Sjogrens Syndrome - Clinical features, diagnosis and management
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Venus Williams was admitted to the clinic suffering from dry eyes, dry mouth, dry skin, and enlarged parotid glands. Various tests confirmed she has Sjögren's syndrome, a systemic autoimmune disorder characterized by lymphocytic infiltration of exocrine glands resulting in sicca symptoms. Her positive SSA and SSB antibodies, low unstimulated salivary flow, positive biopsy, and other test results established the diagnosis of Sjögren's syndrome.
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Sjögren's syndrome
Sjögren's syndrome is an autoimmune disease where the immune system attacks and destroys the moisture-producing exocrine glands. It causes dryness of the eyes, mouth, skin and other tissues. It is the second most common autoimmune disease after lupus. While the cause is unknown, it is believed to involve genetic and environmental factors. There is no cure for Sjögren's syndrome, but treatments aim to relieve symptoms like dryness with moisturizers, artificial tears, and other supplements.
Sjogren syndrome
This document provides information on Sjögren's syndrome including its definition, epidemiology, pathogenesis, clinical manifestations, diagnostic criteria, and treatment approaches. Sjögren's syndrome is a chronic autoimmune disease characterized by lymphocytic infiltration of the exocrine glands resulting in dry eyes and dry mouth. It primarily affects middle-aged women and has a prevalence of 0.5-1%. The pathogenesis involves lymphocytic infiltration of the exocrine glands and B lymphocyte hyperreactivity. Clinical manifestations include dry eyes, dry mouth, arthritis, and involvement of other organ systems. Diagnosis is based on symptoms, objective measures of ocular and oral involvement, histopathology, and serology. Treatment focuses on
Sjogren's syndrome
Sjogren's syndrome is an autoimmune disease that causes dryness in the body's moisture-producing glands due to antibodies attacking the glands. It most commonly affects the eyes and mouth, causing dryness, as well as other organs. Complications can include infections, kidney problems, or pancreatitis in rare cases. Symptoms include dry eyes and mouth, fatigue, joint pain, and lung involvement. The condition is diagnosed through tests of tear and saliva production, along with biopsies of affected glands. Treatment focuses on replacing moisture and suppressing the immune system.
sjogren's syndrome
Sjogren's syndrome is an autoimmune disease characterized by dry mouth and dry eyes due to lymphocytic infiltration and destruction of the salivary and lacrimal glands. It was first described in 1933 by Swedish ophthalmologist Henrik Sjogren. The disease causes immune-mediated inflammation of the salivary, lacrimal, and sweat glands. Diagnosis involves evaluating symptoms, signs of dryness, blood tests for autoantibodies, and biopsy of the salivary glands showing lymphocytic infiltration. Treatment focuses on relieving dryness symptoms with artificial tears and saliva, as well as immunosuppressants in severe cases.
Sjogren’s syndrome
Sjogren's syndrome is an autoimmune disease characterized by lymphocytic infiltration and destruction of the exocrine glands, mainly the lacrimal and salivary glands, causing symptoms of dry eyes and dry mouth. It most commonly affects middle-aged women and clinical features include keratoconjunctivitis sicca, xerostomia, and a dry, sticky oral mucosa. Sjogren's syndrome can occur alone as primary Sjogren's or associated with other autoimmune diseases like SLE or RA, known as secondary Sjogren's. Diagnosis involves blood tests for autoantibodies, biopsy of the labial salivary gland, and tests of tear and saliva production
Sjogren's syndrome
This document discusses Sjogren's syndrome, an autoimmune disorder characterized by lymphocytic infiltration of exocrine glands causing dry eyes and mouth. It covers diagnostic criteria including symptoms, objective signs of dryness, autoimmune involvement and biopsy findings. Treatment focuses on managing sicca symptoms through salivary stimulation and substitutes. Rituximab has shown promise in improving dryness symptoms and systemic manifestations in early studies. The risk of lymphoma is higher in Sjogren's patients, especially those with prognostic factors like low C4 levels or purpura.
Sjogren's Syndrome
An Autoimmune Disease : Sjogren's Syndrome, also known as Sicca Syndrome was described by Dr. Henrik Sjogren. It is a triad of Dry eyes, Dry mouth & Rheumatoid Arthritis. The presentation provides a guide to the students regarding the disease including Types, History, Epidemiology, Etiopathogenesis, Clinical features, Systemic & Oral manifestations, Diagostic criteria, Histopathological features, Serological findings, Radiography & Salivary gland imaging (involving sialography, scintigraphy, sonography & MRI), its Treatment along with the advancements in treatment, Complications & Prognosis.
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Bullous Pemphigoid
1) The patient, a 45-year-old female, presented with multiple fluid-filled itchy lesions over her limbs and face for the past week and developed oral lesions 3 days after admission.
2) A clinical diagnosis of bullous pemphigoid was made based on tense fluid-filled cutaneous lesions, involvement of oral mucosa, histopathology showing subepidermal blistering, and direct immunofluorescence demonstrating linear deposition of IgG and C3 along the basement membrane zone.
3) The patient was treated with systemic corticosteroids and immunosuppressants along with topical corticosteroids for oral lesions and advised long-term follow-up to manage the chronic autoimmune condition.
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Sjögren's syndrome
Sjögren's syndrome is an autoimmune disease where the immune system attacks and destroys the moisture-producing exocrine glands. It causes dryness of the eyes, mouth, skin and other tissues. It is the second most common autoimmune disease after lupus. While the cause is unknown, it is believed to involve genetic and environmental factors. There is no cure for Sjögren's syndrome, but treatments aim to relieve symptoms like dryness with moisturizers, artificial tears, and other supplements.
Sjogren syndrome
This document provides information on Sjögren's syndrome including its definition, epidemiology, pathogenesis, clinical manifestations, diagnostic criteria, and treatment approaches. Sjögren's syndrome is a chronic autoimmune disease characterized by lymphocytic infiltration of the exocrine glands resulting in dry eyes and dry mouth. It primarily affects middle-aged women and has a prevalence of 0.5-1%. The pathogenesis involves lymphocytic infiltration of the exocrine glands and B lymphocyte hyperreactivity. Clinical manifestations include dry eyes, dry mouth, arthritis, and involvement of other organ systems. Diagnosis is based on symptoms, objective measures of ocular and oral involvement, histopathology, and serology. Treatment focuses on
Sjogren's syndrome
Sjogren's syndrome is an autoimmune disease that causes dryness in the body's moisture-producing glands due to antibodies attacking the glands. It most commonly affects the eyes and mouth, causing dryness, as well as other organs. Complications can include infections, kidney problems, or pancreatitis in rare cases. Symptoms include dry eyes and mouth, fatigue, joint pain, and lung involvement. The condition is diagnosed through tests of tear and saliva production, along with biopsies of affected glands. Treatment focuses on replacing moisture and suppressing the immune system.
sjogren's syndrome
Sjogren's syndrome is an autoimmune disease characterized by dry mouth and dry eyes due to lymphocytic infiltration and destruction of the salivary and lacrimal glands. It was first described in 1933 by Swedish ophthalmologist Henrik Sjogren. The disease causes immune-mediated inflammation of the salivary, lacrimal, and sweat glands. Diagnosis involves evaluating symptoms, signs of dryness, blood tests for autoantibodies, and biopsy of the salivary glands showing lymphocytic infiltration. Treatment focuses on relieving dryness symptoms with artificial tears and saliva, as well as immunosuppressants in severe cases.
Sjogren’s syndrome
Sjogren's syndrome is an autoimmune disease characterized by lymphocytic infiltration and destruction of the exocrine glands, mainly the lacrimal and salivary glands, causing symptoms of dry eyes and dry mouth. It most commonly affects middle-aged women and clinical features include keratoconjunctivitis sicca, xerostomia, and a dry, sticky oral mucosa. Sjogren's syndrome can occur alone as primary Sjogren's or associated with other autoimmune diseases like SLE or RA, known as secondary Sjogren's. Diagnosis involves blood tests for autoantibodies, biopsy of the labial salivary gland, and tests of tear and saliva production
Sjogren's syndrome
This document discusses Sjogren's syndrome, an autoimmune disorder characterized by lymphocytic infiltration of exocrine glands causing dry eyes and mouth. It covers diagnostic criteria including symptoms, objective signs of dryness, autoimmune involvement and biopsy findings. Treatment focuses on managing sicca symptoms through salivary stimulation and substitutes. Rituximab has shown promise in improving dryness symptoms and systemic manifestations in early studies. The risk of lymphoma is higher in Sjogren's patients, especially those with prognostic factors like low C4 levels or purpura.
Sjogren's Syndrome
An Autoimmune Disease : Sjogren's Syndrome, also known as Sicca Syndrome was described by Dr. Henrik Sjogren. It is a triad of Dry eyes, Dry mouth & Rheumatoid Arthritis. The presentation provides a guide to the students regarding the disease including Types, History, Epidemiology, Etiopathogenesis, Clinical features, Systemic & Oral manifestations, Diagostic criteria, Histopathological features, Serological findings, Radiography & Salivary gland imaging (involving sialography, scintigraphy, sonography & MRI), its Treatment along with the advancements in treatment, Complications & Prognosis.
Presentation by - Dr.Harsimran Singh Kapoor
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1) The patient, a 45-year-old female, presented with multiple fluid-filled itchy lesions over her limbs and face for the past week and developed oral lesions 3 days after admission.
2) A clinical diagnosis of bullous pemphigoid was made based on tense fluid-filled cutaneous lesions, involvement of oral mucosa, histopathology showing subepidermal blistering, and direct immunofluorescence demonstrating linear deposition of IgG and C3 along the basement membrane zone.
3) The patient was treated with systemic corticosteroids and immunosuppressants along with topical corticosteroids for oral lesions and advised long-term follow-up to manage the chronic autoimmune condition.
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Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, is a rare autoimmune disease characterized by inflammation of blood vessels. It commonly involves the upper and lower respiratory tracts and kidneys. The disease was first described in 1931 and recognized as a distinct clinical entity in 1936. Common symptoms include nasal inflammation, sinusitis, lung nodules, alveolar hemorrhage, and necrotizing glomerulonephritis. Diagnosis involves meeting criteria established by the American College of Rheumatology or the 2012 revised Chapel Hill criteria through clinical evaluation, biopsy, and ANCA testing. Treatment involves immunosuppressive medications.
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- Sarcoidosis is suspected based on the clinical findings and biopsy results. Sarcoidosis is a multisystem inflammatory disease characterized by the formation of noncaseating granulomas that can affect multiple organ systems, most commonly the lungs.
- Further workup and management are needed to evaluate organ involvement and determine treatment for the patient's sarcoidosis.
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Sjogren syndrome by aseem
Sjögren's syndrome is an autoimmune disease that causes inflammation of the exocrine glands, most commonly the salivary and lacrimal glands, leading to dry eyes and dry mouth. It exists as either a primary form that occurs alone or a secondary form associated with other connective tissue diseases like rheumatoid arthritis. Diagnosis involves evaluating symptoms of dry eyes and dry mouth along with tests like salivary gland biopsy, salivary flow tests, and lab tests for autoantibodies. Treatment focuses on managing symptoms while complications can involve other organ systems.
SJOGREN'S SYNDROME
Venus Williams, a 32-year-old African American female professional tennis player, presented with symptoms of dry eyes, dry mouth, dry skin, and enlarged parotid glands. Laboratory tests confirmed Sjogren's syndrome with a high ESR, positive biopsy and antibody results, and abnormal salivary gland function tests. Sjogren's syndrome is an autoimmune disease characterized by lymphocytic infiltration of exocrine glands causing sicca symptoms. Treatment focuses on symptom management with artificial tears, moisturizers, NSAIDs, and immunosuppressants for major organ involvement.
A Case of Sjogren's Syndrome
The patient is a 60-year-old female who presented with a 2-year history of difficulty swallowing solid foods and dryness of the mouth and eyes. Various tests were performed and she was found to have Sjogren's syndrome based on positive labial biopsy and serum autoantibodies. Sjogren's syndrome is a chronic autoimmune disease characterized by lymphocytic infiltration of the exocrine glands, predominantly affecting middle-aged women. The patient's symptoms, test results, and biopsy findings were consistent with a diagnosis of primary Sjogren's syndrome.
Sjogren's syndrome
Brief presentation about sjogren's syndrome
Brief presentation about sjogren's syndrome
Brief presentation about sjogren's syndrome
Brief presentation about sjogren's syndrome
Brief presentation about sjogren's syndrome
Brief presentation about sjogren's syndrome
Brief presentation about sjogren's syndrome
Brief presentation about sjogren's syndrome
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- There is a spectrum of clinical forms from tuberculoid (high resistance) to lepromatous (low resistance).
- Complications include reactions, nerve damage leading to deformities, and secondary infections.
- Treatment involves multidrug therapy but nerve damage may be permanent without rehabilitation. Advocacy groups continue working to eliminate stigma and provide care.
all about behecets syndrome in detail elaborted
X-RAY LEFT MASTOIDS
• Partial loss of left mastoid pneumatisation likely due to mastoiditis. . No evidence of sclerosis noted on left side.
• Visualized skull vault is normal with no evidence of any trauma or bone lesion.
SUGGESTS:
• Partial loss of left mastoid pneumatisation likely due to mastoiditis. . No evidence of sclerosis noted on left side.
sjogren's syndrome clinical features,diagnosis,management
Sjogren's syndrome is a chronic autoimmune disease characterized by dryness of the eyes and mouth due to lymphocytic infiltration and destruction of the salivary and lacrimal glands. It can occur alone as primary Sjogren's syndrome or associated with other connective tissue diseases as secondary Sjogren's syndrome. Diagnosis involves testing for ocular and oral symptoms, signs on Schirmer's test and Rose Bengal scoring, salivary gland biopsy, and autoantibodies. Treatment focuses on managing dryness symptoms through lubricating eye drops and artificial saliva, as well as medications like hydroxychloroquine.
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- A 41-year-old African American woman presents with a rash on her arm and worsening asthma symptoms over the past month. Biopsy of the rash shows noncaseating granulomas, and chest imaging reveals bilateral interstitial lung disease.
- Sarcoidosis is suspected based on the clinical findings and biopsy results. Sarcoidosis is a multisystem inflammatory disease characterized by the formation of noncaseating granulomas that can affect multiple organ systems, most commonly the lungs.
- Further workup and management are needed to evaluate organ involvement and determine treatment for the patient's sarcoidosis.
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Ramsay Hunt syndrome is caused by reactivation of the varicella zoster virus, which causes chickenpox, in the facial nerve ganglia near the ear. This causes a rash around one ear and facial paralysis on the same side. It affects cranial nerves 7 and 8, resulting in symptoms like facial weakness, hearing loss, and pain. Treatment involves antiviral drugs to treat the infection along with steroids to reduce inflammation. Prognosis is better if treatment begins within 3 days and for children compared to adults. Early diagnosis and treatment improves chances of recovery from facial paralysis.
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This document discusses aphthous ulcers, also known as canker sores, which are characterized by recurring, painful ulcers in the mouth. It describes the three main types: minor aphthous ulcers, which are less than 10mm and heal within 2 weeks; major aphthous ulcers, which are larger than 10mm and can take over 6 weeks to heal, often leaving scars; and herpetiform ulcers, which present as multiple small ulcers that may join together into larger ones and persist for 1-3 years. The document provides details on symptoms, locations in the mouth, appearances, durations, and histopathology of the different types of aphthous ulcers.
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Sjögren's syndrome is an autoimmune disease that causes inflammation of the exocrine glands, most commonly the salivary and lacrimal glands, leading to dry eyes and dry mouth. It exists as either a primary form that occurs alone or a secondary form associated with other connective tissue diseases like rheumatoid arthritis. Diagnosis involves evaluating symptoms of dry eyes and dry mouth along with tests like salivary gland biopsy, salivary flow tests, and lab tests for autoantibodies. Treatment focuses on managing symptoms while complications can involve other organ systems.
SJOGREN'S SYNDROME
Venus Williams, a 32-year-old African American female professional tennis player, presented with symptoms of dry eyes, dry mouth, dry skin, and enlarged parotid glands. Laboratory tests confirmed Sjogren's syndrome with a high ESR, positive biopsy and antibody results, and abnormal salivary gland function tests. Sjogren's syndrome is an autoimmune disease characterized by lymphocytic infiltration of exocrine glands causing sicca symptoms. Treatment focuses on symptom management with artificial tears, moisturizers, NSAIDs, and immunosuppressants for major organ involvement.
A Case of Sjogren's Syndrome
The patient is a 60-year-old female who presented with a 2-year history of difficulty swallowing solid foods and dryness of the mouth and eyes. Various tests were performed and she was found to have Sjogren's syndrome based on positive labial biopsy and serum autoantibodies. Sjogren's syndrome is a chronic autoimmune disease characterized by lymphocytic infiltration of the exocrine glands, predominantly affecting middle-aged women. The patient's symptoms, test results, and biopsy findings were consistent with a diagnosis of primary Sjogren's syndrome.
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Brief presentation about sjogren's syndrome
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- Junctional nevi are located at the dermal-epidermal junction and appear as flat brown-black macules, most often on the hard palate or gingiva.
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- It is caused by Mycobacterium leprae bacteria and primarily affects the skin, nerves, respiratory tract and testes.
- There is a spectrum of clinical forms from tuberculoid (high resistance) to lepromatous (low resistance).
- Complications include reactions, nerve damage leading to deformities, and secondary infections.
- Treatment involves multidrug therapy but nerve damage may be permanent without rehabilitation. Advocacy groups continue working to eliminate stigma and provide care.
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Similar to Sjogrens Syndrome - Clinical features, diagnosis and management
all about behecets syndrome in detail elaborted
X-RAY LEFT MASTOIDS
• Partial loss of left mastoid pneumatisation likely due to mastoiditis. . No evidence of sclerosis noted on left side.
• Visualized skull vault is normal with no evidence of any trauma or bone lesion.
SUGGESTS:
• Partial loss of left mastoid pneumatisation likely due to mastoiditis. . No evidence of sclerosis noted on left side.
sjogren's syndrome clinical features,diagnosis,management
Sjogren's syndrome is a chronic autoimmune disease characterized by dryness of the eyes and mouth due to lymphocytic infiltration and destruction of the salivary and lacrimal glands. It can occur alone as primary Sjogren's syndrome or associated with other connective tissue diseases as secondary Sjogren's syndrome. Diagnosis involves testing for ocular and oral symptoms, signs on Schirmer's test and Rose Bengal scoring, salivary gland biopsy, and autoantibodies. Treatment focuses on managing dryness symptoms through lubricating eye drops and artificial saliva, as well as medications like hydroxychloroquine.
Sjogrens
Sjogren's syndrome is an autoimmune disease characterized by lymphocytic infiltration of the exocrine glands resulting in dry eyes and mouth. It predominantly affects middle-aged women. Approximately one-third of patients experience systemic manifestations such as arthritis, lung involvement, or vasculitis. Pulmonary manifestations include airway disease, interstitial lung disease such as nonspecific interstitial pneumonia, lymphocytic interstitial pneumonitis, and usual interstitial pneumonia. Treatment involves managing dryness symptoms and suppressing inflammation.
Sjögren syndrome
Sjögren syndrome primarily affects women in their 40s-50s and is characterized by dry eyes and dry mouth due to reduced tear and saliva production. It has a variety of systemic manifestations involving organs like lungs, kidneys, blood vessels, and nerves. Diagnosis involves tests of tear and saliva function along with labial gland biopsy showing lymphocytic infiltrates. Treatment focuses on symptom relief and immunosuppression for severe extraglandular disease.
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1. Sjögren's syndrome is an autoimmune disease that primarily affects the lacrimal and salivary glands, causing dry eyes and dry mouth.
2. It most commonly affects women in the fourth to fifth decade of life, with a female to male ratio of approximately 9:1.
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- Gorlin-Goltz syndrome (basal cell nevus syndrome), which causes multiple nevoid basal cell carcinomas and multiple jaw cysts appearing as radiolucencies.
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Sjogren’s Syndrome
Sjögren’s syndrome is an autoimmune disease in which our own body immune system attacks glands that produce moisture. The misdirected immune system leads to inflammation of tissues and dysfunction in glands of the body that are generating tears and saliva.
Sjogren syndrome affects lacrimal glands which produce tears and salivary glands which produce saliva in the mouth. It causes extreme dryness of eyes and mouth.
Bells palsy..............................
Carpal tunnel syndrome results from compression of the median nerve at the wrist. It can be caused by aberrant anatomy, infections, inflammatory conditions, or metabolic diseases that increase the volume in the carpal canal. Symptoms include sensory disturbance, weakness in thumb abduction, and thenar atrophy. Tinel's test involves tapping over the median nerve to elicit tingling in the median nerve distribution.
Bell's palsy is an acute facial paralysis attributed to inflammation of the facial nerve within the stylomastoid foramen. It has various potential causes including trauma, infections, metabolic issues, and idiopathic factors. Clinical features include pain behind the ear, inability to close the eye or raise the eyeb
Sjogrens's Syndrome
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Downs
This document provides details on the case of an 8 month old female child named Azma who presented with recurrent cold, cough, loose motions, and rapid breathing. It summarizes her birth history, developmental milestones, family history, clinical features, genetic causes, common health issues, diagnostic criteria, management, and prognosis of Down syndrome.
Salivary glands
This document discusses the salivary glands, including their anatomy and common disorders. It describes the major salivary glands - parotid, submandibular and sublingual glands, as well as the 800 minor salivary glands in the oral cavity. Common disorders of the minor salivary glands include cysts and tumors. Inflammatory disorders of the major salivary glands like sialadenitis are also discussed. Specific conditions involving each gland like sialolithiasis of the submandibular gland, ranula of the sublingual gland and Sjogren's syndrome are explained. The document concludes with discussing xerostomia, sialorrhea
Dermatology 5th year, 5th lecture (Dr. Kazhan)
Connective tissue disorders involve inflammation of connective tissue that can lead to skin changes like hardening or scarring as well as arthritis and other organ abnormalities. Lupus is an autoimmune disease where the immune system attacks the body's own tissues, affecting women more than men. It can cause a butterfly-shaped rash on the face, mouth sores, and inflammation of organs. Discoid lupus causes red scaly patches on the skin, especially the face, that can lead to scarring and pigment changes. Sjogren's syndrome destroys exocrine glands causing dry eyes and mouth. It is associated with rheumatoid arthritis and has a higher risk of lymphoma. Behcet's disease involves recurring
داون
Down syndrome is a genetic disorder caused by trisomy 21, occurring in approximately 1 in 1000 live births. It is characterized by intellectual disability and distinctive facial features such as a flat nasal bridge and upward slanting eyes. Individuals with Down syndrome often experience certain health problems as well, including heart defects, gastrointestinal abnormalities, hearing or vision issues. Proper management includes ongoing screening and treatment to address common medical comorbidities. With improved healthcare, the average life expectancy for individuals with Down syndrome has risen significantly in recent decades.
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Sjogrens Syndrome - Clinical features, diagnosis and management
- 2. Venus Williams, is an American professional tennis player who is a former World No. 1.She is 32-year-old, African-American female. Was admitted to your clinic suffering severe xerophthalmia (dry eyes), xerostomia (dry mouth), xeroderma and parotid gland enlargement . The erythrocyte sedimentation rate (ESR) was 105 mm/h (N:0-10 mm/h).She complained from severe fatigue episodes while training and playing. Some tests were performed and the results were as shown below: Schirmer's test : <4 mm wetting of the paper after 5 minutes . N: ≥15-10 mm after 5 minutes Positive salivary gland biopsy findings. Positive anti–SSA & anti–SSB antibodies results. unstimulated salivary flow < 1.5mL in 15min N: 0.3 - 0.4 ml/min (5.25 ml in 15 min)
- 3. Venus Williams is suffering from: Sjögren's Syndrome. Overview ) Sjögren's syndrome(S.S ) is a systemic, chronic, autoimmune, inflammatory disorder. Characterized by lymphocytic infiltrates in exocrine organs. Most individuals with Sjögren syndrome present with sicca symptoms. In addition, numerous extraglandular features may develop.
- 4. Primary Sjögren syndrome - absence of another underlying rheumatic disorder, Secondary Sjögren syndrome - associated with another underlying rheumatic disease : (SLE), rheumatoid arthritis (RA), scleroderma.
- 5. Female : male = 9:1 Middle age m/c , can occur at any age Prevalence : Primary 0.5 – 1% Secondary – 30% of pts with autoimmune rheumatic diseases
- 7. Etiopathgenesis Environmental factors ( viral / other infn ) trigger inflammation Infiltration of glandular tissue by CD4+T lymphocytes Glandular cells express high levels of HLA-DR autoantigens to CD4+T lymphocytes Inflammatory cascade : cytokine prodn ( IFN γ , IL-2) Also B cell activation
- 8. Prognosis Sjögren syndrome carries a generally good prognosis. Morbidity associated with Sjögren syndrome is mainly associated with the gradually decreased function of exocrine organs. Patients with primary Sjögren syndrome have a normal life expectancy. Children born to mothers with antibodies against SSA/Ro are at an increased risk of neonatal lupus and congenital heart block.
- 9. Clinical Manifestations Majority – symptoms due to diminished lacrimal + salivary function Primary SS – slow benign course Initially – mucosal / nonspecific dryness 8 – 10 yr from initial symptoms to full blown disease
- 10. Xerostomia Principal oral symptom Difficulty in swallowing dry food, increase in caries, inability to speak continuously O/E – dry erythematous sticky oral mucosa Atrophy of filiform papillae Saliva – not expressable/ cloudy Parotid / major salivary gland enlarged – 2/3rd of patients with primary SS, uncommon in secondary SS
- 11. Diagnosis : Sialometry , sialography , scintigraphy USG , MRI of the glands Biopsy – confirmatory -> focal lymphocytic infiltrates
- 12. Ocular involvement Sandy/ gritty feeling under eyelids Burning, accumulation of secretions in thick strands at inner canthi Decreased tearing Redness Itching Eye fatigue Increased photosensitivity Collectively called keratoconjunctivitis sicca Destruction of corneal + bulbar conj epithelium
- 13. Diagnosis Schirmer I test – measurement of tear flow Determination of tear composition Assessment of tear lysozyme content Slit lamp – rose bengal stain - > punctate corneal ulcerations and attachments of corneal epithelium
- 14. Other exocrine involvement Decreased secretions in upper + lower resp tree dry nose, throat , trachea GIT – esophageal mucosal atrophy, atrophic gastritis Subclinical pancreatitis Dyspareunia – dryness of external genitalia Dry skin
Editor's Notes
- aExclusion criteria: past head and neck radiation treatment, hepatitis C infection, AIDS, preexisting lymphoma, sarcoidosis, graft-versus-host disease, use of anticholinergic drugs. bPrimary Sjögren’s syndrome: any four of the six items, as long as item IV (histopathology) or VI (serology) is positive; or any three of the four objective-criteria items (III, IV, V, VI). cIn patients with a potentially associated disease (e.g., another well-defined connective tissue disease), the presence of item I or item II plus any two from among items III, IV, and V may be considered indicative of secondary Sjögren’s syndrome.