Connective tissue disorders - criteria

 Systemic sclerosis

Major

Thickening of the skin of the hands

Minor

1)Sclerodactyly (i.e., the changes of the major criterion but limited to the fingers)

2)Digital pitting scars or loss of substance from the finger pad: depressed areas at tips of fingers or loss of digital pad tissue as a result of ischemia

3)Bibasilar pulmonary fibrosis

*The major or ≥ 2 minor criteria required for diagnosis.

RHEUMATOID ARTHRITIS

(American Rheumatism Association revised criteria )

1)Morning stiffness (lasting at least 1 hr)

2)Arthritis (soft tissue swelling or fluid) of 3 or more joints (PIP, MCP,wrist, elbow, knee, ankle, MTP joints)

3)Arthritis of hand joints (swelling of at least 1 wrist, MCP, or PIP joint)

4)Symmetrical arthritis (i.e., simultaneous arthritis of the same joints on both sides of the body)

5)Rheumatoid nodules

6)Serum rheumatoid factor positivity (at a level such that < 5% of normal controls are positive)

7)Radiographic hand or wrist changes typical of rheumatoid arthritis

At least 4 criteria for a minimum of 6 weeks

SLE (American College of Rheuma￾tology criteria )

1)Malar rash

2)Discoid rash

3)Photosensitivity skin rash

4)Oral or nasopharyngeal ulceration

5)Non erosive arthritis involving ≥ 2 peripheral joints

6)Serositis (pleuritis or pericarditis)

7)Renal disorder (persistent proteinuria or cellular casts)

8)Neurologic disorder (unexplained seizures or psychosis)

9)Hematologic disorder (hemolytic anemia, leukopenia, lymphopenia,or thrombocytopenia)

10)Immunologic disorder (positive LE cell, anti-DNA antibody, anti-Sm antibody, false-positive syphilis serology)

11)Elevated antinuclear antibodies

*Minimum of 4 criteria required.

POLYMYOSITIS WITH DERMATOMYOSITIs

1)Symmetrical proximal muscle weakness

2)Muscle biopsy specimen showing myositis

3)Elevation of serum skeletal muscle enzymes

4)Characteristic electromyographic pattern of myositis

5)Typical rash of dermatomyositis

definite diagnosis - first four features are present

Probable diagnosis - if any three of the first four features are present

 possible diagnosis - if any two of the first four features are present

MIXED CONNECTIVE TISSUE DISEASE

Presence of antibody to the U1 ribonuclear protein together with the clinical features of hand edema, synovitis, Raynaud phenomenon, acrosclerosis, and myositis

At least three of these clinical features are needed in addition to the autoantibody finding.

A fourth clinical feature is required if the initial three are Raynaud phenomenon, edema, and acrosclerosis

RELAPSING POLYCHONDRITIS

The diagnosis of relapsing polychondritis requires the presence of three or more of the following clinical features269:

bilateral auricular chondritis nonerosive seronegative inflammatory polyarthritis

nasal chondritis

 ocular inflammation

respiratory tract involvement (either upper or lower respiratory tract)

cochlear with or without vestibular abnormality

 positive biopsy specimen

The presence of anti cartilage antibodies may be helpful in the diagnosis 

BEHÇET SYNDROME

Major (required)

Recurrent aphthous ulceration at least 3 times in a 12-mo period

Minor (2 of 4)

Recurrent genital ulceration

Ocular disease

Skin lesions (erythema nodosum, skin ulcers)

Positive pathergy test (a 2-mm erythematous papule or pustule att he prick site 48 hr after the application of a sterile hypodermic 20- to 22-gauge needle that obliquely penetrated avascular antecubital skin to a depth of 5 mm)

SJÖGREN SYNDROME

 sicca symptoms are mandatory

 supportive evidence including ocular signs (positive Schirmer test testing reduced tear formation, rose bengal score > 3 for staining of conjunction and cornea)

 typical histologic appearances  salivary gland biopsy

antibodies to Ro (SS-A) or La (SS-B) or

 reduced salivary flow.