Sjögren's syndrome history and symptoms

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Farima Kahe M.D. [2]

Overview

The most common symptoms of Sjögren’s syndrome include ocular and oral symptoms. Patients with Sjögren’s syndrome may have a positive history of rheumatoid arthritis (RA), systemic lupus erythematous (SLE) and non-Hodgkin B-cell lymphoma.

History and Symptoms

The most common symptoms of Sjögren’s syndrome include ocular and oral symptoms.

History

Patients with Sjögren’s syndrome may have positive past and family history of:

Common Symptoms

Common symptoms of Sjögren’s syndrome include:^[1]^[2]^[3]^[4]^[5]^[6]^[7]^[8]^[9]^[10]

Skin manifestations
- Xerosis
- Purpura, associated with vascular or hematologic abnormalities
- Raynaud phenomenon
- Cutaneous vasculitis
- Annular erythema
- Erythema nodosum
- Livedo reticularis
- Lichen planus
- Vitiligo
- Cutaneous amyloidosis
- Granuloma annulare
- Angular cheilitis
- Eyelid dermatitis
Musculoskeletal manifestations
Fatigue and fibromyalgia
Pulmonary symptoms
- Dryness of the tracheobronchial mucosa (xerotrachea)
- Dry cough
- Recurrent bronchitis
- Pneumonitis (infectious or noninfectious)
- Dyspnea from interstitial lung disease
Gastrointestinal manifestations
- Dysphagia
- Nausea
- Dyspepsia and gastritis
- Celiac disease
- Hepatic abnormalities
- subclinical pancreatic disease
Cardiac involvement
- Pericarditis
- Pulmonary hypertension
- Dysfunction of autonomic control of blood pressure
- Myocardial disease
Neurologic and psychiatric symptoms
- Myelopathy
- Optic neuropathy
- Seizures
- Cognitive dysfunction
- Encephalopathy
- Painful sensory neuropathy
- Sensory ataxic neuropathy
- Pure sensory trigeminal neuropathy
- Axonal sensorimotor polyneuropathy
- Mononeuritis multiplex
- Multiple cranial neuropathies
- Radiculoneuropathy
- Autonomic neuropathy
- Depression
Urogenital manifestations
- Interstitial nephritis
- Interstitial cystitis
- Vulvovaginal dryness, pruritus, and dyspareunia
Parotitis
Vasculitis
Hematologic manifestations
- Anemia
- Leukopenia
- Mild thrombocytopenia
Hypergammaglobulinemia and hypogammaglobulinemia
- Monoclonal gammopathy
- Cryoglobulins
Thyroid disease
- Autoimmune thyroiditis

References

↑ Kittridge A, Routhouska SB, Korman NJ (2011). “Dermatologic manifestations of Sjögren syndrome”. J Cutan Med Surg. 15 (1): 8–14. doi:10.2310/7750.2010.09033. PMID 21291650.
↑ Roguedas AM, Misery L, Sassolas B, Le Masson G, Pennec YL, Youinou P (2004). “Cutaneous manifestations of primary Sjögren’s syndrome are underestimated”. Clin. Exp. Rheumatol. 22 (5): 632–6. PMID 15485020.
↑ Baimpa E, Dahabreh IJ, Voulgarelis M, Moutsopoulos HM (September 2009). “Hematologic manifestations and predictors of lymphoma development in primary Sjögren syndrome: clinical and pathophysiologic aspects”. Medicine (Baltimore). 88 (5): 284–93. doi:10.1097/MD.0b013e3181b76ab5. PMID 19745687.
↑ Amezcua-Guerra LM, Hofmann F, Vargas A, Rodriguez-Henriquez P, Solano C, Hernández-Díaz C, Castillo-Martinez D, Ventura-Ríos L, Gutiérrez M, Pineda C (2013). “Joint involvement in primary Sjögren’s syndrome: an ultrasound “target area approach to arthritis““. Biomed Res Int. 2013: 640265. doi:10.1155/2013/640265. PMC 3722787. PMID 23936829.
↑ Kanellopoulos P, Baltoyiannis C, Tzioufas AG (April 2002). “Primary Sjögren’s syndrome associated with inclusion body myositis”. Rheumatology (Oxford). 41 (4): 440–4. PMID 11961175.
↑ Pérez B, Kraus A, López G, Cifuentes M, Alarcón-Segovia D (November 1995). “Autoimmune thyroid disease in primary Sjögren’s syndrome”. Am. J. Med. 99 (5): 480–4. PMID 7485204.
↑ Ramirez-Mata M, Pena Ancira FF, Alarcon-Segovia D (March 1976). “Abnormal esophageal motility in primary Sjögren’s syndrome”. J. Rheumatol. 3 (1): 63–9. PMID 1271390.
↑ Lehrer S, Bogursky E, Yemini M, Kase NG, Birkenfeld A (March 1994). “Gynecologic manifestations of Sjögren’s syndrome”. Am. J. Obstet. Gynecol. 170 (3): 835–7. PMID 8141212.
↑ Binder A, Snaith ML, Isenberg D (August 1988). “Sjögren’s syndrome: a study of its neurological complications”. Br. J. Rheumatol. 27 (4): 275–80. PMID 3261608.
↑ Ramos-Casals M, Font J, Garcia-Carrasco M, Brito MP, Rosas J, Calvo-Alen J, Pallares L, Cervera R, Ingelmo M (July 2002). “Primary Sjögren syndrome: hematologic patterns of disease expression”. Medicine (Baltimore). 81 (4): 281–92. PMID 12169883.

[pmid21291650-1] Kittridge A, Routhouska SB, Korman NJ (2011). “Dermatologic manifestations of Sjögren syndrome”. J Cutan Med Surg. 15 (1): 8–14. doi:10.2310/7750.2010.09033. PMID 21291650.

[pmid15485020-2] Roguedas AM, Misery L, Sassolas B, Le Masson G, Pennec YL, Youinou P (2004). “Cutaneous manifestations of primary Sjögren’s syndrome are underestimated”. Clin. Exp. Rheumatol. 22 (5): 632–6. PMID 15485020.

[pmid19745687-3] Baimpa E, Dahabreh IJ, Voulgarelis M, Moutsopoulos HM (September 2009). “Hematologic manifestations and predictors of lymphoma development in primary Sjögren syndrome: clinical and pathophysiologic aspects”. Medicine (Baltimore). 88 (5): 284–93. doi:10.1097/MD.0b013e3181b76ab5. PMID 19745687.

[pmid23936829-4] Amezcua-Guerra LM, Hofmann F, Vargas A, Rodriguez-Henriquez P, Solano C, Hernández-Díaz C, Castillo-Martinez D, Ventura-Ríos L, Gutiérrez M, Pineda C (2013). “Joint involvement in primary Sjögren’s syndrome: an ultrasound “target area approach to arthritis““. Biomed Res Int. 2013: 640265. doi:10.1155/2013/640265. PMC 3722787. PMID 23936829.

[pmid11961175-5] Kanellopoulos P, Baltoyiannis C, Tzioufas AG (April 2002). “Primary Sjögren’s syndrome associated with inclusion body myositis”. Rheumatology (Oxford). 41 (4): 440–4. PMID 11961175.

[pmid7485204-6] Pérez B, Kraus A, López G, Cifuentes M, Alarcón-Segovia D (November 1995). “Autoimmune thyroid disease in primary Sjögren’s syndrome”. Am. J. Med. 99 (5): 480–4. PMID 7485204.

[pmid1271390-7] Ramirez-Mata M, Pena Ancira FF, Alarcon-Segovia D (March 1976). “Abnormal esophageal motility in primary Sjögren’s syndrome”. J. Rheumatol. 3 (1): 63–9. PMID 1271390.

[pmid8141212-8] Lehrer S, Bogursky E, Yemini M, Kase NG, Birkenfeld A (March 1994). “Gynecologic manifestations of Sjögren’s syndrome”. Am. J. Obstet. Gynecol. 170 (3): 835–7. PMID 8141212.

[pmid3261608-9] Binder A, Snaith ML, Isenberg D (August 1988). “Sjögren’s syndrome: a study of its neurological complications”. Br. J. Rheumatol. 27 (4): 275–80. PMID 3261608.

[pmid12169883-10] Ramos-Casals M, Font J, Garcia-Carrasco M, Brito MP, Rosas J, Calvo-Alen J, Pallares L, Cervera R, Ingelmo M (July 2002). “Primary Sjögren syndrome: hematologic patterns of disease expression”. Medicine (Baltimore). 81 (4): 281–92. PMID 12169883.

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