Sarcoidosis

Sarcoidosis, formerly called Boeck sarcoid, is a noncontagious multisystem disorder characterized by epithelioid granular tumors (granulomas) that most frequently affect the lung; more than 90% of cases involve lung or intrathoracic lymph nodes. Pulmonary sarcoidosis is usually a chronic disorder associated with an intense cellular immune response in the alveolar structures of the lungs. A series of interactions between lymphocytes (primarily responsible for the granuloma formation), macrophages/monocytes (primarily responsible for interstitial fibrosis), epithelioid cells, and giant cells lead to the formation of noncaseating granulomas. The granulomas can lead to fibrosis, which affects the lung's ability to exchange gases. Other recurrent sites include the liver, lymph nodes, bone marrow, skin, and eyes. Sarcoidosis is detected occasionally in the spleen, joints, heart, skeletal muscle, phalangeal bones, parotid glands, and central nervous system (CNS).

About 65% to 70% of the cases of sarcoidosis usually resolve spontaneously within 2 years. Without treatment, however, sarcoidosis can lead to chronic progressive sarcoidosis, which is associated with complications such as pulmonary fibrosis, scarring, and progressive pulmonary disease. In such cases, when the heart can no longer pump against the noncompliant fibrotic lungs, cor pulmonale can develop. Other potentially lethal complications are superinfections by organisms such as Aspergillus. The overall mortality is less than 5% for patients who do not receive treatment.

Causes ⬆ ⬇

Sarcoidosis is a complex and mysterious disease of unknown origin, although 80% of patients with sarcoidosis have high titers of the Epstein-Barr virus. It is not a malignant disease, and it is not an autoimmune disease. There is increasing evidence that a triggering agent strikes and stimulates an enhanced cell-mediated immune process at the site of involvement. The triggering agent may be a fungus, an atypical mycobacterium, pine pollen, or a toxic chemical such as zirconium or beryllium, which can lead to illnesses resembling sarcoidosis. Because there is a slightly higher incidence of sarcoidosis in the same family, the triggering agent may be genetic. The primary risk factor seems to be a family history, but environmental exposure to toxins and chemicals may contribute. The risk seems to be enhanced in people working in jobs related to agriculture, water, construction, metal work, education, and health. Smoking is also a risk factor.

Genetic Considerations ⬆ ⬇

Allelic variation in human leukocyte antigen (HLA)-DRB1 increases susceptibility for sarcoidosis. A mutation near the HLA-DRBI gene, in the BTNL2 gene, also independently affects susceptibility to sarcoidosis, while mutations in the NOD2 gene cause an early-onset form of sarcoidosis. Polymorphisms in the ANXA11 gene are also associated with sarcoidosis, a result that is consistent across ethnic groups. Confusingly, familial clustering of this disease may also be due to a shared environment. The much greater frequency of Black people with sarcoidosis as compared to other groups in the United States suggests a genetic contribution to etiology. A genome-wide association study conducted in Black individuals demonstrated an SNP in the NOTCH4 gene as well as the XAF1 gene. Familial transmission patterns do not always follow simple Mendelian rules, making complex transmission combining both genetic and environmental factors most likely.

Sex and Life Span Considerations ⬆ ⬇

Disease onset is usually between ages 20 and 30 years or between 45 and 65 years. It is more frequent among women than men, and women have poorer outcomes.

Health Disparities and Sexual/Gender Minority Health ⬆ ⬇

Frequency of sarcoidosis is 10 times higher in Black persons as compared to White persons, and Black women of childbearing age develop sarcoidosis twice as frequently as Black men. Black patients experience more severe pulmonary disease, more multi-organ involvement, higher rates of hospitalization, and a worse prognosis than other groups. The financial strain of the condition is highest among women and Black persons (Ogundipe et al., 2019 [see Evidence-Based Practice and Health Policy]). Sexual and gender minority status has no known effect on the risk for sarcoidosis.

Global Health Considerations ⬆ ⬇

The overall global prevalence of sarcoidosis is similar to that in the United States. The highest reported prevalence is in Sweden, and the lowest is in Poland. People of Scandinavian ancestry have higher rates of the disease than their other European counterparts. In developing countries without experience with the disease or without diagnostic capabilities, it may be misdiagnosed as tuberculosis.

Assessment ⬆ ⬇

ASSESSMENT

History

Sarcoidosis is known as the great masquerader because it presents in a variety of guises from lymphadenopathy to erythema nodosum (red and painful nodules on the legs, associated with rheumatism). Establish a history of arthralgia in the wrists, ankles, and elbows, which is an initial symptom of sarcoidosis. Ask if the patient has experienced fatigue, malaise, weakness, anorexia, fever, or weight loss. Elicit a history of respiratory difficulties, such as breathlessness, cough (generally nonproductive but may be blood tinged [hemoptysis]), or substernal chest pain. Ask if the patient has had any visual deficits or any eye pain, night sweats, seizures, or cranial or peripheral nerve palsies, which are signs of CNS involvement. Determine if a family history of sarcoidosis exists. Ask if the patient has been exposed to a viral or bacterial infection or to chemicals such as beryllium or zirconium.

Physical Examination

Common symptoms are organ specific but may include fever, fatigue, anorexia, muscle pain, dyspnea, cough, and chest pain. Examine the patient's skin for lesions, plaques, papules, and subcutaneous nodules on the face, neck, and extremities. A common skin lesion, erythema nodosum, is seen in 10% of cases; other lesions, such as lupus pernio (hard, blue-purple, swollen, shiny lesions of the nose, cheeks, lips, ears, fingers, and knees), are seen in 15% of patients. When granulomas affect the face, they tend to occur around the nose and may cause nasal destruction and disfigurement. Note the patient's skin tone for signs of jaundice.

Up to 60% of patients develop intraocular symptoms. Examine the patient's eyes for signs of enlarged tear glands, conjunctival infections, and granulomatous uveitis; if the patient has noted visual difficulties, perform a vision examination. Bilateral granulomatous uveitis occurs in 15% of cases and can lead to loss of vision because of secondary glaucoma. Other ocular symptoms may include retinal periphlebitis, lacrimal gland enlargement, and conjunctival infiltration, which can result in blurred vision, ocular pain, conjunctival infections, and iritis.

Inspect the patient's legs and arms for muscle wasting and enlarged or reddened joints. Ask the patient to move the joints in full range of motion to determine if pain or tenderness occurs. Palpate the salivary and parotid glands to determine if nontender enlargement is present. Palpate the lymph nodes to assess for lymphadenopathy and the abdomen for an enlarged liver and spleen. Auscultate the patient's chest to determine if diminished breath sounds indicate pulmonary fibrosis, infiltration, or restrictive disease. If breath sounds are not audible in one lung, suspect a pneumothorax. If you hear adventitious breath sounds or lung consolidation, suspect pulmonary infection. Auscultate the patient's heart and note any irregularities that might indicate bundle-branch block or ventricular ectopy.

Psychosocial

Sarcoidosis is associated with fatigue and can be linked with depression, bipolar disorder, panic disorder, or anxiety disorder. As with any chronic disease, the patient and family need continual support and caring from healthcare professionals. Assess the patient's and family's ability to cope with a chronic disease and the change in roles that a chronic disease demands. The patient may also be distressed over changes in appearance that have been caused by lesions on the face in particular.

Diagnostic Highlights

General Comments: Sarcoidosis is primarily diagnosed by exclusion or by finding characteristic noncaseating granulomas in the affected organs.

Test	Normal Result	Abnormality With Condition	Explanation
Tuberculin skin test, fungal serologies, and sputum cultures for mycobacteria and fungi	Negative	Used to make a differential diagnosis; positive results indicate diagnosis other than sarcoidosis	Rule out tuberculosis
Tissue biopsy	Negative	Used to make a differential diagnosis; positive results indicate diagnosis other than sarcoidosis	Rule out other conditions
High-resolution computed tomography	Clear lung fields with no lymphadenopathy	Active alveolitis or fibrosis	Demonstrates extent of air trapping

Other Tests: Tests include complete blood count, serum electrolytes with calcium, gallium-67 scanning, electrocardiogram, bronchoalveolar lavage, pulmonary function tests, antinuclear antibodies, rheumatoid factor levels, and hypergammaglobulinemia. Other tests may include serum amyloid A (SAA), soluble interleukin-2 receptor (sIL-2R), alkaline phosphatase, lysozyme, angiotensin-converting enzyme (ACE), and glycoprotein KL-6.

Primary Nursing Diagnosis ⬆ ⬇

Diagnosis

DiagnosisImpaired gas exchange related to altered alveolar-capillary membrane changes and decreased oxygen-carrying ability as evidenced by fatigue, dyspnea, diminished breath sounds, and/or cough

Outcomes

OutcomesRespiratory status: Gas exchange; Respiratory status: Ventilation; Symptom severity; Symptom management; Knowledge: Disease process

Interventions

InterventionsAirway management; Oxygen therapy; Respiratory management; Respiratory monitoring; Ventilation assistance; Teaching: Disease process

Planning and Implementation ⬆ ⬇

PLANNING AND IMPLEMENTATION

Collaborative

Asymptomatic sarcoidosis requires no treatment, although ongoing assessment is called for. Sarcoidosis with ocular, respiratory, CNS, cardiac, or systemic symptoms requires treatment with systemic or topical corticosteroids. Other treatment includes a low-calcium, high-calorie nutritional diet with an increase in fluids to prevent malnutrition, hypercalcemia, and dehydration. A low-sodium diet may be indicated if sodium retention occurs because of prednisone. Ongoing monitoring of the patient's physical condition by physical examination and diagnostic tests indicates the patient's response to treatment and the appearance of complications.

Pharmacologic Highlights

Medication or Drug Class	Dosage	Description	Rationale
Prednisone	May range from 30–60 mg/day PO, tapering over 4–8 wk to a maintenance dose of 10–15 mg for 6 mo every other day	Corticosteroids remain the mainstay of therapy for the first 1–2 yr, but some patients may require lifelong steroid therapy	Relieves symptoms and reverses fibrosis of pulmonary tissue

Other Drugs: Optic agents such as methylcellulose eyedrops and other ophthalmic ointments are used to treat ocular manifestations. Antidysrhythmic agents are used to treat ventricular ectopy. Salicylates and other NSAIDs are used for the treatment of arthritis manifestations. Cytotoxic agents such as methotrexate may be used to inhibit T-cell production. Other medications used to treat cutaneous sarcoidosis include hydroxychloroquine, azathioprine, cyclosporine, chlorambucil, allopurinol, doxycycline, and infliximab.

Independent

Because many patients have pulmonary granulomas that have the potential to affect airway, breathing, and gas exchange, the primary nursing focus is to ensure that these essential functions are preserved. Maintain an oral airway and endotracheal intubation equipment near the patient at all times in case they are needed to clear airway obstruction. Support the patient's breathing by positioning the patient for comfort (often with the head of the bed elevated and the arms raised slightly on pillows). Adjust the patient's activity to reduce oxygen demands. Space all activities with adequate periods of rest. Provide uninterrupted periods of sleep at night and at least one 2-hour rest period during the day. Schedule diagnostic tests to provide adequate rest and work with the family and other visitors to conserve the patient's energy.

Changes in vision place the patient at risk for injury. Teach the patient to scan the area for obstructions before beginning to walk. Remove any obstructions or rugs in the path between the patient's bed and the bathroom. Encourage the patient to wear well-fitting shoes or slippers when ambulating. The patient's impaired vision, intolerance to activity, and any lesions on the face may lead to a disturbance in self-concept or body image. From the patient, elicit priorities for a “good” appearance and support those activities that the patient finds beneficial. Those activities may include extra hair care, using makeup, wearing clothing from home, maintaining a beard or moustache, or other similar grooming strategies. Help the patient maintain the highest level of activity that the disease allows. As with any chronic, debilitating disease with no cure, the patient is expected to have times of depression and anxiety. Use a supportive, nonjudgmental approach and active listening. Answer the patient's questions honestly and provide information about the long-range prognosis of the condition. If the patient or family demonstrates ineffective coping, refer the patient or significant others for counseling or to a support group.

Evidence-Based Practice and Health Policy

Ogundipe, F., Mehari, A., & Gillum, R. (2019). Disparities in sarcoidosis mortality by region, urbanization, and race in the United States: A multiple cause of death analysis. American Journal of Medicine, 132, 1062–1068. https://doi.org/10.1016/j.rmed.2017.11.008

The goal of this study was to assess if the disparity of the sarcoidosis-related mortality rates varies by sex, race/ethnicity, region, and urbanization. U.S. data for multiple causes of death for 1999 to 2016 were used to determine numbers of deaths (N = 28,923) and age-adjusted rates for sarcoidosis as a cause of death. In the years 2008 to 2016, 9,112 deaths had sarcoidosis as the underlying cause (56%) compared with 16,129 with sarcoidosis listed as any cause.
Age-adjusted annual death rates were higher for females than males and lower for Hispanics than for non-Hispanics. Rates in non-Hispanic Blacks were eight times those in non-Hispanic Whites. Among females, the highest rate was in non-Hispanic Blacks in the East-Central division. Sarcoidosis-related multiple cause of death mortality rates were highest in females and in non-Hispanic Blacks, and they varied geographically.

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DRG Information ⬇

Introduction ⬆ ⬇