National Sarcoidosis Organization

Canadian Sarcoidosis Awareness and Research Organizaion

News & Info

Childhood Sarcoidosis

Posted by Melany Sealy on February 4, 2012 at 1:50 PM

Childhood Sarcoidosis

Childhood sarcoidosis is not as uncommon as some may thing due to the fact it is commonly misdiagnose such as in adults. It is a multi systemic glaucomatous disorder of unknown causes much the same as adults get.

It presents itself in different ways as it affects different organs and depending on the age of the child it can mimic different disorders. There are two distinct forms of sarcoidosis that exist in children. Older children are more commonly diagnosed with the multi system disease similar to the adult manifestations, with lymphadenopathy and pulmonary symptoms. The younger children tend to have more skin, bone and eye involvement. For both forms of the disease oral corticosteroids tend to be the leading choice of treatment which is administered in smaller doses for shorter periods of time.

Childhood sarcoidosis can be self limiting to the child or chronic, and its characteristics vary among different populations with some evidence pointing towards African American children being of higher risk for the disease. The causes are unknown but point towards bacterial, fungus, virus as well as genetics may play some part but it has not been proven as to how anyone gets the disease.

Compared to the adult population where it has shown that 10 – 40 out of 100,000 people get the disease it is much lower in children 0.22 – 0.27 out of 100,000 children are diagnosed. In children it is diagnosed more frequently between the ages of 13 – 15 with joint pains and pulmonary involvement. Younger children before the age of 10 years is a rare diagnosis because they may be asymptomatic until they get older. Usually in very young children the common symptoms are fever, fatigue, malaise and weight loss along with other symptoms from certain organs such as lungs, eyes, skin, and lymph nodes.

Lymphatic Symptoms – This presents itself with enlarged lymph nodes which are usually firm, not tender and move freely. 40 – 70% of childhood cases show this involvement.

Eyes – The child will show signs of pain, blurry vision, sensitivity, and redness in their eyes. Approximately 29% of children show eye involvement and if left untreated may cause serious damage or even blindness.

Skin – The rash most commonly seen in children with the disease are red to yellowish brown colored rash, or flat topped red papules usually on the face. You may notice large purplish scar looking formations on the trunk of the child or even nodules that are pigmented and ulcers. Children are commonly known to have rashes from many things such as diaper rash, allergies and even other illnesses so it is hard to diagnose skin involvement with out biopsy.

Bones and Joints – Symptoms are joint pain, stiffness, and lack of movement. It can affect any joint and cause a form of arthritis similar to adults. Sarcoid arthritis can be commonly confused with juvenile rheumatoid arthritis making diagnosis hard. It is thought that bone involvement is rare in children with approximately 15% to 58% of patients that have involvement in their musculoskeletal system.

Other Organs – There are many organs much like the adult form of sarcoidosis which can be affected. It shows that childhood sarcoidosis can involve the Parotid Gland, Heart, Nervous System, Kidneys, Pancreas and Liver. There are no limitations when it comes to where this disease can and can not affect.

With it being an inflammatory disease and with it affecting children which may not be able to articulate the way they are feeling, makes both care givers, doctors and patents struggle to diagnose and treat this disease.

The prognosis of childhood onset of sarcoidosis is unclear because of the rarity of the disease but however the overall prognosis is good compared to adult onset of the disease. It has been stated that when the child reaches adulthood and has gone through treatments as a younger child they may still be symptomatic but show no worsening of symptoms. This fact was stated as to the child receiving constant and proper treatment as they grow. With this also being said it has been proven that it will depend on the severity and where the disease has involved in the child.

Childhood sarcoidosis is just as severe and debilitating as adult sarcoidosis. It mimics different disease the same way in children and it causes as much discomfort and frustration to the child as it does with adults. For a parent or care giver it can be exhausting, frustrating and worrisome. Along with the lack of knowledge and resources available it proves more research must be done to find not only answers to the questions we all have but a cure.

Support both for the child and the care giver is essential to a better understanding of the disease and help comfort one another. Open communication between the child, parents or care givers and their doctors plays a big part of treatment along with the proper medications or care needed.

Categories: None

Post a Comment

Oops!

Oops, you forgot something.

Oops!

The words you entered did not match the given text. Please try again.

Already a member? Sign In

2 Comments

Reply Rachel
9:40 PM on February 6, 2012 
Thank you for this great post on childhood sarcoidosis. Here is more information on the preschool sarcoidosis:

Early onset childhood sarcoidosis (ie, with onset in the first 4 y of life) is a rare disease and appears to be different from sarcoidosis in older children and adolescents. A striking predominance of white patients is observed in early onset disease, in contrast to older children with sarcoidosis. Patients with early onset sarcoidosis frequently present in the first year of life, with the characteristic triad of rash, uveitis, and arthritis occurring in children younger than 4 years.

Hetherington et al reviewed 28 cases of early onset disease and noted that a red maculopapular rash (78%) may precede joint symptoms (58%) by months.[14] Uveitis, which occurs in about 58-90% cases of early onset disease, is described in about 20-30% of patients with later-onset disease. Pulmonary involvement, the leading feature of late-onset disease, is unusual in early onset sarcoidosis and was noted in only 22% of children.

Early onset sarcoidosis may be overlooked because of its similarity to systemic-onset juvenile rheumatoid arthritis (JRA). Both entities may be associated with systemic manifestations such as fever, weight loss, and fatigue. Skin changes may help to distinguish between the 2 diseases at the onset. The rash of JRA is pink, evanescent, and macular, whereas the rash of sarcoidosis usually is a persistent follicular or nodular rash with scaling.

Joint involvement in early onset sarcoidosis typically begins as early morning stiffness with minimal functional impairment and is characterized by painless, boggy effusions with synovial thickening that commonly affects the wrists and knees. Fusiform swelling of the finger joints and skeletal abnormalities are more common in this age group. Sarcoid arthritis typically is persistent and nondestructive, affecting predominantly the large joints; however, painful destructive polyarthritis with functional impairment indistinguishable from that associated with JRA has also been described in early onset sarcoidosis.

Uveitis is a useful differentiating feature. In JRA, the corneal cellular aggregates are more centrally located and the edges are less firm and distinct. In addition, the synechia formation is more diffuse in JRA and tends to be more focal in sarcoidosis. Furthermore, uveitis is very unusual in systemic-onset JRA, which is most often confused with early onset sarcoidosis.

The other organs involved in preschool children with sarcoidosis include liver and spleen (52%), lymph nodes (42%), parotid gland (13%), bones (13%), and lungs (13%). Because liver involvement occurs in approximately half of the cases, liver biopsy is an important diagnostic tool in patients in whom findings on other tissue biopsies are not helpful or in whom affected organs are not easily accessible.

Cardiac, renal, and central nervous system (CNS) involvement have been reported infrequently in early onset disease. Blindness is a rare, but severe, late complication in early onset sarcoidosis. Periodic ophthalmologic evaluations are essential in all cases of childhood sarcoidosis to identify ocular disease and prevent further morbidity.

Early onset sarcoidosis with involvement of the eyes, joints, and skin suggests a guarded prognosis, with the likelihood existing of a chronic, progressive course.
Reply Melany Sealy
10:57 PM on February 6, 2012 
Thank You Rachel that is great information. For someone who deals with this first hand you have much more information and a much better insight. I wish you much luck on your journey with your daughter having to go through this.

Google Translator

Upcoming Events

No upcoming events

Twitter Tweet Button

Facebook Like Button

CORPORATE SPONSORS OF N.S.O.