Löfgren’s Syndrome: A Clinical Variant of Sarcoidosis

[Mr. Nine]

<b>Löfgren’s syndrome</b> is an acute variant of sarcoidosis
characterized by the triad of erythema nodosum, bilateral hilar
adenopathy, and arthritis or arthralgias.
Unlike sarcoidosis, which is usually a chronic disease requiring long-
term cortico- steroid treatment, Löfgren’s syndrome has an excellent
prognosis.

<i>Note: <b>Loffler's syndrome</b> is a cause of pulmonary
eosinophilia and thought to be caused by parasites such as Ascaris
lumbricoides.</i>


Presentation
It is characterized by enlargement of the lymph nodes near the inner
border of the lungs (called "hilar lymphadenopathy") as seen on x-ray,
and tender red nodules (erythema nodosum) are classically present on
the shins. It may also be accompanied by arthritis and fever.
Löfgren syndrome consists of the triad of erythema nodosum, bilateral
hilar adenopathy on chest radiograph, and arthritis.

Prognosis
Löfgren syndrome is associated with a good prognosis,with >90% of
patients experiencing disease resolution within 2 years.
In contrast, patients with the disfiguring skin condition lupus pernio
or cardiac or neurologic involvement rarely experience disease
remission.

Genetics
Recent studies have demonstrated that the HLA-DQB1*0201 is strongly
associated with Löfgren syndrome.
It is associated with HLA-DRB1*03.

Lofgren's syndrome, a type of sarcoidosis, is more common in people of
<b>European descent</b>. Lofgren's syndrome may involve fever,
enlarged lymph nodes, arthritis (usually in the ankles), and/or
erythema nodosum. Erythema nodosum is a rash of red or reddish-purple
bumps on your ankles and shins. The rash may be warm and tender to the
touch.

Sarcoidosis is somewhat more common in women than in men. The disease
usually develops between the ages of 20 and 50.

People who have certain jobs also may be at higher risk for
sarcoidosis. Examples include:

* Health care workers
* Elementary and secondary school teachers
* People whose jobs expose them to agricultural dust,
insecticides, pesticides, or mold
* Suppliers of building materials, hardware, or gardening
materials
* Firefighters

People who have a family history of sarcoidosis also are at higher
risk for the disease.

<i>Sarcoidosis affects people of all ages and races. However, it's
more common among African Americans and Northern Europeans. In the
United States, the disease affects African Americans somewhat more
often and more severely than Whites.
Studies have shown that sarcoidosis tends to vary in different ethnic
groups. For example, <b>eye problems</b> due to the disease are more
common in Japanese people.</i>

For more information, please visit http://ajrccm.atsjournals.org/cgi/content/abstract/179/4/307
Thank you

[Mr. Nine]

Thank u doctor for ur questions.

I mentioned for sarcoidosis as this disease affect more in health care
workers and school teacher. I didn't mean for Lofgren's Syndrome. As u
know sarcoidosis is a chronic granulomatous inflammation of unknown
cause, but maybe associated with immune system & immune response, so
these people (health care workers and school teacher, firefighters,
etc ) have higher risk for this disease due to occupational hazards.
But to occur Lofgren's $, it is strongly associated with HLA.
I would like to apologise for my bad presentation which confuses
readers between the different forms of diseases - Sarcoidosis &
Lofgren's $. I will try my best in my next discussion to present
clearly.

Yes, hilar nodes is one of the diagnostic triad, so it can't be
diagnosed without X-rays. But in cases of erythema nodosum plus fever
with or without obvious joint inflammation, we should suspect
Lofgran's $ and should be done X- rays if available. If not available,
we must consider this $, in every step of management in such patients.

We don't need to give 2 yrs course of steroid therapy, because this
disease is an acute course( unlike sarcoidosis, which is chronic
disease requiring long-
term cortico- steroid treatment), the line of management is short term
bed rest & NSAIDs in most cases. In some cases with parenchymal
pulmonary involvement, non- caseating granulomatous skin lesions,
hypercalcaemia, and severe arthritis, we need to treat with
corticosteroids but not long term, only with short course to control
severe symptoms. Most patients had remission of disease within 3 to 12
months after treated conservatively.

thank u for ur discussion.


On Sat, Nov 14, 2009 at 2:42 PM, winmin latt <minl...@gmail.com>
wrote:

can i ask u some questions?
why does this disease affect more in health care workers and
school teacher?(as u said, it is strongly associated with HLA)
can it be diagnosed without X-rays?(for the hilar lymph
nodes ,which is one of the diagnostic triad)
how do we give 2 yrs course of steroid in menopausal women?( high
risk person for osteoporosis)

[winmin latt]

very thank
i am very glad that i know about Lofgren 's Syndrome a little
it is very kind of u  to reply and explain what i keen to know
OK ,wishing u be able to present more!

[Doctor . Htet Aung]

Differential Diagnosis of Erythema Nodosum

Etiology                        Cases,%
(1) Idiopathic                      34
(2) Sarcoidosis/Lofgren's $  22
(3) URTI(viral)                     20
(4) Group A beta-
     haemolytic streptococcal
     pharyngitis                   7
(5) Tuberculosis                 5
(6) Drugs (penicillin, sulfa,
     Oral contraceptives)      3
(7) Other                           9
     - Inflammatory bowel disease
     - Behcet's syndrome
     - Sweet's syndrome
     - malignancy                          


Data from Garcia- Porrua et al.