Lacrimal Gland Enlargement

  […] of keratoconjunctivitis sicca with or without lacrimal gland enlargement, xerostomia with or without salivary gland enlargement, and the presence of a connective tissue disease, usually rheumatoid arthritis but sometimes systemic lupus erythematosus, [icd9data.com]

  Definition (CSP) symptom complex of unknown etiology, usually occurring in middle-aged or older women, marked by the triad of keratoconjunctivitis sicca with or without lacrimal gland enlargement, xerostomia with or without salivary gland enlargement, [fpnotebook.com]

  Disease Entity History The first clinical description of Sjögren syndrome was by Mikulicz, who described a 42-year-old with bilateral parotid and lacrimal gland enlargement in 1892. [eyewiki.aao.org]

  History The first clinical description of Sjögren syndrome was by Mikulicz, who described a 42-year-old with bilateral parotid and lacrimal gland enlargement in 1892. [eyewiki.org]

  Historical information The first clinical description of Sjögren syndrome was by Mikulicz, who described a 42-year-old with bilateral parotid and lacrimal gland enlargement in 1892. [emedicine.com]

• Paresthesia

  Peripheral neuropathy: paresthesias, numbness, and/or motor defects of the lower/upper extremities confirmed by electromyography. Sural nerve biopsy showing vasculitis involving vasa nervorum when performed. [doi.org]

  3 (16.6%) – Non-specific ulcerations 2 (9.1%) 4 (22.2%) 1 (6.7%) Small aphtae 3 (13.6%) 2 (11.1%) 1 (6.7%) Sutton's aphtae 1 (4.5%) – – Geographic tongue – 2 (11.1%) 1 (6.7%) Fissured tongue 1 (4.5%) – – Enlargement of salivary glands 2 (9.1%) – – Paresthesias [ncbi.nlm.nih.gov]

• Vaginal Dryness

  In addition, Sjogren's syndrome may cause skin, nose, and vaginal dryness, and may affect other organs of the body including the kidneys, blood vessels, lungs, liver, pancreas, and brain. [ninds.nih.gov]

  For Vaginal Dryness Water-based vaginal lubricants (K-Y Jelly, Astroglide, Replens, Luvena) can ease vaginal dryness and painful intercourse. [arthritis.org]

  dryness can be treated with lubricants, hormone replacement therapy, and estrogen creams. [symptoma.com]

  In women (who are most commonly affected), the glands that keep the vagina moist can also be affected, leading to vaginal dryness. Dry eyes There is a deficiency of the watery component of the tears. [sruk.co.uk]

  Vaginal dryness The symptoms of vaginal dryness can be treated using a lubricant, such as KY Jelly. Some women also consider using oestrogen creams or hormone replacement therapy (HRT). [hse.ie]

No single examination is sufficiently sensitive or specific in the diagnosis of the disorder. Blood tests may reveal elevated erythrocyte sedimentation rate and transaminase levels, anemia, leukopenia, eosinophilia, hypergammaglobulinemia, presence of antinuclear antibodies, anti–alpha-fodrin antibody and rheumatoid factor. Rose Bengal test measures the function of the lacrimal glands and Schirmer's test the production of tears.

Radiological procedures such as sialography and scintigraphy or a salivary gland biopsy may be recommended to aid in the diagnosis of Sjogren’s syndrome [7] [8] [9].

• Leukopenia

  Blood tests may reveal elevated erythrocyte sedimentation rate and transaminase levels, anemia, leukopenia, eosinophilia, hypergammaglobulinemia, presence of antinuclear antibodies, anti–alpha-fodrin antibody and rheumatoid factor. [symptoma.com]

  If we make blood tests, we can see leukopenia, anemia, an increased Erythrocyte sedimentation rate, and some autoantibodies. The autoantibodies are Rheumatoid factors and antinuclear antibodies called SS-A (Ro) and SS-B (La). [youtube.com]

  About 33% of people have a decreased number of red blood cells (anemia), and up to 25% of people have a decreased number of certain types of white blood cells (leukopenia). [msdmanuals.com]

• Neutropenia

  For non-B-cell hematological cancers, prognostic factors included anemia (HR 4.02; p = 0.009), neutropenia (HR 5.86; p = 0.002), thrombocytopenia (HR 4.85; p = 0.004), and cryoglobulins (HR 4.36; p = 0.034). [jhoonline.biomedcentral.com]

  Cytopenia due to drug-induced toxicity, viral infections or haematological disorder other than autoimmunity or lymphoma; Anaemia due to iron or vitamin deficiency; Neutropenia of ethnic origin or due to drug associated agranulocytosis; Thrombocytopenia [rmdopen.bmj.com]

  Methotrexate [87] 10-20mg/week orally or intramuscular Add folate to prevent GI toxic effects Insufficient effect of HCQ on chronic complaints Liver and severe renal failure, severe respiratory failure, alcohol abuse, pregnant or lactating women GI effects, neutropenia [medsci.org]

• Normocytic Anemia

  anemia; thrombocytopenia; mild leukopenia; lymphopenia Biochemical tests, bone marrow Biological [29, 109] 36-62 Hypergammaglobulinemia; hypogammaglobulinemia; hypocomplementia; cryoglobulinemia Serology and biological tests, bone marrow Abbreviations [medsci.org]

• Hyperglobulinemia

  Hyperglobulinemia may lead to the formation of immune complexes with the potential to precipitate in major organs leading to (irreversible) damage [30]. [medsci.org]

• Hyperglobulinemia

  Hyperglobulinemia may lead to the formation of immune complexes with the potential to precipitate in major organs leading to (irreversible) damage [30]. [medsci.org]

• Lymphocytic Infiltrate

  Sjogren's syndrome is a chronic inflammatory disorder characterized by salivary insufficiency and lymphocytic infiltrates in exocrine organs. [symptoma.com]

  The clinical presentation of SjS is heterogeneous and can vary from sicca symptoms to systemic disease (characterized by peri-epithelial lymphocytic infiltration of the affected tissue or the deposition of the immune complex) and lymphoma. [ncbi.nlm.nih.gov]

  Sjögren’s syndrome is characterized by both lymphocytic infiltration of the exocrine glands and B lymphocyte hyperreactivity. [accessmedicine.mhmedical.com]

• HLA-DR3

  We found significantly increased frequency of HLA-DR3 (P less than .001), HLA-DW52a (P less than .001), and HLA-DQA4 (P less than .05), in comparison to normal controls. [ncbi.nlm.nih.gov]

  We found significantly increased frequency of HLA‐DR3 ( P < .001), HLA‐DW52a ( P < .001), and HLA‐DQA4 ( P < .05), in comparison to normal controls. [doi.org]

  Genetic tests can show an association of Sjögrens syndrome with HLA DR3. [youtube.com]

  These "histocompatibility antigens" may include HLA-B8 and HLA-DR3. [lupus.org]

  Genetic associations have been found (eg, HLA-DR3 antigens in White people with primary Sjögren syndrome) but are not necessary for diagnosis or clinical management. [msdmanuals.com]

• HLA-B8

  These "histocompatibility antigens" may include HLA-B8 and HLA-DR3. [lupus.org]

  The association of Sjögren syndrome with human leukocyte antigen (HLA)–B8, HLA-Dw3, HLA-DR3, and the DQA1*0501 allele supports the notion of genetic susceptibility. [16] An active area of research involves genetic polymorphisms of cytokine genes, including [emedicine.com]

• HLA-Dw3

  The association of Sjögren syndrome with human leukocyte antigen (HLA)–B8, HLA-Dw3, HLA-DR3, and the DQA1*0501 allele supports the notion of genetic susceptibility. [16] An active area of research involves genetic polymorphisms of cytokine genes, including [emedicine.com]

Treatment of the disorder is generally symptomatic and supportive. It depends on the manifestations and varies for every patient. Moisturizing treatments, nonsteroidal anti-inflammatory drugs and low-dose glucocorticoids are most commonly used.

The prognosis of primary Sjogren's syndrome is generally good, but for secondary cases, the prognosis is closely related to the associated disease.

Sjogren's syndrome exists as a primary or as a secondary disease. The two forms occur in similar frequency. The secondary form of the disease is associated with disorders such as system lupus erythematosus, polyarteritis nodosa, scleroderma, cryoglobulinemia, and systemic sclerosis. Regardless of whether the syndrome is primary or secondary, the parotid glands, the lungs, eyes, the mouth, the nervous systems, and the skin are affected.

The exact etiology of the disease is currently unknown. So far, it has been established that the pathogenesis is multifactorial. Environmental and hormonal factors are thought to trigger inflammation in individuals with a genetic predisposition to the disorder.

Viruses have been poised as being the number one candidates in possible environmental triggers. Some of the viruses that may play a role include HIV, HTLV-1, human hepersvirus 6, Epstein-Barr virus, cytomegalovirus, and hepatitis C virus [3].

Sjogren's syndrome is ranked as the second most common rheumatologic disorder behind systemic lupus erythematosus. The disorder affects about 0.1% to 4% of the population in the United States. The wide range reflects the lack of a standard diagnosis method [2].

Globally, the disease has shown to remain homogenous among various ethnic groups and environments. The majority of cases occur in individuals above 40 years of age and mainly women are affected [1].

Sjogren's syndrome is a chronic inflammatory disorder characterized by salivary insufficiency and lymphocytic infiltrates in exocrine organs. Even though the mechanism of its pathology has been studied since the discovery, the roles of different populations of immune cells in the pathogenesis remain inconclusive [4] [5].

There are no guidelines for prevention of Sjogren's syndrome.

Sjogren's syndrome is a chronic inflammatory disorder marked by salivary insufficiency and lymphocytic infiltrates in exocrine organs. It is a relatively common disease, although often under-diagnosed.

In 1933, Dr. Henrik Sjögren published an article describing a group of women in a small Swedish town presenting with keratoconjunctivitis, lymphoid infiltrations of the conjunctiva, cornea, lacrimal glands, and parotid glands, a history of arthritis, swelling of the salivary glands, and dryness of the oronasopharynx. Two years later the observation was connected with Mikulicz’s disease and together formed the basis for this syndrome. In 1936, Duke Elder honored Sjögren by naming the disease Sjogren’s syndrome.

The disorder can occur either primary or secondary. Secondary Sjogren's syndrome appears several years after other associated disorders such as scleroderma, rheumatoid arthritis, and primary billiary cirrhosis.

Sjogren's syndrome is common among women above the age of 40 years. The female-to-male ratio is 9:1. There are no geographical differences in the prevalence of Sjogren's syndrome [1].

The disorder is complicated by the fact that there is no sufficiently sensitive or specific test in the diagnosis of Sjogren's syndrome. Moreover, so far there is no known cure for the disease. Symptomatic treatments control the severity of the symptoms associated with the disease.

Once diagnosed with Sjogren's syndrome, the main challenge is not having the disorder, but not knowing what to do next to live positively with it. The good news is that Sjogren’s syndrome does not have to impede your normal lifestyle.

Several remedies are used to treat Sjogren’s syndrome effects. Artificial tears are effective in remedying against dry eye. Tears in normal eyes are drained through tear ducts. Through surgery, these ducts can be sealed with small plugs. Therefore, the little tears formed in the eyes are not drained hence stay for longer. The process is known as punctual occlusion and it is recommended only after the other possible alternatives have failed.

Possible treatments for dry mouth:

• Consumption of more liquid
• Suckling of ice cubes that keeps the mouth moist and lubricated
• Regular rising of the mouth to protect against infections as well as sooth the mouth
• Maintenance of good oral hygiene to prevent tooth decay and gum diseases
• Stop smoking for those who smoke as smoking evaporates the mouth as well as causes an irritation
• Chewing of sugar free gums which stimulates the production of saliva
• Use of saliva substitutes

Vaginal dryness can be treated with lubricants, hormone replacement therapy, and estrogen creams. Joint and muscle aching can be relieved with non-steroidal anti-inflammation drugs. Systemic corticosteroids or immunosuppressive agents have been used for various extraglandular symptoms, such as vasculitis, lung involvement and kidney involvement.

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2. Helmick CG, Felson DT, Lawrence RC, et al. Estimates of the prevalence of arthritis and other rheumatic conditions in the United States. Part I. Arthritis Rheum. Jan 2008;58(1):15-25.
3. Jonsson R, Vogelsang P, Volchenkov R, Espinosa A. The complexity of Sjogren's syndrome: Novel aspects on pathogenesis. Immunol. Lett. 141 2011, 1; 1-9; 9
4. Voulgarelis M, Tzioufas AG. Pathogenetic mechanisms in the initiation and perpetuation of Sjogren's syndrome. Nature Reviews Rheumatology 6 2010, 529-537.
5. Nocturne G, Mariette X. Advances in understanding the pathogenesis of primary Sjögren's syndrome. Nature Reviews Rheumatology 9 2013, 544-556.
6. Tzioufas AG, Voulgarelis M. Update on Sjögren's syndrome autoimmune epithelitis: from classification to increased neoplasias. Best Pract Res Clin Rheumatol. Dec 2007;21(6):989-1010.
7. Montaño-Loza AJ, Crispín-Acuña JC, Remes-Troche JM, Uribe M. Abnormal hepatic biochemistries and clinical liver disease in patients with primary Sjögren's syndrome. Ann Hepatol. Jul-Sep 2007;6(3):150-5.
8. Daniels TE. Labial salivary gland biopsy in Sjögren's syndrome. Assessment as a diagnostic criterion in 362 suspected cases. Arthritis Rheum. Feb 1984;27(2):147-56.
9. García-Carrasco M, Ramos-Casals M, Rosas J, et al. Primary Sjögren syndrome: clinical and immunologic disease patterns in a cohort of 400 patients. Medicine (Baltimore). Jul 2002;81(4):270-80.