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Sjögren’s syndrome (SS). Defin ition : Autoimmun e inflammation of the exocrine (lacrimal, salivary, etc) glands resulting in decreased production of tears and saliva (xerophthalmia és xerostomia) .

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sj gren s syndrome ss
Sjögren’s syndrome (SS)

Definition: Autoimmune inflammation of the exocrine (lacrimal,salivary, etc) glands resulting in decreased production of tears and saliva (xerophthalmia és xerostomia).

Primary and secondary forms (the latter accompanies otherautoimmun diseases (e.g.: RA, SLE, etc).

Epidemiology:One of the most frequent autoimmune diseases;

estimated incidence: 1-10/1000, prevalence: 1%. Male: female

ratio cca 1:9.

Pathogenesis:Lymphoid infliltration of exocrine glands

(infiltrating lymphocytesare mostly CD4+ ab T cells); with

concomitant inflammation and slow destruction.

histology of salivary gland in sj gren s syndrome
Histology of salivary gland in Sjögren’s syndrome

Histology of salivary gland in Sjögren’s syndrome

parotitis in ss
Parotitisin SS


in SS

keratoconjunctivitis sicca rose bengal test in ss
Keratoconjunctivitis sicca (rose bengal test) in SS

Keratoconjunctivitis sicca (rose bengal test) in SS

schirmer s test
Schirmer’s test

Schirmer’s test

diagnostic criteria of ss ecsg 1993
Diagnostic criteria of SS (ECSG, 1993)

Diagnostic criteria of SS (ECSG, 1993)

1. Ocular symptoms (at least one the following):

a/ symptomatic dry eyes for at least 3 months

b/ repeated sensation of foreign bodies in the eyes

c/ artificial tears are required at least 3 times a day

2. Mouth symptoms (at least one of the following):

a/ symptomatic dry mouth for at least 3 months

b/ recurrent salivary gland enlargement

c/ requirement of frequent drinking while swallowing dry food

3. Positive Schirmer’s test ( 5mm/5min) (or positive rose bengal test)

4. Positive salivary gland (lower lip) biopsy. In histology at least 1 lymphoid

follicle ( 50 cells) / 4 mm2 tissue)

5. Salivary gland involvement (at least one of the following):

a/ positive scintigraphy

b/ positive parotid sialography

c/ unstimulated salivary flow ( 1.5 ml/15 min)

6. Autoantibodies (at least one of the following):

a/ SS-A (Ro) or SS-B (La) antibody

b/ antinuclear antibody (ANA)

c/ rheumatoid factor (RF)

Definite diagnosis requires at least 4 criteria. In the absence of other

(systemic) autoimmune disease, the diagnosis is primary SS.

therapy of ss
Therapy of SS
  • Therapy of SS
  • symptomatic: artificial tears, physostigmine (?)
  • systemic manifestations: myositis, pneumonitis, neuritis – corticosteroid and/or Imuran or other immunosuppressivedrugs
  • parotitis: NSAID or corticosteroid (sialolithiasis or suppurative parotitis should be excluded!)
  • B cell lymphoma with moderate malignity
  • Definition:Inflammatory/degenerative disease of the connective
  • tissues with consecutive fibrosis. Skin, vessels, and muscles are
  • involved, less commonly also internal organs (GI system, lungs, heart,
  • kidney).
  • Classification:
  • Diffuse cutaneous scleroderma (progressive systemic sclerosisnak; PSS)
  • Limited cutaneous scleroderma (acrosclerosisforms, e.g. CREST)
  • Overlap syndromes, mixedconnective tissue disease (MCTD) and undifferentiatedconnective tissue disease (UCTD)
  • Localized scleroderma (morphea & linear scleroderma)
  • Epidemiology:not rare; incidence: 19/1 million; prevalence 19-75/100,
  • 000. More common in women;in the 30-55 ys age group, male:
  • female ratio is 7-12:1.
raynaud s phenomenon
Raynaud’s phenomenon

Raynaud’s phenomenon

nailfold capillaries in scleroderma
Nailfold capillaries in scleroderma

Nailfold capillaries in scleroderma

morphea generalized
Morphea (generalized)



severe acrosclerosis
Severe acrosclerosis

Severe acrosclerosis

calcinosis in scleroderma
Calcinosis in scleroderma

Calcinosis in


teleangiectasia in scleroderma
Teleangiectasia in scleroderma

Teleangiectasia in scleroderma





centromere antibodies in acrosclerosis
Centromere antibodies in acrosclerosis

Centromere antibodies in acrosclerosis

nucleolar antibodies in scleroderma
Nucleolar antibodies in scleroderma

Nucleolar antibodies in scleroderma

bibasilar pulmonary fibrosis in pss
Bibasilar pulmonary fibrosis in PSS

Bibasilar pulmonary

fibrosis in PSS

classification criteria of scleroderma ara 1980
Classification criteria of scleroderma (ARA, 1980)
  • Classification criteria of scleroderma(ARA, 1980)
  • A/ Major criterium:
  • Proximalscleroderma: symmetric thickening, tightening, and
  • indurationof the skin of the fingers and the skin proximal to the MCP
  • or MTP joints. The changes may affect the entire extremity, face,
  • neck, and trunk (thorax and abdomen).
  • B/ Minor kritériumok:
  • 2. Sclerodactyly: as above limited to the fingers
  • 3. Digital pitted scars or loss of substance from the fingerpad: depressed
  • areas at tips of fingers or loss of digital pad tissue as a result of ischemia.
  • 4.Bibasilar pulmonary fibrosis: bilateral reticular pattern of linear or
  • lineonodular densities most pronounced in basilar portions of the lungs
  • on standard chest roentgenogram: may assume appearance of diffuse
  • mottling or “honeycomb” lung. These changes should not be attributable
  • to primarylung disease.
  • Definite diagnosis requires the major and 2 minor criteria.
scleroderma like diseases


EOSINOPHILIC FASCIITIS: (Shulman’s syndrome)

diffuse fasciitis with eosinophilia)


A mixture of SLE, scleroderma, PM, RA (SS).

Clinical picture: most prominent symptoms are:


synovitis: arthritis/arthralgia,

„sausage-like" fingers, handsand/or sclerodactyly,

esophagus motility disorder (dysphagia),

myositis (CPK elevation),

pneumonitis, pulmonary fibrosis.

Laboratory: U1-RNP antibodies


UCTD = in most cases, preceding SLE or scleroderma

therapy of scleroderma
Therapy of scleroderma
  • Therapy of scleroderma:
  • systemic: penicillamine (ineffective)
  • vasodilators (Ca-channel blockers, prostacyclin)
  • GI system: reflux - metoclopramide, slow motility – octreotid, antibiotics
  • pulmonary hypertension: ACE-inhibitors are ineffective, prostacyclin infusion
  • pneumonitis/fibrosis: corticosteroid/cytostatics
  • kidney: ACE-inhibitors
polymyositis dermatomyositis idiopathic inflammatory myopathies
Polymyositis/Dermatomyositis(Idiopathic inflammatory myopathies)

Definition:a heterogenic group, characterized by

proximal muscle weakness (involvement of shoulder and pelvic girdle).


I. Adult polymyositis (PM)

II. Adult dermatomyositis (DM)

III. Myositis associated with malignancy

IV. Childhood myositis

V. Myositis associated with systemic autoimmune disease

VI. Other (inclusion body, eosinophilic and localized)

gottron s sign in dm
Gottron’s sign in DM

Gottron’s sign in DM

gottron s sign in pm
Gottron’s sign in PM

Gottron’s sign in PM

splinter haemorrhage sign in pm dm
Splinter haemorrhage sign in PM/DM

Splinter haemorrhage sign in PM/DM

heliotrope rash in dm
Heliotrope rash in DM

Heliotrope rash in DM

dm shawl sign
DM – shawl sign

DM – shawl sign

lymphocytic infiltration in early pm
Lymphocytic infiltration in early PM

Lymphocytic infiltration in early PM

classification criteria of pm dm bohan peter 1975
Classification criteria of PM/DM (Bohan & Peter, 1975)

Classification criteria of PM/DM (Bohan & Peter, 1975)

1. symmetrical proximal muscular weakness

2. elevated serum enzymes (CPK, LDH, transaminases, aldolase)

3. Characteristic triad by EMG:

a) small amplitude, short polyphasic waves,

b) fibrillation, irritability,

c) spontaneous, bizarre discharges

4. Biopsy (=infiltration, necrosis, degenerative-regenerative signs

5. Heliotrope rash*


* Gottron’s papules or Gottron’s sign are thought to be more specific

Diagnosis:PM = 4criteria; DM =5th criterium + 4other)

autoantibodies specific for pm dm
Autoantibodies specific for PM/DM

Autoantibodies specific for PM/DM

a) anti- ‘synthetase’ antibodies, specific for ‘anti-synthetase


anti-aminoacyl-tRNA synthetase antibodies:

anti-histidyl- (= Jo-1)

anti-alanyl- (= PL-12)

anti-threonyl- (= Pl-7)

anti-isoleucyl-(= OJ)

anti-glicyl- (= EJ)

b) anti- SRP (signal recognition particle), specific for another


c) other autoantibodies:

anti-Mi-2 (antibody against a 220 kD nuclear protein)

anti-MAS (antibody against a 4S sedimentation RNA)

therapy of pm dm
Therapy of PM/DM
  • Therapy of PM/DM:
  • early diagnosis – early treatment!
  • high dose corticosteroid (CS)
  • in DM, especially when CS resistant: IVIG
  • Imuran or methotrexate
  • Other: e.g. cyclosporin
relapsing polychondritis
Relapsing Polychondritis

Definition:Rare destructing inflammatory disorder, frequently in relapsing form, affecting cartilages in many organs.

Pathogenesis:directly pathogenic autoantibodies againsttype II collagen and proteoglycan.

Diagnostic criteria of polychondritis(McAdam et al, 1976)

1. 1. symmetrical auricular chondritis

2. 2. nonerosive, seronegative polyarthritis

3. 3. nasal chondritis

3. 4. eye involvement (conjunctivitis, keratitis, scleritis/episcleritis, uveitis)

5. 5. Upper airway (larynx-, trachea-) chondritis

6. 6. Cochlear and/or vestibular functional impairment (hearing loss, tinnitus, vertigo)

Diagnosis: 3 (or more) criteria present.

Therapy: CS, cyclosporine

polychondritis of the ear
Polychondritis ofthe ear

Polychondritis of

the ear