Sarcoidosis: Symptoms, Causes and Treatment

Sarcoidosis is an inflammatory condition which affects the multiple organs of the body but the lungs are the most commonly involved organ. It is characterized as an autoimmune disease in which different patients show different outcomes of the disease as some patients show complete remission and some develops chronic disease for decades. The disease is prevalent worldwide but it is more common in african americans because of the severity of the disease is high among africans.

Sarcoidosis Symptoms

• Patient will present with shortness of breath and persistent dry cough.
• There will be red papules and nodules on the skin of lower limbs in some cases.
• Ophthalmological symptoms like dry or sore eyes and vision problems can develop.
• Lymphadenopathy
• Musculoskeletal features like joint pain and bone pain can be present.

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Sarcoidosis Causes and Pathogenesis

• The etiology of the disease is not very clear but there are some infectious and non-infectious agents ( insecticides) that are studied in recent times which induces inflammatory reaction in the body .
•  Studies have suggested that some patients are genetically susceptible to the sarcoidosis and some patients have family members suffering from sarcoidosis.
• The pathognomonic hallmark for sarcoidosis is granuloma formation which is accumulation of inflammatory cells that includes T helper cells and monocytes.

                                              Granuloma formation

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Sarcoidosis Complications/Clinical Manifestations

As sarcoidosis is a multiorgan disease, it can affect different organ systems.

• Pulmonary Sarcoidosis - The major presentation will be bilateral hilar lymphadenopathy and on chest xray the major findings will be interstitial infiltrates. As the disease progresses the patient will start having symptoms of Restrictive lung disease.
• Cutaneous/skin Sarcoidosis-  There will be papules, nodules and plaques. There is one form of disease called as Lofgren’s syndrome which consists of erythema nodosum and hilar lymphadenopathy in sarcoidosis patients. But it has a good prognosis.
• Ophthalmologic Sarcoidosis- It mainly presents as Anterior and posterior uveitis along with the Keratoconjuctivitis Sicca in which patient feels excessive dryness and irritation in the eyes and there is continuous watering from the eyes.
• Neurologic Sarcoidosis- The patient can present with several neurological issues like Facial nerve palsy, Central Diabetes Inspidus and Hypogonadotropic hypogonadism. The other cranial nerve manifestation is Optic neuritis.
• Cardiac sarcoidosis- There is reduction in functioning of the left ventricle due to the infiltration of the heart muscle by granulomas. Generally features of congestive heart failure are seen but if the granulomas infiltrate the AV node it can lead to heart block.
• Gastrointestinal Sarcoidosis- Liver is involved mainly and there will be elevation of the Liver enzymes like alkaline phophatase. Hepatosplenomegaly will be noticed.
• Hypercalcemia- It is very common in the sarcoidosis patients that there will be hypercalcemia and hypercalciurea. Reason for hypercalcemia is increased production of 1,25-dihydroxyvitaminD  by the granuloma. This leads to increased intestinal absorption calcium leading to hypercalcemia and the PTH levels will be low.
• Other manifestations are polyarthritis, Constitutional symptoms like fever, malaise and Parotid gland swelling.

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Sarcoidosis Diagnosis

• Most commonly used imaging for diagnosis of sarcoidosis is chest xray that will detect the bilateral hilar lymphadenopathy. CT scan can show the presence of Interstitial Lung Disease.
• Cardiac PET scan is used to diagnose the Cardiac sarcoidosis.
• MRI is useful to diagnose the extrapulmonary manifestations of the Sarcoidosis.
• Serum levels of Angiotensin converting enzyme are helpful in the diagnosis.
• On biopsy if the granulomas are suspected then it is corelated with the clinical diagnosis. Other symptoms like uveitis, abnormal LFTs, Seizures due to neuro problems etc. Should be corelated carefully.
•  Skin biopsy can be done to take the samples. But if the granulomas are suspected make sure to rule out other causes like malignancy, TB.

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Sarcoidosis Treatment

• The main motive of treating the sarcoidosis patients is to improve the quality of life and try to keep the symptoms normal.
• Steroids like prednisone are very useful and dose is maintained on the basis of severity and the tolerability of the patient. For example- The dosage is higher among neurosarcoidosis patients but lower in the cutaneous sarcoidosis.
• It is usually a self limiting disease but the mortality is mainly found in pulmonary fibrosis and pulmonary hypertension patients.

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