3. Sarcoidosis is a multisystem
inflammatory disease of unknown
etiology that predominantly affects
the lungs and intrathoracic lymph
nodes.
4. Epidemiology
All racial .
All ethnic groups.
All ages (with the incidence peaking at 20 to 39 years).
M-F ratio 2:1.
5. Clinical presentation depends on the extent and
severity of the organ involved.
Approximately 5% of cases are asymptomatic
Systemic symptoms occur in 45% of cases such as :
Fever.
anorexia
Fatigue.
Night sweats .
Weight loss .
Cough
Chest pain
6. Löfgren's syndrome, an acute presentation
consisting of:
Fever.
Arthralgia.
erythema nodosum.
occurs in 9 to 34% of patients.
9. Treatment:
Sarcoid granulomas can resolve
spontaneously without complications or heal
with residual scarring.
symptomatic treatment with NSAIDs
like ibuprofen or aspirin
11. PAGET’S DISEASE
Synonym
Osteitis deformans
Paget’s disease is named after SIR JAMES PAGET
Paget’s disease is characterised by excessive & abnormal remodeling of
bone.This excessive remodelling gives rise to bones that are extensively
vascularized, weak, enlarged & deformed with subsequent complications.
12. • ETIOLOGY- Unknown.
• Genetic link, as 7 to 10 fold increase in incidence of the disease
was observed in relatives of patients.
autosomal dominant inheritance.
Paget’s disease is characterised by enhanced resorption of bone by
giant multinucleated osteoclasts with formation of disorganized
woven bone by osteoblasts. Three phases are seen
- Lytic
- Mixed lytic & blastic
- Sclerotic
13. Clinical features
Age of occurrence
• Prevalence of the disease increases with age.
• Recognised after 50years of age.
Sex predilection
• Male to female ratio is approximately 1:1
14. PAIN-- dull constant aching pain deep below the soft tissues.
Involved bones become warm to touch because of increased
vascularity.
Other typical findings & complaints include :
pathologic fractures
non specific headaches
impaired hearing & tinnitus
15. Involvement of facial bones is occasionally seen –
LEONTIASIS OSSEA.
16. ORAL MANIFESTATIONS
• Predilection seen for maxilla – 2:1
• . The maxilla exhibits progressive enlargement,
alveolar ridge becomes widened & palate flattened.
17. The teeth present may become loose & migrate, producing
spacing.
As the disease progresses, the mouth may remain open,
exposing the teeth, because the lips are too small to cover
the enlarged jaw.
Edentulous patients with dentures commonly complain of an
inability to wear them because of increasing tightness due to
expansion of the jaw.
18. RADIOGRAPHIC FEATURES
The radiographic features of Paget’s disease are varied & depend on the stage of the
disease encountered.
Osteolytic areas are seen as multiple & diffuse or isolated destructive lesions.
The osteoblastic areas appear as radioopacities with patchy distribution.
This patchiness has been termed a ‘COTTON-WOOL’ appearance.
19. Other findings:
Generalised hypercementosis
Loss of lamina dura
Root resorption has been reported in some cases,
but this is unusual
20. LABORATORY FINDINGS
Serum calcium & phosphorous levels are usually within
normal limits.
Serum alkaline phosphatase level may be elevated, however
to extreme limits – over 250 Bodansky units.
Urinary hydroxyproline levels are elevated as they reflect
increased osteoclastic activity & bone resorption.
21. HISTOLOGIC FEATURES
The microscopic appearance of bone varies remarkably, depending
upon the stage of disease encountered.
Repeated formation & removal of bone results in the appearance of
many small irregularly shaped bone fragments that appear to be
joined in a JIGSAW or MOSAIC pattern, with deeply staining
hematoxyphilic reversal lines. This is the histologic hallmark of
Paget’s disease
22. TREATMENT & PROGNOSIS
There is no specific treatment.
Vitamin, hormone & radiation therapy have all been
utilized with sporadic reports of cures, but these have not
been confirmed.
Very promising results have recently been obtained in the
treatment of this disease by the use of calcitonin.
25. PRIMARY HYPERPARATHYROIDISM
Is the uncontrolled production of parathormone (PTH)
usually as a result of a
Parathyroid adenoma (80 to 90 % of cases)
Parathyroid hyperplasia (10 to 15%)
Parathyroid carcinoma (1%)
SECONDARY HYPERPARATHYROIDISM
PTH is continuously produced in response to chronic
low levels of serum calcium, a situation usually
associated with chronic renal disease.
26. AGE OF OCCURRENCE :
Most patients are older than 60 years of age.
SEX PREDILECTION :
Women have this condition 2 to 4 times more often than men.
CLINICAL FEATURES :
Typically the condition is identified on routine serologic testing
&
majority of patients are relatively asymptomatic.
27. Patients with classic signs & symptoms are described
as having
STONES
BONES
GROANS
MOANS
28. STONES
renal calculi (kidney stones) because of elevated serum
calcium levels.
Metastatic calcifications are also seen frequently involving other
soft tissues, such as blood vessel walls, subcutaneous soft
tissues, the sclera, dura and region around the joints.
29. BONES
Refers to a variety of osseous changes that may occur
With persistent disease, other osseous lesions develop, such as
BROWN TUMOUR OF HYPERPARATHYOIDISM.
This lesion derives its name from the colour of the tissue specimen,
which is usually a dark red-brown because of the abundant
hemorrhage & hemosiderin deposition within the tumour.
30. They commonly affect the MANDIBLE, CLAVICLE,
RIBS & PELVIS. They may be solitary but are often
multiple & long standing lesions may produce significant
CORTICAL EXPANSION.
The most severe skeletal manifestation of chronic
hyperparathyroidism is OSTEITIS FIBROSA
CYSTICA, a condition that develops from central
degeneration & fibrosis of long standing brown tumours.
31. GROANS
Refers to the tendency for the development of duodenal ulcers.
MOANS
Changes in mental status are often seen, ranging from lethargy &
weakness to confusion or dementia.
32. GENERAL RADIOGRAPHIC FEATURES :
Osteitis fibrosa cystica are localized regions of bone loss produced by osteoclastic
activity, resulting in a loss of all apparent bone structure.
Demineralisation & thinning of cortical boundaries often occur in the jaws such as the
inferior border of mandible & the cortical outlines of the maxillary sinuses
In prominent hyperparathyroidism, the entire calvarium has a granular
appearance caused by the loss of central trabeculae & thinning
of cortical plates.
33. HISTOPATHOLOGICAL FEATURES :
The brown tumour of hyperparathyroidism is histopathologically
identical to the CGCG of the jaws.
Both lesions are characterised by a proliferation of exceedingly
vascular granulation tissue, which serves as a background for
numerous multinucleated osteoclast type giant cells.
34. TREATMENT
PRIMARY - hyperplastic parathyroid tissue or
the functional tumor must be removed surgically to
reduce PTH levels to normal.
SECONDARY - signs and symptoms related to
renal calculi. Restriction of dietary phosphate &
use of phosphate-binding agents.
PARATHYROIDECTOMY
35. GIANT CELL FIBROMA
An oral tumour first described in 1974 by Weathers & Callihan as a
distinctive entity.
Unlike the traumatic fibroma, it doesnot appear to be associated with
chronic irritation.
36. CLINICAL FEATURES :
represents approximately 2 to 5% of all oral fibrous
proliferations.
AGE OF OCCURRENCE :
In about 60% of the cases, first 3 decades of life.
SEX PREDILECTION :
Some studies have suggested a slight female predilection
SITE :
Most common site is mandibular gingiva, followed by
maxillary gingiva, tongue & palate
37. The lesion usually presents as an asymptomatic
sessile or pedunculated nodule that is less than
1cm in diameter
DIFFERENTIAL DIAGNOSIS
Squamous papilloma
traumatic fibroma
Pyogenic granuloma
Peripheral giant cell granuloma
38. HISTOLOGICAL FEATURES :
Appears as an unencapsulated mass of loose fibrous connective tissue
that contains numerous chracteristic large, plump, spindle shaped &
stellate fibroblasts some of which are multinucleated.
TREATMENT & PROGNOSIS :
Conservative excisional biopsy is curative & recurrence is rare
39. 39
OSTEOBLASTOMA (GIANT OSTEOID OSTEOMA)
An osteoblastoma is uncommon, benign tumor of
osteoblasts with areas of osteoid and calcific tissue.
Rare occurance
male to female ratio is 2:1
2nd and 3rd decades
characterized clinically by pain and swelling at the tumor site
40. 40
RADIOGRAPHIC FEATURES
LOCATION:
Osteoblastomas are found in the
tooth bearing regions and around
the temporomandibular joint .
PERIPHERY:
Borders may be diffuse or may
show some thinning of cortex.
Lesions often have soft tissue
capsule around the periphery and
abnormal bone at the central
regions.
41. 41
EFFECTS ON SURROUNDING STRUCTURE:
Osteoblastomas can expand bone, but usually a thin
outer cortex is maintained.
This lesion may invaginate the maxillary sinus or
the middle cranial fossa.
42. 42
HISTOLOGIC FEATURES
many dilated capillaries scattered through out the tissues.
Moderate number of multinucleated giant cells
Active proliferating osteoblasts
TREATMENT
Osteoblastomas are treated with curettage or local excision.
Recurrence is rare.
43. In 1890, J Hutchinson described an old
man with inflamed and swollen
temporal arteries- having a peculiar
form of thrombotic arteritis.
Horton disease
GILMOUR suggested the name
GIANT CELL ARTERITIS
Affects- SUPERFICIAL TEMPORAL
ARTERY
44. CLINICAL FEATURES
AGE : older persons – 55-80 yrs
GENDER : Women > Men
CLINICAL PRESENTATION :
slow onset
Often the first sign is pain on chewing.
followed by headache , burning ,and throbbing type of pain
pain – localized in teeth, TMJ, occipital region
Ocular symptoms- loss of vision or retro orbital pain
SUPERFICIAL TEMPORAL ARTERY- sensitive to palpation
& eventually appears erythematous, swollen, tortuous.
45. TREATMENT FOR GIANT CELL ARTERITIS
•If Giant Cell Arteritis is caught early it can be treated with
steroid tablets
•Steroids help to reduce swelling.
•The blood can then flow freely to the optic nerve again.
This prevents the loss of vision.
Editor's Notes
The etiology is still unknown. Evidence exists of a genetic link, as 7 to
10 fold increase in incidence of the disease was observed in relatives of patients.
The most common presenting complaint is pain. The bone pain is perceived as a dull constant aching pain deep below the soft tissues. It may persist or exacerbate during the night.
Initial osteolytic phase is marked by disordered areas of resorption by an increased number of large osteoclasts.
These abnormal osteoclasts may contain as many as 100 nuclei.
The subsequent osteoblastic phase follows with haphazard laying of new bone matrix & formation of woven bone.
Refers to the fact that these patients, particularly those with primary hyperparathyroidism have a marked tendency to develop
Subperiosteal resorption of the phalanges of index & middle fingers
Generalised loss of lamina dura
Alterations of trabecular pattern – GROUND GLASS APPEARANCE