Sarcoidosis is a multisystem inflammatory disease, most commonly affecting the lungs and lymph glands. It is characterized by the formation of multiple granulomas consisting of inflamed tissues in certain organs of the body. These granulomas alter the normal structure and the functioning of the affected organ.

ETIOLOGY

The etiology of pulmonary sarcoidosis is unknown. However, it is found to be related to age, gender, sex and there is also a certain degree of genetic predisposition to the disease. It commonly occurs between the ages of 20 to 40. Women are more prone to get the disease than men. Also, people of African-American descent are more likely to get the disease than Caucasians. (Robert P Baughman et al., 2001) The disease is mostly thought to be autoimmune. Several infectious organisms are also found to be related to the disease.

SYMPTOMS

Thirty percent to sixty percent of patients with sarcoidosis are asymptomatic with incidental findings on chest radiographs. Chronic pulmonary sarcoidosis persists for years, resulting in an inevitable loss of lung function and destruction of the alveolar structure. Fatalities associated with sarcoidosis occur in 1% to 6% of patients. (Lynch, Kazerooni, & Gay, 1997)

The symptoms of pulmonary sarcoidosis are variable. The most common respiratory systems include dry cough, dyspnea at exertion, and chest pain. Chest X‐ray is abnormal in 85-90% of cases.

EXTRAPULMONARY SYMPTOMS

Sarcoidosis may affect other organ systems. Following are the organ systems that are affected by this disease.

Skin Lesions

It affects the skin in 25% of the patients, the most common lesions being plaques, erythema nodosum, subcutaneous nodules, maculopapular eruptions, and lupus pernio. There may also be hair loss, rashes, and skin sores. The lesions usually resolve spontaneously in 2–4 weeks so no treatment is required. (Mañá & Marcoval, 2012)

Cardiovascular Symptoms

The heart can also be involved in the disease and is more common in people of Japanese descent. Conduction abnormalities, ventricular arrhythmias, fibrosis, granuloma formation, and congestive heart failure are the most common complications in this involvement. All these symptoms need cardiologic treatment. (Chapelon-Abric, 2012)

Ocular Symptoms

Eye complications occur in about 10–90% of cases. These include uveitis and retinal inflammation. This may result in loss of visual acuity or blindness. The ocular sarcoidosis is treated with systemic corticosteroids. (Bodaghi, Touitou, Fardeau, Chapelon, & LeHoang, 2012)

Neurological Involvement

Neurological involvement in sarcoidosis affects mainly the cranial nerves, hypothalamus, and pituitary gland. (Nozaki & Judson, 2012)

Other Symptoms

Other systems that could be involved in the extrapulmonary complications of this disease include the gastrointestinal system, endocrine, and exocrine systems, genitourinary system, blood, bone, joint and muscular systems.

SARCOIDOSIS & PREGNANCY

Sarcoidosis does not prevent pregnancy nor hampers the delivery process. Studies have shown that Sarcoidosis is associated with increased risks for preeclampsia/eclampsia, cesarean delivery, preterm birth as well as certain birth defects. There is a need for awareness to prevent pregnancy complications in mothers with sarcoidosis and their newborns. (Köcher et al., 2020)

STAGES OF PULMONARY SARCOIDOSIS

Radiographic staging of pulmonary sarcoidosis is based on the presence of mediastinal lymphadenopathies or lung infiltration without or with fibrosis. Stage 1 has the involvement of lymph nodes and the disease can spontaneously remit in 55-90% of the cases. Stages 2 and 3 involve diffuse micronodular or reticulomicronodular patterns predominantly affecting the middle and the upper parts of the lungs. Stage 4 is characterized by lung fibrosis having coarse linear opacities along with lung retraction. (Nunes, Soler, & Valeyre, 2005)

DIAGNOSTIC PROCEDURES

The diagnosis of Sarcoidosis is made most commonly through radiographic procedure after taking the patient history and performing the physical examination. CT scan and pulmonary function tests are also conducted to assess the health of the lungs. Blood tests to assess the derangement of oxygen and carbon dioxide concentrations are also done. For patients with pulmonary symptoms, clinicians can make use of various techniques for making the diagnosis, which includes needle aspiration, bronchoalveolar lavage (BAL), transbronchial biopsy, open lung biopsy, and mediastinoscopy. (R. P. Baughman, 2004) It is also required to examine the extrapulmonary complications because sarcoidosis is a multisystem disease not confined only to the lungs.

TREATMENT

The course of treatment employed varies according to the symptoms and degree of complications. Regular checkups, dietary control, healthy diet, proper sleep, abstaining from smoking and healthy lifestyle modifications may help resolve minor symptoms of the disease. There is no need for medicinal involvement in minor granuloma findings because they might resolve on their own.

The disease requires treatment if the function of the lungs starts to get compromised and the patient is stagnant in the same condition for 3-6 months. Corticosteroids are administered to improve lung function, Prednisone being the most common. However, if there is no active granulomatous inflammation and the scarring or fibrosis of the lung is the cause of abnormal lung function, then the therapy should be discontinued as well. If the disease progresses without treatment, then it can lead to permanent lung damage. Involvement of the heart, brain, and lungs in advanced disease stages is inevitably fatal.

LITERATURE CITED

Baughman, R. P. (2004). Pulmonary sarcoidosis. Clin Chest Med, 25(3), 521-530, vi. doi:10.1016/j.ccm.2004.04.006

Baughman, R. P., Teirstein, A. S., Judson, M. A., Rossman, M. D., Yeager, H., Bresnitz, E. A., . . . Group, A. C. C. E. S. O. S. R. (2001). Clinical Characteristics of Patients in a Case Control Study of Sarcoidosis. American Journal of Respiratory and Critical Care Medicine, 164(10), 1885-1889. doi:10.1164/ajrccm.164.10.2104046

Bodaghi, B., Touitou, V., Fardeau, C., Chapelon, C., & LeHoang, P. (2012). Ocular sarcoidosis. La Presse Médicale, 41(6, Part 2), e349-e354. doi:https://doi.org/10.1016/j.lpm.2012.04.004

Chapelon-Abric, C. (2012). Cardiac sarcoidosis. La Presse Médicale, 41(6, Part 2), e317-e330. doi:https://doi.org/10.1016/j.lpm.2012.04.002

Köcher, L., Rossides, M., Remaeus, K., Grunewald, J., Eklund, A., Kullberg, S., & Arkema, E. V. (2020). Maternal and infant outcomes in sarcoidosis pregnancy: a Swedish population-based cohort study of first births. Respiratory Research, 21(1), 225. doi:10.1186/s12931-020-01493-y

Lynch, J. P., Kazerooni, E. A., & Gay, S. E. (1997). PULMONARY SARCOIDOSIS. Clinics in Chest Medicine, 18(4), 755-785. doi:https://doi.org/10.1016/S0272-5231(05)70417-2

Mañá, J., & Marcoval, J. (2012). Skin manifestations of sarcoidosis. La Presse Médicale, 41(6, Part 2), e355-e374. doi:https://doi.org/10.1016/j.lpm.2012.02.046

Nozaki, K., & Judson, M. A. (2012). Neurosarcoidosis: Clinical manifestations, diagnosis and treatment. La Presse Médicale, 41(6, Part 2), e331-e348. doi:https://doi.org/10.1016/j.lpm.2011.12.017

Nunes, H., Soler, P., & Valeyre, D. (2005). Pulmonary sarcoidosis. Allergy, 60(5), 565-582. doi:https://doi.org/10.1111/j.1398-9995.2005.00778.x