Sarcoidosis Flashcards
What is Lofgren syndrome?
It is a variant of sarcoidosis presenting with Fever hilar adenopathy Arthritis Erythema Nodosum
They often require supportive measures and occasionally systemic corticosteroids.
Spontaneous resolution occuring over 1-2 years.
what is Darier-Roussy sarcoidosis ?
A variant of sarcoidosis
present with painless firm subcutaneous nodules or plaques without epidermal changes.
This variant represents sarcoidosis limited to the PANNICULUS , and is often associated with SYSTEMIC SARCOIDOSIS.
What is Lupus Pernio ?
Why is it important to recognize it ? what systemic manifestation is it associated with ?
A variant of sarcoidosis represent as papulonodules and plaques, which are often on the nose and cheeks and have a violaceous color, along with scale; there may be a
beaded appearance along the nasal rim (see Fig. 93.3C igl ).
Recognition of lupus pernio is important because of its association with
chronic sarcoidosis of the lungs (-75% of patients) and of the upper respiratory tract (-50% of patients).
What type of sarcoidosis is erythema nodosum associated with ?
Erythema nodosum is associated with subacute, transient sarcoidosis that usually
resolves spontaneously and typically does not require systemic corticosteroid therapy.
In general, there are no additional cutaneous manifestations.
What are the nail changes associated with sarcoidosis ?
Clubbing
Subungual hyperkeratosis
Onycholysis
What is Heerfordt syndrome ?
Heerfordt syndrome (uveoparotid fever) includes parotid gland enlargement, uveitis, fever, and cranial nerve palsies, usually of the facial nerve .
What is the % of lung involvement in sarcoidosis ?
What is end stage of pulmonary sarcoidosis?
Lung disease occurs in -90% of patients.
Ranging from alveolitis to granulomatous infiltration of the alveoli, blood vessels,
bronchioles, pleura, and fibrous septa.
The end stage of pulmonary sarcoidosis is fibrosis with bronchiolectasis and
“honeycombing” of the lung parenchyma.
Hilar and/or paratracheal lymphadenopathy, which is usually asymptomatic, occurs in 90% of patients.
How does childhood sarcoidosis present ?
Childhood sarcoidosis is rare
it usually presents with a triad of arthritis, uveitis and cutaneous lesions, along with constitutional symptoms.
Peripheral lymphadenopathy is frequently present,
but pulmonary involvement is less common than in adults.
If sarcoidosis is being considered in a child, it is important to exclude Blau syndrome (see Ch. 45 s ).
True or false :
Sarcoidosis is a diagnosis of exclusion, both clinically and histologically.
True
What is the characteristic pathology of sarcoidosis?
The histopathologic hallmark of sarcoidosis is the presence of
superficial and deep dermal epithelioid cell granulomas devoid
of prominent infiltrates of lymphocytes or plasma cells (“naked
tubercles”) (Fig. 93.6 tg ).
Central caseation is usually absent, although fibrinoid deposition may be observed in up to 10% of
cases.
Multinucleated histiocytes (“giant cells”) are usually of the Langhans type, with nuclei arranged in a peripheral arc or circular fashion.
What are asteroid bodies?
The giant cells may contain eosinophilic stellate inclusions known as asteroid bodies (Fig. 93.7 t© )
Asteroid bodies represent engulfed collagen.
What are Schaumann bodies ?
Giant cells in sarcoidosis may include rounded laminated basophilic inclusions known as Schaumann bodies
Schaumann bodies likely represent degenerating lysosomes.
neither Asteroid bodies nor Schaumann bodies is specific or required for the diagnosis.
Does the prescence of a polarizable material exclude the diagnosis of sarcoidosis ?
Notably, up to 20% of biopsies of sarcoidosis contain polarizable material; therefore its presence does not exclude the diagnosis.
What are Epithelioid histiocytes ?
Epithelioidcells are transformed or activated macrophages orhistiocytes, formed in response to various inflammatory mediators. Histologically, they appear as cells with abundant eosinophilic cytoplasm with indistinct cell margins and elongated or slipper-shaped vesicular nuclei with prominent nucleoli
What is the usefullness of ACE levels ?
(ACE) level is elevated in -60% of patients; it has a false-
positive incidence of 10%, making it a more useful test for
monitoring disease progression than for establishing the
diagnosis. An ACE level >2-3 times the upper limit of normal is
more suggestive of sarcoidosis.
NCGs ( non xaseating granuloma) secrete ACE, which may function as a cytokine.
What is the DD of annular sarcoidosis?
The differential diagnosis of annular lesions is wide , see Table 19.1
1) Granuloma annulare
2) Annular elastolytic giant cell granuloma
3) Interstitial granulomatous dermatitis (IGD); interstitial granulomatous drug reaction (IGDR).
4) Borderline or tuberculoid leprosy
What are the common drugs associated with drug-induced cutaneous sarcoidosis ?
(e.g. IFN-a for hepatitis C viral infection, TNF-a inhibitors).
What must be excluded when diagnosing sarcoidosis before initiating immunosuppressive agents ? And How ?
Must exclude infectious etiologies.
Special stains for acid-fast and fungal organisms should be obtained.
When clinically appropriate, tissue culture should be performed.
Increasingly, PCR is being utilized to exclude infections, including mycobacterial.
Both TUBERCULOD LEPROSY LUPUS VULGARIS are in the differential diagnosis and for the
atter, a QuantiFERON®-TB Gold test may provide additional information.
How to diagnose sarcoidosis ?
Sarcoidosis is a diagnosis of exclusion, both clinically and histologically.
In order to establish the diagnosis, a supportive
clinical history must be accompanied by the histologic
presence of non-caseating granulomas in at least one organ
svstem.
Details of systemic manifestations are outlined in Table 93.3
What are the ocular manifestations of sarcoidosis ? % inolvement ?
25%
Uveitis (can be asymptomatic despite being severe),
conjunctivitis
sicca symptoms
Yearly opthalmologic exam
What are neurologic manifestations of sarcoidosis ?
Neuropathies -cranial, spinal cord, peripheral, small fiber .
. Dictated by symptoms (e.g. MRI, nerve conduction studies)
Referral to neurologist
What is % of bone marrow involvemennt and manifestation
50%
. Lymphopenia (+CD4:CD8 ratio), leukopenia, eosinophilia,
hypergammaglobulinemia, nonhemolytic anemia (5%)
Elevated risk of developing lymphoma is debatable**
CBC , SPEP
% and lung manifestations in sarcoidosis
90-95%
Dyspnea, non-productive cough/pulmonary infiltrates, fibrosis, restrictive lung disease (+VC, +RV, +TLC,
reduced DLCO)
CXR, high resolution chest CT scan (more sensitive than CXR),
PFTs that include DLCO
Lymph node involvement in sarcoidosis
30-40 % peripheral Lymphadenopathy
99% hilar Lymphadenopathy
Lymphadenopathy/enlarged hilar and/or paratracheal LN
CXR, high resolution chest CT scan (more sensitive than CXR)
Kidney
10-40%
Nephrolithiasis/hypercalauria , reduced renal function
BUN, crt, serum calcium, spot urine calcium : crt ratio,
24-hour urine to calcium excretion
Referral to nephrologist
Endocrine
5-10
Pituitary or thyroid dysfunction
Hypercalcemia (increased calcitriol synthesis by sarcoidal histiocytes)
Thyroid function tests
Expanded hormonal testing when clinically indicated
Bone and joints/muscles
Bones : 5-10
Usually asymptomatic/lytic
bone lesions
Radiography
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Joints/muscles 5-10
Arthritis, weakness (up to a third of patients have severe fatigue), myopathy
Referral to rheumatologist
Upper respiratory tract , including sinuses
5-10
Sinusitus, nasal congestion,
stridor, parotiditis
Referral to otolaryngologist and/or dedicated imaging
Heart
25 (5% clinically
relevant)
Palpitations, sudden death,
CHF/arrhythmias, cardiomegaly
EKG,
echocardiogram,
Holter monitor
If any abnormalities on history, physical examination, or initial screening,
referral to cardiologist and additional testing (PET scan, cardiac MRI)
Liver /Spleen
10-20
Hepatomegaly and/or splenomegaly (rarely clinically relevant), cirrhosis,
consequences of splenic enlargement (e.g. thrombocytopenia)/
Elevated LFTs,
Reduced Plts
LFTs, physical examination
If clinically relevant hypersplenism is suspected
abdominaI/pelvic CT scan (lymphadenopathy common finding)
Otther histology variants un sarcoidosis ?
Yes ,there are unusual cases with dense lymphocytic and plasmacytic infiltrates around and
within the nodular histiocytic aggregates.
aggregates may extend into the subcutaneous fat, producing the clinical features of Darie-Roussy sarcoidosis.
Histologic mimics of sarcoidosis
Other histologic mimics include foreign body reactions to zirconium, beryllium, silica, tattoo ink, or soft tissue granulomatous mycosis fungoides Hodgkin disease granulomatous rosacea cutaneous Crohn disease, Blau syndrome (see Ch. 45 ) cheilitis granulomatosa, and the sarcoidal reaction to an underlying lymphoma
