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Autoimmunity Case Study

Project Overview

Project Description

Create a case study on the autoimmune disease that your group has been assigned.  Your case study could be based on a real one that you find in the medical literature, or on the web. Alternatively, your case could be a hypothetical one that you have created in order to illustrate some important medical concepts. The basic format of the case is as follows:

The Introduction or Background section should briefly explain the background of the disorder and its clinical presentation. This section is meant to give an introduction to the case report from the standpoint of those without specialist knowledge in the area, clearly explaining the background of the topic. This section should be no longer than 100 words.

The Case Presentation section should present all relevant details concerning the case including a description of the patient’s relevant demographic information; any relevant medical history of the patient; the patient's symptoms and signs; any tests that were carried out and a description of any treatment or intervention. This section may be broken into subsections with appropriate subheadings using Scholar’s structure tool (Creator => About this Work => Structure, see Tutorial 3.5 in the Help area, link in the top right of the screen). Images or other visual media (e.g. videos) are encouraged.

The case presentation should be described in a concise and chronological order. One should usually begin with the primary complaint, salient history (including significant family, occupational, and other social history along with any significant medications taken or allergies), followed by the physical examination, starting with the vital signs presented at the examination, along with pertinent investigations and results. There should be enough detail (but not too much) for the reader to establish his or her own conclusions about the validity. It should contain only pertinent information and nothing superfluous or confusing. This section should be no longer than 300 words.

This Patient’s Perspective section is an opportunity to add a description of a case from the patient’s perspective. This section might include what originally made the patient seek medical advice, a description of their symptoms from their perspective, whether the symptoms were better or worse at different times, how tests and treatments affected them, and how the problem is now. As medicine becomes more person-centered, the voice of the individual patient becomes even more important, both to assist in clinical decision making, and for medical education. This section should be no longer than 200 words.

This Discussion section should state clearly the main conclusions of the case report and give a clear explanation of their importance and relevance. Information should be included on how the case is typical or atypical of a particular disease etiology or treatment. Most importantly, this section should briefly describe the immunopathologic basis of the disease and how this might drive the symptoms, clinical presentation and rationale for the treatment. Images/diagrams are encouraged. New scientific or clinical information that is emerging to aid current and future physicians in the diagnosis and treatment can also be included here. This section should be no longer than 200 words.

How to Create this Work

This is going to be a jointly created work which should be split among different members of the group. The Case Presentation section (see above) represents the foundation which should be developed first since the other sections build off of this. The following are some important instructions on how to create and work in a jointly created work.

  1. Everyone will receive a work request for this project. One person in the group should take the link, then go to Creator => About This Work => Creators and invite the other members to be co-creators. Now, everyone will be working in the same space.
  2. Scholar only allows one person to work in an element (or section) at a time. When another person is working in an element, it is locked to the co-creators. So in order to have multiple people working at the same time, we suggest that an early task should be to go to the Structure tool to create subheadings and sections (Creator => About this Work => Structure, see Tutorial 3.5 in Help).
  3. To discuss the work among the co-creators, use the Dialogue tool: Creator => Project => Dialogue.
Icon for Sjögren’s Syndrome

Sjögren’s Syndrome

Introduction

Introduction

Sjögren’s syndrome is a chronic multisystem inflammatory disorder characterized by lymphocytic infiltration of the lacrimal and salivary glands which can result in dryness of the mouth, eyes, nasal passages, skin, vagina and also cause fever and malaise.

Elements of both innate and adaptive immunity are at play here, as biopsies of affected glandular and nonglandular tissue show WBC infiltrates and immunohistologic interactions. SS is associated with many other pathologies and autoimmune conditions and must be examined holistically. 

Case Presentation

A 55 year old female presents to the hospital complaining of dryness of mouth for a year, and dryness of eyes for 6 years. She states that her dry eyes often have a burning and itchy sensation throughout the day, and her dry mouth makes it difficult to swallow dry and solid food. The dryness has not progressed, but is persistent. She feels the need to drink water often, which often results in her waking up at night to use the bathroom. Additionally she has difficulty opening both eyes in the morning. She has no history of vomiting, hoarseness of voice, regurgitation of food.  

The physician performed a physical exam and upon palpation found bilateral parotid gland enlargement. She had a normal blood pressure, respiration rate, and pulse, but had a fever of 100.4 F.

Figure 1

The physician also performed Schirmer tear test, inserting a small piece of filter paper under her lower eyelid to measure tear production. Her test was abnormal with only 3.5mm of the strip in the right eye and 1.5mm of the strip in the left eye became wet. This confirmed that the patient was not producing enough tears to keep her eyes moist.

Ultrasonography showed bilateral parotid gland enlargement, and sialography (X-ray with contrast) shows diffuse sialectasis (cystic dilation of the ducts of salivary glands).

Figure 2: Sialography showing sialectasis

Serum immunoglobulin was SS-A/RO positive for Sjogren’s syndrome and she had an elevated erythrocyte sedimentation rate (ESR) of 42mm/h, signifying higher than normal inflammatory activity. Lip biopsy (small sliver of salivary glands) was done, which indicated focal lymphocytic sialoadenitis (inflammation of salivary glands). Biopsy showed more than one focus of lymphocytes. It had tight clumps of lymphocytes next to normal gland tissue.

Figure 3: Histopathology of the minor salivary gland in Sjögren's syndrome. The degree of lymphocytic infiltration varies from moderate (A) to diffuse (B). In the most severe forms, germinal center formation can be observed (C). F = lymphocytic focus; GC = germinal center.

The physician suggested using eyedrops, having a humidifier at home, and prescribed Salagen (Pilocarpine) to help increase the production of saliva and a low dose of corticosteroids. Antibiotics and analgesics were given to help with her parotid gland enlargement as well.

Patient Perspective

Symptoms experienced by Sjogren’s patients are severely limiting factors on personal, social, and work life. Patients often feel a sense of a profound loss of control.

Patients may try to plan around the expected, chronic, debilitating fatigue, but its onset may be sudden. Patients may have to give up preferred extracurricular activities. In some instances, they lose control of their appearance, for example, being forced to switch from contacts to glasses because of dry eyes. Dry eye complications also affect reading, driving, and other activities that require sustained visual attention. Many will experience the downside of oral issues such as tooth decay and dry mouth.

Sjogren’s impacts not just patients, but everyone in their families. Playing with children and assisting around the house is affected. A desire to do more with loved ones is at odds with the inability to do anything but rest and recover.

Limitations extend to the workplace. Patients may struggle with unemployment or need to change jobs to avoid physical activity. Those who work must cope with pain.

Compounding this frustration is that patients often do not appear sick. Furthermore, the disease itself is hard to diagnose, leading to misdiagnosis and years of suffering without relief.

Discussion

Sjögren’s syndrome is a chronic multisystem inflammatory disorder characterized by lymphocytic infiltration of the lacrimal and salivary glands which can result in dryness of the mouth, eyes, nasal passages, skin, vagina and also cause fever and malaise.

Figure 3 (above) describes the histopathology of the minor salivary gland in Sjögren's syndrome. The degree of lymphocytic infiltration varies from moderate (A) to diffuse (B). In the most severe forms, germinal center formation can be observed (C). F = lymphocytic focus; GC = germinal center. The purple staining cells are the lymphocytic or white blood cell infiltrates (abnormal). The most remarkable feature is the development of these germinal centers (GC) which means that the white blood cells are actually proliferating, or being produced, in the salivary gland. 

Standard treatment involves both relieving localized symptoms as well as therapies that address systemic presentation.

  • Routine dental care is needed to prevent dental caries as saliva can normally help to clear food residue and bacteria off of teeth.
  • Prevent dryness by maintaining good hydration, avoidance of acidic drinks (carbonated beverages and juices), mouth breathing, medications that worsen oral dryness, low humidity environments, and oral irritants like tobacco, alcohol, coffee, and smoke.
  • Suck on sugar free candies or lozenges to stimulate saliva production.
  • Saliva substitutes.
  • Use of muscarinic agonists (Pilocarpine or Cevimeline) in patients with adequate response to topical stimulants to increase saliva production. Side effects include excessive sweating, increased urinary frequency, flushing, chills, rhinitis, nausea, and diarrhea.
  • Systemic anti-inflammatory and immunosuppressive medications, such as hydroxychloroquine and rituximab have mixed results.

References

Citations for Case Presentation:

Chapel, H., Haeney, M., Misbah, S. A., & Snowden, N. (2014). Essentials of clinical immunology (5th ed.). Retrieved from http://www.immunologyclinic.com/case.asp?chap=10&case=11

Illei G, Danielides S. Chapter 161. Sjögren's Syndrome. In: Goldsmith LA, Katz SI, Gilchrest BA, Paller AS, Leffell DJ, Wolff K. eds. Fitzpatrick's Dermatology in General Medicine, 8e New York, NY: McGraw-Hill; 2012. http://accessmedicine.mhmedical.com.proxy.cc.uic.edu/content.aspx?bookid=392&sectionid=41138885. Accessed February 23, 2017.

Indian Journal of Dental Research. (n.d.). Sialogram [Digital image]. Retrieved February 23, 2017, from http://www.ijdr.in/viewimage.asp?img=IndianJDentRes_2009_20_4_499_59449_u1.jpg

Jadhav S, Jadhav A, Thopte S, et al. Sjögren's Syndrome: A Case Study. J Int Oral Health. 2015;7(3):72-4.

Salivary Glands [Digital image]. (2017, February 25). Retrieved March 1, 2017, from https://static1.squarespace.com/static/5681a0e2d8af1011fc075fca/t/5685558ed82d5eb43267b90a/1451578776306

Sjögren's Syndrome Information. (n.d.). Retrieved February 23, 2017, from https://www.hopkinssjogrens.org/disease-information/diagnosis-sjogrens-syndrome/labial-gland-lip-biopsy/

Sjogren's syndrome: Lifestyle and home remedies. (2014, July 8). Retrieved February 23, 2017, from http://www.mayoclinic.org/diseases-conditions/sjogrens-syndrome/basics/lifestyle-home-remedies/con-20020275

 

Citations for Patient Perspective:

Barrow K. Patient Voices: Sjogren’s Syndrome. New York Times. September 15, 2011. http://www.nytimes.com/interactive/2011/09/16/health/healthguide/sjogrens_syndrome.html?_r=0. Accessed February 22, 2017.

Michael D. Turner, DDS, MD1, Jonathan A. Ship, DMD, FDS RCS. September, 2007. “Dry Mouth and Its Effects on the Oral Health of Elderly People.” JADA. http://jada.ada.org/article/S0002-8177(14)62738-0/abstract.

Uchino, M., & Schaumberg, D. A. (2013). Dry Eye Disease: Impact on Quality of Life and Vision. Current Ophthalmology Reports, 1(2), 51-57. http://doi.org/10.1007/s40135-013-009-1.