Sarcoidosis: Nursing

Sarcoidosis is a chronic, multisystemic disease characterized by the formation of non-caseating, meaning non-necrotizing granulomas, which are nodules of chronically inflamed tissue in the lungs and lymph nodes.

Less commonly, these granulomas can accumulate in the heart, kidneys, joints, eyes, liver, spleen, and skin.Now, let’s quickly recap the physiology of the immune system.

So, normally, the cells of the immune system are ready to spot and destroy any foreign pathogens that could harm the body.

Some cells that help with this are called macrophages. When these cells come in contact with a pathogen, they latch onto it and then engulf or swallow it.

The macrophage then breaks down the pathogen, presents a piece of it, called an antigen, to its surface, and carries it to a lymph node.

That’s where macrophages find other immune cells called T-helper lymphocytes, which recognize and bind to the antigen, and start proliferating.

Proinflammatory cytokines, or signaling molecules, like tumor necrosis factor alpha, or TNF-ɑ for short, are then released by macrophages to help activate the helper T-cell and it begins to divide or proliferate.

The new T-cells leave the lymph node and start secreting other cytokines that recruit more immune cells like additional T-cells and macrophages.

Okay, now, the exact cause behind sarcoidosis is still unknown, but there are several risk factors that can be grouped into modifiable and non-modifiable ones.

Modifiable risk factors include exposure to mold, silica, or pesticides, whereas non-modifiable risk factors include age between 20 and 60 years, being assigned female at birth, and family history of pulmonary sarcoidosis.

Clients who are of Black race or Northern European descent are also at a higher risk of developing pulmonary sarcoidosis.Now, the pathology of pulmonary sarcoidosis starts with an antigen entering the body.

This antigen gets picked up by macrophages, which secrete cytokines that attract other immune cells, in addition to activating T helper cells.

As more and more immune cells gather at a particular spot, they form small nodules, called granulomas, that have T cells on the periphery and macrophages in the center.

Oftentimes, macrophages fuse together to form a single large multinucleated cell called a Langhans giant cell. The granulomas in sarcoidosis are noncaseating, which means the tissue at the center of the granuloma doesn’t necrose and turn into a yellow-greyish substance, unlike some other granulomatous diseases like tuberculosis.

With time, these non-caseating granulomas accumulate in the lungs or other organs, damaging their structure and affecting their function.

Granulomas often also form in the lymph nodes causing them to enlarge. When granulomas form in the heart, it can lead to arrhythmias; in the kidneys, it can lead to kidney stones and reduced kidney function; in the joints, it can cause arthritis; and in the eyes, it can result in uveitis, or inflammation of the pigmented layer of the eye beneath the cornea and sclera.

Sarcoid granulomas can also develop in the liver and spleen, causing them to enlarge, or it can develop in the skin, causing a variety of cutaneous lesions such as papules and nodules.

Vitamin D dysregulation is common, which can result in hypercalcemia, ultimately leading to kidney stones or osteoporosis.

Finally, the most serious complications of sarcoidosis include pulmonary fibrosis, leading to pulmonary hypertension, cor pulmonale, and right ventricular failure, as well as malignant cardiac arrhythmias and sudden cardiac death.Now, clients with sarcoidosis are typically asymptomatic, but some can present with fatigue and weight loss,in addition to respiratory symptoms, such as dry cough, dyspnea, and chest pain.

Other clinical manifestations of sarcoidosis depend on the involved organ and may include palpitations, syncope, flank pain, joint pain or swelling, vision changes such as photophobia, floaters, and blurry vision, as well as hepatomegaly, and splenomegaly.

Finally, maculopapular lesions can be present on the neck, chin, nose, eyelids, and lips, as well as the torso or extensor surfaces of the arms.

Clients can also develop painful nodules on the lower legs, along the tibias.The diagnosis of sarcoidosis starts with the client's history and physical assessment, followed by a chest X-ray and CT scan, which typically show bilateral hilar lymph node enlargement.

Pulmonary function tests can be also performed to look for decreased lung function. Additional diagnostic tests can be used to detect the involvement of other organs, and these tests include complete blood count, liver and kidney function tests, serum creatinine, serum and urinary calcium, as well as an ECG, which can show atrioventricular blocks, or atrial or ventricular arrhythmias.

Finally, biopsies can be taken from lymph nodes, skin lesions, or the lacrimal glands. The treatment of sarcoidosis depends on whether the client is symptomatic or not.

In asymptomatic clients with a normal chest X-ray, clients should undergo periodic monitoring, including pulmonary function tests, chest X-rays, and laboratory test results such as CBC, liver function tests, serum creatinine, and ECG.

On the other hand, symptomatic clients are typically treated with oral glucocorticoids; immunosuppressants, such as methotrexate and azathioprine, in addition to TNF alpha inhibitors, such as infliximab.

These treatment regimens go hand in hand with pulmonary rehabilitation and physical therapy. Finally, clients with end-stage disease may require lung transplantation.

All right, now let’s look at the nursing care you'll provide for a client with sarcoidosis. The priority goal for clients with pulmonary manifestations of sarcoidosis is to promote adequate oxygenation and ventilation.

Begin by assessing their vital signs and instituting pulse oximetry. Focus your respiratory assessment on their respiratory rate, auscultating their lungs, and taking note of symptoms such as respiratory crackles and cough.

If their SpO2 is less than 90%, position them in the high Fowler position and administer supplemental oxygen per nasal cannula.

Be sure to report if your client experiences dyspnea, chest pain, or palpitations. Continue to monitor your client closely and administer the prescribed medications.

Report to the healthcare provider if you notice indications of hypercalcemia such as mental status changes, nausea, or muscle weakness.

Finally, determine how your client's health related quality of life has been affected by their diagnosis, allow them time to express their concerns, and ensure there is a referral in place for psychosocial support, as needed.All right, now let’s move on to talk about general client and family teaching.

Begin by explaining how sarcoidosis causes nodules called granulomas to form in different tissues around the body that can impair normal body functioning, especially lung function.

Teach them about common extrapulmonary manifestations and instruct them to contact their healthcare provider right away if they experience new symptoms such as vision changes, flank pain, joint pain, skin lesions, fainting spells, or leg swelling.

Review the plan of care, and teach them about their prescribed medications, stressing the importance of taking them exactly as directed.

Next, encourage behaviors to maintain their general health. Promote smoking cessation and teach them to avoid secondhand smoke to prevent further injury to the lungs.

Talk about the importance of staying hydrated, and maintaining a healthy, balanced diet in order to prevent fatigue and weight loss associated with sarcoidosis.

Additionally, let them know that regular weight bearing exercise balanced with periods of rest as needed can help build strength, reduce fatigue, and decrease the risk of osteoporosis.

Lastly, stress the importance of keeping all of their follow-up appointments so their progress can be monitored.Finally, teach your client to immediately contact their health care provider if they experience increasing shortness of breath, increasing fatigue, new or worsening cough, chest pain, or palpitations..

All right, as a quick recap… Sarcoidosis is a chronic, multi-system disease, characterized by the formation of granulomas, which are collections of inflammatory tissue.

These granulomas deposit in the lungs and lymph nodes, as well as in the kidneys, liver, spleen, heart, eyes, joints, and skin, resulting in inflammation and damage to these structures.

Manifestations depend on the organ affected, and range from being asymptomatic to having fatigue, weight loss, respiratory symptoms like dry cough, dyspnea and chest pain; as well as palpitations, visual changes, skin lesions and painful nodules on the lower legs.Diagnosis is made based on the client’s history and physical exam, imaging, including a chest x-ray and CT scan, pulmonary function tests, lab tests including a CBC, kidney and liver function tests, electrolyte levels, and ECG.

Treatment for asymptomatic cases only involves monitoring, while symptomatic treatment includes corticosteroids and immunosuppressive medications.

Nursing care focuses on maintaining adequate oxygenation and ventilation, while client and family teaching focuses on learning about their diagnosis, how to manage the condition at home, and when to contact the healthcare provider.