Due to its varying symptoms, sarcoidosis is typically categorized into types depending on which part of the body it affects.
Cardiac Sarcoidosis
Cardiac sarcoidosis affects the heart and is found in approximately one-third of sarcoidosis patients, although only 5% will actually experience symptoms. The granulomas produced by sarcoidosis interfere with the electrical signals that trigger the contraction of the heart muscle and can manifest as advanced heart block, arrhythmias, or irregular heart beats, and in severe cases, congestive heart failure. Symptoms of cardiac sarcoidosis can include chest pain, fainting, fatigue, palpitations, shortness of breath, and other potential life-threatening symptoms. If you are having cardiac symptoms, please call 911 or go to the emergency room immediately.
Diagnosis & Treatment for Cardiac Sarcoidosis
In order to diagnose cardiac sarcoidosis, your doctors may eliminate the possibility of other diseases through blood tests, ultrasound imaging of the heart, a PET scan, biopsies, or an MRI, among other diagnostic testing procedures. Once diagnosed with cardiac sarcoidosis, treatment may include immunosuppressants, a pacemaker, or in more severe cases, a heart transplant.
Cutaneous Sarcoidosis
Sarcoidosis of the skin, also known as cutaneous sarcoidosis, can present in many different ways and occurs in approximately 20 to 35 percent of patients. There are two main ways that cutaneous sarcoidosis presents: erythema nodosum and lupus pernio. Erythema nodosum causes painful, red lumps on the lower arms, legs, or shins and is often accompanied by Lofgren syndrome, or arthritis in the ankles, elbows, wrists, and hands. The lupus pernio variant is a painful bluish-red, purple discoloration of nodules of the skin found on the face, ears, fingers, or toes. Overall, lesions can vary in severity and can be lumps or rashes located on the extremities, face, scalp, back, and buttocks.
Diagnosis & Treatment for Cutaneous Sarcoidosis
In order to diagnose sarcoidosis of the skin, a skin biopsy is the most likely option in conjunction with a physical examination and discussion of your medical history. Cutaneous sarcoidosis typically requires treatment to heal, and the treatment plan will vary depending on the number of skin lesions, how quickly new lesions appear, and the location of the lesions. Treatment for mild cases typically involve creams, ointments, or injections into the lesions, but more severe cases may require additional oral or intravenous medications.
Hepatic Sarcoidosis
Hepatic sarcoidosis involves granulomas in the liver. Liver involvement is extremely common in sarcoidosis and occurs in approximately 75% of patients, although most cases are asymptomatic. Those with hepatic sarcoidosis may experience fever, abdominal pain, weight loss, enlargement of the liver, changes in liver enzyme levels, jaundice, malaise, and fatigue.
Diagnosis & Treatment for Hepatic Sarcoidosis
Sarcoidosis of the liver will usually be picked up when testing for sarcoidosis in other parts of the body but is typically diagnosed through a liver biopsy or CT scan. Since the majority of patients with hepatic sarcoidosis do not display symptoms, they do not require specific treatment. Those that do experience symptoms may be treated with corticosteroids and be referred to a liver specialist or gastroenterologist. For patients with decompensated liver disease, liver transplantation may be a valid option.
Musculoskeletal Sarcoidosis
Musculoskeletal sarcoidosis affects the bones, muscles, and joints, and can present as arthritic-like symptoms such as the swelling and tenderness of the lower legs, ankles, knees, and fingers. Some patients experience sudden onset pain and swelling in the joints in conjunction with red or purple bumps at the shins. If these symptoms occur together, your doctor may arrange a chest x-ray to look for enlarged lymph glands in the chest. The combination of these three symptoms – joint swelling, skin changes, and enlarged lymph glands – is called Lofgren’s Syndrome. Sarcoidosis can also affect bone density, which could be a side effect of prednisone and corticosteroid treatment, a treatment common for sarcoidosis treatments. A loss of bone density can result in an increased risk of fractures should bones eventually weaken.
Diagnosis & Treatment for Musculoskeletal Sarcoidosis
In order to diagnose musculoskeletal sarcoidosis, a healthcare provider may use x-rays, CT scans, MRIs, PET scans, or ultrasounds. Biopsies can also be taken from affected muscles or swollen joints to search for abnormalities, such as a granuloma. Treatments for musculoskeletal sarcoidosis are typically non-steroidal anti-inflammatory (NSAIDs) drugs or corticosteroids like prednisone, but can also include encouragement to keep moving and exercise daily to reduce stiffness felt around the joints.
Neurosarcoidosis
Neurosarcoidosis affects between 5 and 15 percent of patients. This type of sarcoidosis occurs when granulomas develop in and around the central nervous system, which encompasses the brain, spinal cord, and optic nerve. The inflammation from the granulomas can destroy the coating that surrounds and protects nerve fibers resulting in the disruption of the normal flow of nerve impulses from the central nervous system; ultimately, causing a reduction or loss of body function.
Neurosarcoidosis is rare, but can be severe and debilitating. It generally affects the cranial and facial nerves, the hypothalamus area of the brain, and the pituitary gland. Symptoms vary depending on the areas of the central nervous system that are affected. Pituitary symptoms may include fatigue, changes in the menstrual cycle for women, excessive urination, and increased thirst. Neurosarcoidosis patients may also experience confusion, facial palsy, seizures, speech impairments, and more.
Diagnosis & Treatment for Neurosarcoidosis
If a patient has a history of sarcoidosis followed by nerve issues, it’s likely they have developed neurosarcoidosis. Diagnosis begins with a detailed medical history and neurological exam, followed by an MRI, spinal tap, or chest x-rays if necessary. There is no cure for neurosarcoidosis and treatment will usually occur when patients are experiencing severe or progressing symptoms. This can include corticosteroids, physical or occupational therapy, and general pain management.
Ocular Sarcoidosis
Ocular sarcoidosis affects the eyes and is a common type of sarcoidosis, occurring in approximately 50% of sarcoidosis patients. This type of sarcoidosis can involve any part of the eye and its surrounding tissues. Symptoms may include uveitis, eyelid abnormalities, conjunctival granulomas, optic neuropathy, lacrimal gland enlargement, and orbital inflammation. Uveitis is the most common diagnosis and can cause pain, redness, swelling, and vision problems. Conjunctival granulomas, or painful lesions in the eye, are rare. Patients with ocular sarcoidosis symptoms should seek treatment immediately to avoid major complications such as cataracts or glaucoma.
Diagnosis & Treatment for Ocular Sarcoidosis
Diagnosis of ocular sarcoidosis is usually based on a combination of medical history, physical examinations, and the exclusion of other granulomatous diseases such as tuberculosis or syphilis. Most patients have pulmonary involvement, so chest-x-rays or CT scans are frequently used as an additional screening tool. Much like other types of sarcoidosis, treatment often includes corticosteroids and immunosuppressive agents for intraocular manifestations, and cyclosporine eye drops for ocular surface manifestations, although rare.
Pulmonary Sarcoidosis
While sarcoidosis can occur in virtually any organ, pulmonary sarcoidosis, or sarcoidosis of the lungs, is the most common type of sarcoidosis. Many sarcoidosis patients do not have symptoms and do not know they have the disease. However, those with symptoms may experience a shortness of breath, dry cough, chest pain, swollen lymph nodes, and unexplained weight loss. Almost every symptom of pulmonary sarcoidosis can be explained by other health issues, so it’s important to see a healthcare provider for proper diagnosis.
Diagnosis & Treatment for Pulmonary Sarcoidosis
In order to diagnose pulmonary sarcoidosis, your doctor may order chest x-rays, a CT scan, pulmonary function tests to measure the lungs’ ability to move air in and out, blood tests to determine oxygen levels, a bronchoscopy, or a lung biopsy. Once diagnosed with pulmonary sarcoidosis, treatment is determined primarily to control symptoms and improve organ function. Steroid medication, such as prednisone, can help reduce inflammation. In severe cases, a lung transplant or oxygen therapy may be required, although this is rare.
Renal Sarcoidosis
Renal sarcoidosis is characterized by the formation of granulomas in the kidney, and occurs in 35 to 50 percent of patients. The granulomas can produce an enzyme which can result in elevated calcium levels in the blood and urine which can lead to dehydration, and the development of kidney stones. Symptoms of renal sarcoidosis include inflammation, hypercalcemia, or high levels of calcium in the blood, hypercalciuria, or high levels of calcium in the urine, and dehydration. In rare cases, kidney failure may occur.
Diagnosis & Treatment for Renal Sarcoidosis
Renal sarcoidosis is typically diagnosed through a renal biopsy. The standard treatment is corticosteroids, but if renal sarcoidosis patients do not respond to them, other immunosuppressants may be used. Early diagnosis and prompt treatment can help reduce the risks of kidney failure.
Sarcoidosis in the Salivary Glands
Sarcoidosis in the salivary glands presents with a painless and persistent enlargement of the parotid glands. The parotid glands are two salivary glands situated in front of each ear. Patients often present with dry mouth, or xerostomia, where the salivary glands don’t produce enough saliva to keep your mouth wet. Otolaryngologic manifestations are seen in 10 to 15 percent of sarcoidosis patients.
Diagnosis & Treatment for Sarcoidosis in the Salivary Glands
A parotidectomy is the most common diagnostic tool for determining whether a patient has sarcoidosis in the salivary glands. As far as treatment, most patients are treated with corticosteroids in order to improve symptoms, quality of life, and control.
Sarcoidosis of the Sinuses
Sarcoidosis of the sinuses has been reported in up to 38% of sarcoidosis cases. Patients with sarcoidosis nasal involvement have presented with symptoms of nasal crusting, congestion, bleeding from the nose, pain, the loss of the sense of smell, the lining of the nasal cavity is easily torn, and nasal polyps. Most patients experiencing sarcoidosis of the sinuses have abnormal sinus x-rays with either thickening of the lining of the nasal cavity or inflammation of the sinuses.
Diagnosis & Treatment for Sarcoidosis of the Sinuses
In order to diagnose sarcoidosis of the sinuses, there must be radiological evidence in conjunction with no evidence of other diseases associated with granulomatous nasal or sinus inflammation. In sinonasal sarcoidosis, minimally invasive surgeries can significantly improve the quality of life, especially in those with severe nasal obstruction. Frequent saline nasal irrigation is also recommended in all sinonasal sarcoidosis patients to eliminate nasal crusting.
Sarcoidosis of the Spleen and Bone Marrow
Sarcoidosis of the spleen and bone marrow occurs with the formation of granulomas in those specific organs. According to reports, 34% of sarcoidosis patients experience sarcoidosis of the spleen and 10% experience bone marrow involvement. Bone marrow is the spongy tissue found in the center of our largest bones and contains stem cells. The stem cells produce red blood cells that transport oxygen throughout the body, and white blood cells that fight infection. While the spleen is widely known as an organ you can live without, it does have a primary function – to filter the blood. It’s a soft, purple-ish organ located under the rib cage in the left upper quadrant of the abdomen. Spleen or bone marrow sarcoidosis may lead to anemia, a lack of red blood cells to transport oxygen, leukopenia, a low white blood cell count, and thrombocytopenia, low levels of platelets in the blood.
Diagnosis & Treatment for Sarcoidosis of the Spleen and Bone Marrow
Diagnosing sarcoidosis of the spleen and bone marrow happens through a combination of tests, including physical exams, blood tests, imaging, and biopsies. Your doctor may also perform other tests to rule out viruses, bacterial infections, and lymphoma. In patients with sarcoidosis of the spleen and bone marrow, doctors may prescribe medicine that normalizes blood cell counts in addition to corticosteroids.
Sarcoidosis Research and Education
Due to its complexity, sarcoidosis can be difficult to understand; particularly to those people who have been newly diagnosed. Whether you’re looking for traditional treatment options, want to understand the risk factors involved in sarcoidosis, or simply trying to find a doctor who is well-versed in sarcoidosis treatment and management, there’s a ton of resources out there to support you.
View Our Sarcoidosis Resources