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Connective Tissue Diseases (CTDs) Patient Management

After a diagnosis of CTD has been made through detailed patient history, serological testing, and clinical observations—long term management of your patient’s disease state will depend largely on their unique mix of symptoms. That plan can include a variety of approaches, depending on the disease or diseases present. CTDs affect the joints with varying degrees of severity.

The goals of any management plan are to maintain joint function and reduce symptoms. Diagnostic testing can help you develop a targeted plan that addresses all present diseases, improves your patient’s quality of life, and maintains mobility.

Prognosis

CTDs are complex diseases with variable presentations, courses and prognosis. Management of patient’s presenting indicative symptoms of CTDs should be tailored to the individual symptoms presented, and in accordance with the organ systems affected.

The prognosis of patients diagnosed with CTDs can vary between diseases, from a benign course to severe disease progression. A patient may also present with more than one CTD, a factor that is critical in determining what his or her prognosis may be long term.
 

  • Sjögren's Syndrome: The prognosis of SS may be contingent upon risk factors such as the presence of life threatening vasculitis, or B-cell lymphoma.1
  • SLE: SLE has a highly variable clinical course. Morbidity and mortality in SLE patients is considerable despite immunosuppressive therapies. Due to an increased risk of poor outcomes, pregnancy may be discouraged in patients who are planning to become pregnant.2
  • Systemic Sclerosis (SSc): The presence of cutaneous manifestations is critical in deciding upon management of SSc patients—the level of skin involvement will dictate how rapidly the disease progresses. Pregnancy may be discouraged in patients who are planning to become pregnant due to increased risk for poor outcomes. 3
  • Polymyositis/Dermatomyositis: Dermatomyositis and polymyositis are serious diseases that carry with them a disease mortality rate of up to 10%. These diseases have a major impact on quality of life, despite the potential for regained muscle strength.4
  • Mixed Connective Tissue Diseases (MCTDs): outlook for patients with MCTDs varies contingent upon which organ systems are affected. Patients who are treated early with targeted therapies and subsequently monitored may have a better projected prognosis.5


Patients with a confirmed diagnosis of one or multiple CTDs, who are pregnant or planning to become pregnant carry with them additional risk factors in terms of long term prognosis. Pregnancy can cause symptom flares, which need to be accounted for during treatment to ensure healthy gestation and optimal outcomes.6

Overlapping Disorders: Understanding Comorbidities

As with many autoimmune diseases, CTDs are associated with a variety of comorbid conditions. When making a diagnosis of CTD it is important to consider, monitor and exclude these comorbidities.

Some examples include: 7,8

  • Sjögren’s Syndrome
  • SLE
  • APS
  • SSc
  • Hashimoto's thyroiditis
  • Primary Biliary Cirrhosis (PBC)

Diagnostic testing can provide you with further details needed to clarify a patient’s overlapping symptoms and achieve an accurate diagnosis of one or more autoimmune diseases.

Precise Management – A Tailored Approach for Your Patients

Due to the chronic and incurable nature of CTDs, overall management goals of patient care should be centered on reduction in symptom flares and overall quality of patient life. A multipronged approach is generally employed to manage CTDs, as there is no one size fits all treatment. Developing a personalized management plan that best helps your patient should be based on his or her individual manifestations of any of these potentially overlapping CTDs:
 

  • Sjögren’s Syndrome: Management decisions in SS are determined based on initial evaluation of symptoms and extraglandular manifestations. General management of SS is stratified according to severity of symptoms presented by each patient.9
  • SLE: The treatment of SLE patients will vary depending upon the extent of major organ involvement.10
  • SSc: Current pharmacological agents primarily focus on immunosuppression and immunomodulation. Structured care is recommended based on a patient’s individual presentation of the disease. 11
  • Polymyositis: The use of medications may be used in conjunction with physical therapy, speech therapy, and dietetic assessment, depending on symptom severity.
  • MCTDs: Treatment is generally targeted at suppressing inflammation present in tissues. Therapies must be tailored towards the organ system affected in individual patients and directed at specific clinical manifestations.12,13


Keeping an open line of communication with your patients about proper exercise regimen and diet may help them maximize the efficacy of their management strategy. Continual monitoring will help you and your patients assess the success of the plan and modify it as needed.

Continued Testing and Monitoring

Patients with autoimmune diseases are inherently more likely to develop additional or related autoimmune conditions, so it’s important that a long-term management plan be tailored to the signs and symptoms presented at the time. This is particularly important with overlap syndrome, which may cause manifestations due to multiple syndromes or diseases.

The ongoing treatment of CTDs should be monitored according to the specific CTD a patient is suffering from. Studies have suggested that patients with certain CTD Associated Pulmonary Arterial Hypertension (CTD-PAH), may have a poorer response to selected therapies than those with idiopathic PAH.14

Several tests also exist to help you continually monitor patients with one or more autoimmune diseases so that you can modify the plan as needed.

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References
  1. Brito-Zerón P, Ramos-Casals M, Bove A, et al. Predicting adverse outcomes in primary Sjögren's syndrome: identification of prognostic factors, Rheumatology. 2007;46:1359-1362.
  2. Gordon C. Long‐term complications of systemic lupus erythematosus. Rheumatology. 2002;41(10):1095-1100.
  3. Bronner IM, van der Meulen MFG, de Visser M, et al. Long‐term outcome in polymyositis and dermatomyositis. Ann Rheum Dis. 2006;65(11):1456-1461.
  4. Shah AA, Wigley FM. My Approach to the Treatment of Scleroderma. Mayo Clin Proced. 2013;88(4):377-393.
  5. MedicineNet.com. Mixed Connective Tissue Disease (MCTD). https://www.medicinenet.com/mixed_connective_tissue_disease/article.htm#what_is_the_prognosis_of_mixed_connective_tissue_disease. Accessed December 2017.
  6. Marder W, Littlejohn EA, Somers EC. Pregnancy and autoimmune connective tissue diseases. Best Pract Res Clin Rheum. 2016;30(1):63-80.
  7. Spagnolo P, Cordier JF, Cottin V. Connective tissue diseases, multimorbidity and the ageing lung. Eur Respir J. 2016 May;47(5):1535-1558.
  8. Robazzi TC, Adan LF. Autoimmune thyroid disease in patients with rheumatic diseases. Rev Bras Rheumatol. 2012;52(3):417-430.
  9. Stefanski AL, Tomiak C, Uwe P, et al. The Diagnosis and Treatment of Sjögren’s Syndrome. Dtsch Arztebl Int. 2017;114(20):354-361.
  10. Bertsias G, Ioannidis JPA, Boletis J, et al. EULAR recommendations for the management of systemic lupus erythematosus. Report of a Task Force of the EULAR Standing Committee for International Clinical Studies Including Therapeutics. Ann Rheum Dis. 2008;67:195-205.
  11. Kowal-Bielecka O, Fransen J, Avouac J, et al. Update of EULAR recommendations for the treatment of systemic sclerosis. Ann Rheum Dis. 2017;76(8):1327-1339.
  12. Mayo Clinic. Mixed connective tissue disease. https://www.mayoclinic.org/diseases-conditions/mixed-connective-tissue-disease/diagnosis-treatment/drc-20375152. Accessed December 2017.
  13. Kim P, Grossman JM. Treatment of mixed connective tissue disease. Rheum Dis Clin North Am. 2005(3):549-65. https://www.ncbi.nlm.nih.gov/pubmed/16084325
  14. Rhee, RL, Gabler NB, Sangani S, et al. Comparison of Treatment Response in Idiopathic and Connective Tissue Disease - Associated Pulmonary Arterial Hypertension. Am J Respir Crit Care Med. 2015;192(9):1111–1117.