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Sarcoidosis Flashcards

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1
Q

Introduction

A

Multisystem granulomatous disease of unknown aetiology
Mainly skin, eyes, lungs, mediastinal and peripheral lymph nodes

Skin lesions may be disfiguring —> strong psychosocial impact

One of the great imitators - variable cutaneous manifestations

Specific (histopathologically display sarcoidosis granulomas) vs non-specific lesions

Women > men

African Americans/Indians - more severe and chronic disease
Caucasians - asymptomatic disease, Lofgren syndrome

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2
Q

Associated diseases

A

Increased risk of lymphoproliferative disease I.e. Hodgkin lymphoma

Concomitant autoimmune disease _

  • Sjogren syndrome
  • systemic sclerosis
  • RA
  • vasculitis
  • psoriasis
  • autoimmune chronic hepatitis
  • primary biliary cirrhosis
  • autoimmune thyroid disease esp Graves disease
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3
Q

Systemic manifestations

A

Lung

Extrapulmonary -

  • Eye
  • Reticuloendothelial (lymph nodes, spleen, bone marrow)
  • Liver
  • MSK
  • Heart
  • Other I.e. upper respiratory tract, parotid, hypercalcaemia/hypercalciuria, renal, GI, genital, endocrine

Acute/subacute (weeks/few months) vs insidious onset (several months)

Acute/subacute = good prognosis, Mild constitutional symptoms -

  • Fatigue
  • Malaise
  • Anorexia
  • Weight loss
  • Low grade fever
  • Arthralgia
  • Respiratory symptoms

Insidious = chronic course + permanent damage, respiratory complaints w/o constitutional symptoms/symptoms referable to organs other than the lung

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4
Q

Lung sarcoidosis

A

90% of cases
Dry cough, SOB on exercise vs asymptomatic

Grading by CXR stages -
0 - normal
I - bilateral hilar lymphadenopathy w/o lung involvement
II - bilateral hilar lymphadenopathy w/ lung involvement
III - lung involvement w/o bilateral hilar lymphadenopathy (less likely to resolve)

Advanced cases - pulmonary HTN, fibrosis, destruction/obliteration lung vasculature, chronic hypoxaemia

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5
Q

Eye sarcoidosis

A

May be asymptomatic —> refer to Opthal for all patients for slit lamp and ophthalmoscope exam

Anterior/posterior uveitis
Chorioretinitis
Periphlebitis
Papilloedema
Retinal haemorrhage
Keratoconjunctivitis sicca
Lacrimal gland involvement

Late complications in untreated cases -
Secondary glaucoma
Cataract
Blindness

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6
Q

Reticuloendothelial sarcoidosis

A

Peripheral LN - cervical, supraclavicular, axillary, inguinal

Intrathoracic LN

Mesenteric LN

Retroperitoneal LN

Spleen - hypersplenism, pancytopenia

Bone marrow (rare)

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7
Q

Liver sarcoidosis

A

Mild hepatomegaly + mild cholestasis

Severe chronic cholestasis syndrome (rare)

Portal HTN (rare)

Budd-Chiari syndrome (rare)

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8
Q

Neurosarcodosis

A

Cranial nerve involvement —> facial paralysis (common)

Aseptic meningitis
Seizures
Pyramidal tract 
Optic nerve dysfunction
Papilloedema
Hypothalamic/pituitary lesions —> diabetes insipidus, hypopituitarism
Cognitive impairment
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9
Q

MSK sarcoidosis

A

Transient vs chronic polyarthralgias (arthritis uncommon)

Asymptomatic muscle involvement (symptomatic diffuse/nodular myopathy rare)

Osteolytic bone lesions on hands and feet (uncommon)

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10
Q

Heart sarcoidosis

A

Arrhythmias (supraventricular, ventricular)

Complete heart block

Sudden death

Congestive heart failure

Cor pulmonale (secondary to chronic pulmonary fibrosis)

Ix - cardiac MRI/PET scan

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11
Q

Other organ sarcoidosis

A

Upper respiratory tract —> nasal stuffiness

Parotid - bilateral parotid enlargement, xerostomia

Transient/persistent hypercalcemia and hypercalciuria due to increased production of 1,25 dihydroxyvitamin D by granulomas —> nephrocalcinosis, nephrolithiasis

Renal - interstitial lymphocytic and granulomatous nephritis

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12
Q

Course/prognosis

A

Acute sarcoidosis resolves spontaneously without sequelae within 2-5 years

Lofgren syndrome -
excellent prognosis
Recurrence after spontaneous remission

Chronic progressive sarcoidosis sometimes assoc with irreversible fibrotic changes

Pregnancy not C/I except in severe chronic disease

Relapse post partum

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13
Q

Specific lesions (histopathologically display sarcoidosis granulomas)

A 
Maculopapules
Nodules and Plaques
Lupus pernio
Scar sarcoidosis
Subcutaneous sarcoidosis

Red-brown/red-violaceous in colour
Multiple
Asymptomatic
Diascopy - subtle brown-yellow/apple jelly colour

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14
Q

Non-specific lesions

A

Erythema nodosum

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15
Q

Maculopapular sarcoidosis

A

Most common specific lesion
Transient, appear to herald onset of sarcoidosis
Yellow-brown/red-brown
No clinically evident epidermal change
Face - mainly periorbital, nasolabial folds
Other sites also including mucous membranes
Papular sarcoidosis of the knees - frequently with linear arrangement and Lichenoid appearance, polarisable foreign bodies in most biopsies, transient
Resolve spontaneously or < 2yrs with treatment without significant scarring (favourable prognosis)
Commonly assoc with acute systemic sarcoidosis - hilar LN, peripheral LN, erythema nodosum, acute uveitis, parotid enlargement

DDx - 
Xanthelasma
Rosacea
Secondary syphilis
LE
Trichoepithelioma
Sebaceous adenoma
Syringoma
GA
Lichen nitidus
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16
Q

Nodular and plaque sarcoidosis

A

Multiple round/oval infiltrated reddish-brown plaques
Thicker, more indurated, persistent than papules
Sometimes mamillated (rounded protuberances)
Tend to recur after treatment
Can leave permanent scarring when lesions resolve
Assoc with chronic systemic sarcoidosis - pulmonary fibrosis, peripheral LN, splenomegaly, chronic uveitis
Disease activity >2 yrs

DDx - 
Lupus vulgaris 
NLD
GA
Leprosy
Morphoea
DLE
Leishmaniasis
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17
Q

Lupus pernio

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A

Infiltrated red-violaceous plaques

Disfiguring

Nose, cheeks - prominent telengiectatic component 
Ears
Lips
Forehead
Fingers

Painless
Doesn’t tend to ulcerate

Assoc with chronic course -
Sarcoidosis of upper respiratory tract (esp when there is involvement of nasal rim) - unique to lupus pernio
Bone cysts esp terminal phalanges - unique to lupus pernio
Pulmonary fibrosis
Chronic uveitis

DDx -
Rosacea
Lupus vulgaris
DLE

18
Q

Scar sarcoidosis

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A

Involve old scars from surgery, trauma, acne, gene puncture, vaccination, herpes zoster, Mantoux test

Old scars become infiltrated and red —> sarcoidal granulomas histopathologically

Frequently on knees

Tends to follow activity of the disease I.e. appear at onset

Assoc with -
Chronic pulmonary and mediastinal involvement
Peripheral LN
Uveitis
Bone cysts
Parotid infiltration

Tattoo sarcoidosis is a variant - occurs decades after tattooing (DDx foreign body reaction to tattoo pigment)

19
Q

Subcutaneous sarcoidosis

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A

Sarcoidosis granulomas limited to subcut tissue

Multiple indurated subcut nodules in the extremities
Covered by normal-appearing skin
Mostly involves forearms with indurated linear bands from elbow to hand (fusiform)
Painless/non-tender

Appears at the onset of systemic sarcoidosis

Assoc with stage I sarcoidosis CXR change
Favourable prognosis - systemic sarcoidosis < 2 yrs

DDx - 
Epidermal cyst
Lipoma
Calcinosis
Rheumatoid nodules
Morphoea
Cutaneous mets
20
Q

Less common forms of cutaneous sarcoidosis

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A 
Angiolupoid sarcoidosis 
Hypopigmented sarcoidosis
Lichenoid sarcoidosis
Ulcerative sarcoidosis
Psoriasiform sarcoidosis
Verrucous sarcoidosis
Ichthyosiform sarcoidosis
Erythrodermic sarcoidosis
Necrobiosis-lipoidica like lesions
Morphoea-like lesions
Livedo
21
Q

Angiolupoid sarcoidosis

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A

Some consider this to be discrete variant of lupus pernio with prominent large telengiectatic venules
Single raised plaque on bridge of the nose, entrap face, ears, scalp

22
Q

Hypopigmented sarcoidosis

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A

Hypopigmented
Well demarcated round to oval patches
Some lesions —> Erythematous papules in the centre of the hypopigmented patch —> fried egg appearance
Limbs

Interface dermatitis assoc with sarcoidal granulomas may explain hypomelanosis

23
Q

Lichenoid sarcoidosis

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A

More frequent in children
Small flat topped, dome shaped red/skin coloured papules
Wickham striae absent
Extensive on trunk, limbs, face

24
Q

Ulcerative sarcoidosis

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A

Usually develops on papulonodular/atrophic lesions on the lower legs
Heals with scarring

25
Psoriasiform sarcoidosis
May be clinically indistinguishable from psoriasis | Though psoriasis is salmon pink, have larger scale, heals without scarring
26
Verrucous sarcoidosis
Lower extremities | Assoc with African descent and long-standing systemic sarcoidosis
27
Ichthyosiform sarcoidosis
Adherent polygonal grey brown scales Lower extremities African descent Compact orthokeratosis, diminished granular layer, sarcoidosis granulomas
28
Erythrodermic sarcoidosis
Infiltrated red plaques coalescing over large areas | Some areas of skin spared
29
Necrobiosis lipoidica like lesions
Pink-violaceous plaques with depressed centres | Shins
30
Morphoea-like lesions
Indurated atrophic plaques Some show linear distribution resembling linear morphoea Dermal sclerosis + sarcoidal epitheliod granulomas
31
Livedo
Japanese women Epitheliod granulomas around blood vessels with luminal narrowing Eye, CNS involvement
32
Special locations of specific cutaneous lesions of sarcoidosis
Scarring alopecia Nail changes - assoc with bony cysts of terminal phalanx, chronic course - Splinter haemorrhage - Thinning - Thickening - Subungual hyperkeratosis - Pitting - Longitudinal ridging - Trachyonychia - Onycholysis - Total loss of nail - Pterygium - Paronychia Oral - Diffuse submucous thickening Firm nodule buccal mucosa Genital - testicular/epidydymal mass - vulva
33
Non specific sarcoidosis lesions
Erythema nodosum (most common) - Frequently initial manifestation of sarcoidosis - Marker of acute, benign, self-limited sarcoidosis - Lofgren syndrome (erythema nodosum, bilateral hilar and right paratracheal LN +/- pulmonary infiltrates) - may be accompanied by fever, polyarthralgia, uveitis - very good prognosis, resolves spontaneously within 1 yr Erythema multiforme Prurigo Cutaneous calcinosis Clubbing - poor prognostic sign Pseudo-clubbing (granulomatous infiltrate of the terminal phalanx) Sweet syndrome - assoc with onset of acute sarcoidosis
34
Investigations/basic assessment
History including occupational, environmental exposures Physical exam Ophthal exam - slit lamp, ophthalmoscopic exam CXR Standard haem, biochem profiles - - FBC - LFT - UEC - Urine and serum calcium level - Serum ACE - 60% increased level ECG PFT including spirometry and diffusing capacity for carbon monoxide (DLCO) - decreased forced vital capacity, DLCO Tuberculin skin test - negative Biopsies for histo and culture for mycobacteria and fungus If requiring oral steroids - baseline bone mineral density test Consider High res CT if CXR has atypical findings (not indicated when CXR has typical sarcoidosis findings) - Bilat hilar LN, widespread pulmonary infiltrates Consider F-FDG PET scan - detection of occult granuloma sites for biopsy, assess activity and extension of sarcoidosis, detect residual activity in those with fibrotic pulmonary sarcoidosis
35
Diagnostic criteria
Based on compatible clinical + radiological picture Demonstration of non-caseating granulomas —> negative cultures for mycobacteria, fungus When clinical + radiological findings not typical esp with stage 0 CXR —> advisable to obtain at least 2 positive biopsies (transbronchial, skin, peripheral LN) Histo confirmation may not be necessary in Lofgren syndrome (recognisable)
36
Management for systemic sarcoidosis
Treatment of choice = Oral pred 30-40mg/d, with gradual reduction to 5-10mg/d or 10-20mg/48hr for at least 1 yr 1mg/kg/d may be required for severe uveitis, neurosarcoidosis, symptomatic cardiac involvement Steroid sparing agent can be considered if oral pred cannot be withdrawn - MTX for both skin + lung disease Cutaneous lesions frequently recur when tapering oral pred used for systemic sarcoidosis When no significant systemic sarcoidosis to justify oral pred, options for skin sarcoidosis not completely effective Rx may be unnecessary for cosmetically insignificant/asymptomatic skin lesions
37
First line Rx for mild to moderate skin disease
Potent TCS | ILCS triamcinolone acetonide 5-20mg/mL every 3-4 weeks
38
First line Rx for severe skin disfigurement/lupus pernio
Oral pred 20-60mg/d until clinical response (typically 1-3 months), then tapered by 5-10mg/wk to the lowest dose that prevents relapse If requiring at least 10mg/d oral pred, add steroid sparing agent Osteoporosis - - Oral calcium to prevent osteoporosis (increased risk in sarcoidosis, oral pred) if no hypercalcemia/nephrolithiasis - Bisphosphonates to treat steroid-induced osteoporosis - Use of vitamin D controversial due to increased production of vit D from granulomas that causes hypercalcaemia/hypercalciuria
39
Second line Rx for mild to moderate chronic/recalcitrant skin disease
1st Rx to be initiated = Tetracycline (Minocycline 100mg BD for 3 months) - poor response in lupus pernio If unsatisfactory to 3 months minocycline —> add Antimalarials (HCQ 200-400mg/d, chloroquine 250-500mg/d) If no improvement with HCQ —> add MTX (Response may take at least 6 months)
40
3rd line Rx for refractory skin sarcoidosis
Infliximab | Adalimumab
41
Other non-systemic Rx for skin sarcoidosis
Topical Rx- - Topical tacrolimus - Topical psoralen gel Physical Rx - - UVA1 - PDT - CO2 laser - PDL - RTX Surgery
42
Cutaneous/idiopathic sarcoid reaction
Skin sarcoid granulomas of unknown aetiology Absence of other organ involvement Important to follow up —> Some will develop involvement of other organs and fulfil criteria for sarcoidosis

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