Connective Tissue Disease (CSQ)

The Connective Tissue Disease Screening Questionnaire (CSQ) is a highly reproducible, sensitive, and moderately specific instrument for screening subjects with potential Connective Tissue Disease (CTD).
Note that musculoskeletal symptoms and diseases were also assessed in general within Lifelines (section: diseases & symptoms).

Background

Epidemiologic studies of the incidence of connective tissue diseases (CTDs) or their potential associations with exogenous risk factors are limited by the low prevalence, diverse manifestations, lack of specific diagnostic tests, and the expense associated with confirming the diagnosis. Screening by questionnaires such as the CTQ is a useful, validated, efficient case-finding strategy for systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), and ankylosing spondylitis.

The CSQ was developed in English and translated to Dutch. It has 30 items and can be completed within 30 minutes.
For all intended purposes a person who does not meet the CTD criteria by the CSQ, as described below, does not have a CTD. The instrument can be used to identify a smaller group for subsequent detailed, specific examination. It should prove useful in population studies of the incidence, prevalence, and etiology of CTDs¹⁾.

Diagnostic criteria

An algorithm for identifying potential CTD based on the ACR or published clinical criteria was used.

CTD	Diagnostic criteria
Rheumatoid Arthritis (4 out of 6 criteria)	swelling of wrist, metacarpophalangeal, or proximal interphalangeal joints for more than 6 weeks
	swelling of three or more joints for more than 6 weeks
	swelling of wrist, metacarpophalangeal, or proximal interphalangeal joints for more than 6 weeks
	symmetric joint swelling
	rheumatoid nodules
	positive test result for rheumatoid factor.
	X-ray findings were not ascertained.
Lupus (SLE) (4 out of 11 criteria)	malar rash
	discoid rash
	photosensitivity
	oral ulcers
	arthritis
	serositis
	proteinuria
	hematologic disorder (anemia, leukopenia, low platelet count)
	positive antinuclear antibody (ANA) titer
	alopecia and Raynaud’s phenomenon*
	seizures and psychosis were excluded*
Scleroderma (1 major or 2> minor criteria)	major: skin thickening proximal to the metacarpophalangeal joints
	minor: sclerodactyly, digital pitting scars or loss of substance from the finger pad
	minor: pulmonary fibrosis
polymyositis or dermatomyositis (2 out of 4 criteria)	muscle weakness for more than 3 months
	upper extremity proximal muscle weakness for more than 3 months
	lower extremity proximal muscle weakness for more than 3 months
	a history of an elevated muscle enzyme level (creatinine phosphokinase)
	electromyography and muscle biopsy results were not ascertained.
Sjögren's syndrome (1 of 2 criteria)	dry eyes and dry mouth
	positive serologic test results (ANA or rheumatoid factor) plus dry eyes or dry mouth
	lip biospy, Rose-bengal staining, and Schirmer’s test results were not ascertained.
Raynaud’s phenomenon	cold sensitivity plus white, blue, or purple color changes of the fingers on exposure to cold
Mixed connective tissue disease (4 out of 5 criteria)	synovitis as defined by the RA algorithm (see above)
	hand edema
	Raynaud’s phenomenon (see above)
	myositis as defined by the polymyositis/dermatomyositis algorithm (see above)
	acrosclerosis

*Alopecia and Raynaud’s phenomenon were demonstrated by Tan and colleagues to have good sensitivity for SLE but lower specificity in distinguishing SLE from other CTDs. These were included because the goal of our study was to develop a highly sensitive instrument for detecting any CTD.
*Seizures and psychosis were not included because of their relatively low sensitivity (12 and 13%, respectively) in the study by Tan and colleagues. Results of tests for immunologic disorders, anti-Smith (Sm) antibody, anti-double stranded DNA (dsDNA) antibody, and false positive serologic test for syphilis were not ascertained by questionnaire because we thought that most patients would not know the results of these laboratory tests.

Validation

See²⁾

Sensitivity and Specificity
Sensitivity of the CSQ (the proportion meeting CTD criteria on the CSQamong those with confirmed disease) ranged from 83% to 96% for individual CTDs. Specificity of the CSQ (the proportion not meeting CTD criteria among those without confirmed disease) ranged from 83 to 93%. Specificity for individual CTDs was better among general medical control subjects than among non-CTD control subjects. Specificity was lowest for polymyositis/dermatomyositis and Sjogren’s syndrome. Likelihood ratios (the ability of the test to distinguish between diseased and nondiseased subjects) ranged from 5.0 for Sjogren’s syndrome to 11.9 for MCTD1.

Stratified Analysis of Specificity
Socioeconomic status was measured by educational level and health insurance status. We found significantly lower specificity among subjects with Medicaid or no health insurance and slightly lower specificity among less-educated subjects and those who were nonwhite. Specificity for detecting certain CTDs was significantly lower in patients with heart disease and depression.

Predictive Values
Positive predictive values in the study population, where the prevalence of CTD was much higher than in the general population, ranged from 23.5 to 84.9%. Positive predictive values in the general population, calculated assuming prevalence rates from population-based studies of CTD, ranged from 0.03% to 9.7%. As one would expect, the highest positive predictive value was for RA, the CTD with the highest prevalence in the general population. Negative predictive values were higher than 92% in the study population and higher than 99% in the general population.

Reliability
The K statistic calculated to evaluate test-retest reliability for detection of any potential CTD was 0.82, a rate indicating “excellent” reproducibility. Test-retest reliability values for specific CTDs were 0.50 for SLE, 0.80 for RA, 0.68 for scleroderma, 0.56 for polymyositis/dermatomyositis, 0.71 for Sjogren’s syndrome, and 0.68 for MCTD.

Variables

Questions in English	Questions in Dutch	Code	Variable	Assessment	Age
do you have joint pain?	hebt u pijn in uw gewrichten?	csq_jointpain_adu_q_01	csq1	1C	18+
do you have wrist or hand pain?	hebt u pijn in uw polsen of handen?	csq_wristhand_adu_q_02	csq2	1C	18+
are your wrists or hands swollen?	zijn uw polsen of handen gezwollen?	csq_swelling_adu_q_03	csq3	1C	18+
is it difficult for you to make a fist?	kost het u moeite om een vuist te maken?	csq_fist_adu_q_04	csq4	1C	18+
do you have joint stiffness in the morning?	zijn uw gewrichten ‘s ochtends stijf?	csq_stiffjoints_adu_q_05	csq5	1C	18+
if yes, how long does the stiffness last? / do you have joint stiffness in the morning?	zo ja, hoe lang duurt deze stijfheid? / zijn uw gewrichten s ochtends stijf?	csq_stiffjoints_adu_q_05_a	csq5a	1C	18+
do you have complaints in the same joints on both sides of your body?	hebt u aan beide kanten van uw lichaam klachten in dezelfde gewrichten ?	csq_jointpain_adu_q_06	csq6	1C	18+
do joint complaints limit you in your daily activities (work, leisure, personal care)?	beperken gewrichtsklachten u in uw dagelijkse bezigheden (werk, vrije tijd, persoonlijke verzorging)?	csq_jointpain_adu_q_07	csq7	1C	18+
do you have easily bleeding gums, spontaneously or after dental care?	hebt u last van snel bloedend tandvlees spontaan of na tandverzorging?	csq_bleedinggums_adu_q_08	csq8	1C	18+
do you have any loose teeth?	hebt u loszittende tanden?	csq_looseteeth_adu_q_09	csq9	1C	18+
can you see the roots of your teeth (necks of the teeth)?	kunt u uw tandwortels (tandhalzen) zien?	csq_recedinggums_adu_q_10	csq10	1C	18+
have you had lower back pain for longer than 3 months now?	hebt u al langer dan drie maanden last van uw onderrug?	csq_backpain_adu_q_11	csq11	1C	18+
did your back complaints begin before age 45?	zijn uw rugklachten begonnen voor uw 45e levensjaar?	csq_backpain_adu_q_12	csq12	1C
do you wake up at dawn because of pain in your lower back?	wordt u tegen de ochtend wakker van pijn in uw onderrug?	csq_backpain_adu_q_13	csq13	1C	18+
do you have morning stiffness in the back that lasts more than 30 minutes?	hebt u ochtendstijfheid in de rug die langer duurt dan 30 minuten?	csq_stiffback_adu_q_14	csq14	1C	18+
does exercising reduce the back complaints?	verminderen de rugklachten door bewegen?	csq_backpain_adu_q_15	csq15	1C	18+
how did your back pain begin, suddenly or gradually?	hoe is uw rugpijn ontstaan, plotseling of geleidelijk?	csq_backpain_adu_q_15_a	csq15a	1C	18+
have you had joint complaints for more than 3 months now?	hebt u al langer dan 3 maanden gewrichtsklachten?	csq_jointpain_adu_q_16	csq16	1C	18+
have you ever had joint stiffness in the morning lasting at least 1 hour for more than 6 weeks?	hebt u ooit langer dan 6 weken ’s ochtends minimaal 1 uur stijve gewrichten gehad?	csq_stiffjoints_adu_q_17	csq17	1C	18+
have you ever had nodules or bumps under the skin around your elbows or ankles?	hebt u ooit knobbels of bulten onder de huid rond uw ellebogen of enkels gehad?	csq_nodules_adu_q_18	csq18	1C	18+
left wrist / have you ever had swelling in any of the following joints, lasting more than 6 weeks?	pols (links) / zijn de volgende gewrichten ooit langer dan 6 weken gezwollen geweest?	csq_swelling_adu_q_19_a1	csq19a1	1C	18+
right wrist / have you ever had swelling in any of the following joints, lasting more than 6 weeks?	pols (rechts) / zijn de volgende gewrichten ooit langer dan 6 weken gezwollen geweest?	csq_swelling_adu_q_19_a2	csq19a2	1C	18+
left finger joints (but not the joints nearest the fingernails) / have you ever had swelling in any of the following joints, lasting more than 6 weeks?	vingergewrichten (het gewricht het dichtst bij de nagel niet meetellen) (links) / zijn de volgende gewrichten ooit langer dan 6 weken gezwollen geweest?	csq_swelling_adu_q_19_b1	csq19b1	1C	18+
right finger joints (but not the joints nearest the fingernails) / have you ever had swelling in any of the following joints, lasting more than 6 weeks?	vingergewrichten (het gewricht het dichtst bij de nagel niet meetellen) (rechts) / zijn de volgende gewrichten ooit langer dan 6 weken gezwollen geweest?	csq_swelling_adu_q_19_b2	csq19b2	1C	18+
left elbow / have you ever had swelling in any of the following joints, lasting more than 6 weeks?	elleboog (links) / zijn de volgende gewrichten ooit langer dan 6 weken gezwollen geweest?	csq_swelling_adu_q_19_c1	csq19c1	1C	18+
right elbow / have you ever had swelling in any of the following joints, lasting more than 6 weeks?	elleboog (rechts) / zijn de volgende gewrichten ooit langer dan 6 weken gezwollen geweest?	csq_swelling_adu_q_19_c2	csq19c2	1C	18+
left knee / have you ever had swelling in any of the following joints, lasting more than 6 weeks?	knie (links) / zijn de volgende gewrichten ooit langer dan 6 weken gezwollen geweest?	csq_swelling_adu_q_19_d1	csq19d1	1C	18+
right knee / have you ever had swelling in any of the following joints, lasting more than 6 weeks?	knie (rechts) / zijn de volgende gewrichten ooit langer dan 6 weken gezwollen geweest?	csq_swelling_adu_q_19_d2	csq19d2	1C	18+
have you ever had a blood test for rheumatoid arthritis?	is uw bloed ooit getest op reumatoïde artritis?	csq_arthritis_adu_q_20	csq20	1C	18+
if so, was the result: / have you ever had a blood test for rheumatoid arthritis?	zo ja, was de uitslag: / is uw bloed ooit getest op reumatide artritis?	csq_arthritis_adu_q_20_a	csq20a	1C	18+
have you ever had a blood test for lupus/sle? (e.g. ana or ena)	is uw bloed ooit getest op lupus/ sle ? (bijvoorbeeld ana of ena)	csq_lupus_adu_q_21	csq21	1C	18+
if you have ever had a blood test for lupus/sle, was the result:	zo ja, was de uitslag: / is uw bloed ooit getest op lupus/sle? (bijvoorbeeld ana of ena)	csq_lupus_adu_q_21_a	csq21a	1C	18+
are your fingers unusually sensitive to the cold?	zijn uw vingers overmatig gevoelig voor kou?	csq_fingers_adu_q_22	csq22	1C	18+
have your fingers ever shown any unusual color changes in the cold?	zijn uw vingers ooit abnormaal verkleurd geweest in de kou?	csq_fingers_adu_q_23	csq23	1C	18+
if yes, was the color white? / have your fingers ever shown any unusual color changes in the cold?	zo ja, was de kleur wit? / zijn uw vingers ooit abnormaal verkleurd geweest in de kou?	csq_fingers_adu_q_23_a	csq23a	1C	18+
if yes, was the color blue? / have your fingers ever shown any unusual color changes in the cold?	zo ja, was de kleur blauw? / zijn uw vingers ooit abnormaal verkleurd geweest in de kou?	csq_fingers_adu_q_23_b	csq23b	1C	18+
if yes, was the color purple? / have your fingers ever shown any unusual color changes in the cold?	zo ja, was de kleur paars? / zijn uw vingers ooit abnormaal verkleurd geweest in de kou?	csq_fingers_adu_q_23_c	csq23c	1C	18+
if yes, was the color red? / have your fingers ever shown any unusual color changes in the cold?	zo ja, was de kleur rood? / zijn uw vingers ooit abnormaal verkleurd geweest in de kou?	csq_fingers_adu_q_23_d	csq23d	1C	18+
have you ever had sores in your mouth or nose for more than 2 weeks at a time?	hebt u ooit langer dan 2 weken zweertjes in uw mond of neus gehad?	csq_sores_adu_q_24	csq24	1C	18+
have you ever had a red rash on your cheeks for more than a month?	hebt u ooit langer dan een maand rode uitslag op uw wangen gehad?	csq_rash_adu_q_25	csq25	1C	18+
have you ever had skin break out (rash) after being in the sun (not sunburn)?	hebt u ooit huiduitslag (zonlichtallergie) gehad nadat u in de zon geweest bent (geen verbranding)?	csq_sunrash_adu_q_26	csq26	1C	18+
have you ever had pleurisy or chest pain made worse with deep breaths for more than a few days (not caused by a bruised rib or a cold)?	hebt u ooit langer dan een paar dagen pijn gehad in uw longen, die erger werd bij diep inademen (niet veroorzaakt door een gekneusde rib of verkoudheid)	csq_pleurisy_adu_q_27	csq27	1C	18+
have you ever had rapid loss of lots of hair?	hebt u ooit veel haar verloren in korte tijd?	csq_hairloss_adu_q_28	csq28	1C	18+
have you ever had an epileptic seizure?	hebt u ooit een epileptische aanval gehad?	csq_seizures_adu_q_29	csq29	1C	18+
have you ever had puffy, swollen fingers for more than a month?	hebt u ooit langer dan een maand opgezette vingers gehad?	csq_swelling_adu_q_30	csq30	1C	18+
have you ever had skin thickening or tightening of face, neck, trunk, arms or legs ? (not caused by fluid retention)	is de huid van uw gezicht, nek romp, armen of benen ooit dikker of strakker dan normaal geweest? (niet veroorzaakt door het vasthouden van vocht)	csq_skinthickening_adu_q_31	csq31	1C	18+
have you ever had skin thickening or tightening of the fingers or toes?	is de huid van uw vingers of tenen ooit dikker of strakker dan normaal geweest ?	csq_skinthickening_adu_q_32	csq32	1C	18+
have you ever had sores leaving scars in the finger tips?	hebt u ooit zweertjes op uw vingertoppen gehad die littekens achterlieten?	csq_sores_adu_q_33	csq33	1C	18+
have you ever had muscle weakness (less muscle strength than usual) for more than 3 months?	hebt u ooit langer dan 3 maanden spierzwakte (minder spierkracht dan normaal) gehad?	csq_weakness_adu_q_34	csq34	1C	18+
have you ever had difficulty rising from a chair for more than 3 months?	hebt u ooit langer dan 3 maanden moeite gehad met het opstaan uit een stoel?	csq_weakness_adu_q_35	csq35	1C	18+
have you ever had difficulty combing your hair for more than 3 months?	hebt u ooit langer dan 3 maanden moeite gehad met het kammen van uw haar?	csq_weakness_adu_q_36	csq36	1C	18+
have you ever had a gritty or sandy sensation in your eyes?	hebt u ooit een korrelig of zanderig gevoel in uw ogen gehad?	csq_grittyeyes_adu_q_37	csq37	1C	18+
do you regularly wake up with a dry mouth, requiring you to drink water?	wordt u regelmatig wakker door een droge mond waarvoor u water moet gaan drinken?	csq_drymouth_adu_q_38	csq38	1C	18+
anemia? / have you ever been told by a doctor that you had	bloedarmoede? / heeft een dokter u ooit verteld dat u het volgende had:	csq_anemia_adu_q_39_a	csq39a	1C	18+
low white cell count? / have you ever been told by a doctor that you had	laag aantal witte bloedcellen? / heeft een dokter u ooit verteld dat u het volgende had:	csq_whitecellcount_adu_q_39_b	csq39b	1C	18+
low platelet count? / have you ever been told by a doctor that you had	laag aantal bloedplaatjes? / heeft een dokter u ooit verteld dat u het volgende had:	csq_plateletcount_adu_q_39_c	csq39c	1C	18+
protein in your urine? / have you ever been told by a doctor that you had	eiwit in uw urine? / heeft een dokter u ooit verteld dat u het volgende had:	csq_proteinurine_adu_q_39_d	csq39d	1C	18+
lupus/sle of the skin? have you ever been told by a doctor that you had	lupus/sle van de huid? / heeft een dokter u ooit verteld dat u het volgende had:	csq_lupus_adu_q_39_e	csq39e	1C	18+
scarring of the lungs (pulmonary fibrosis)? / have you ever been told by a doctor that you had	littekenweefsel in uw longen (longfibrose)? / heeft een dokter u ooit verteld dat u het volgende had:	csq_lungscars_adu_q_39_f	csq39f	1C	18+
high cpk (muscle enzyme) in your blood? / have you ever been told by a doctor that you had	hoog cpk (spier-eiwit) in uw bloed? / heeft een dokter u ooit verteld dat u het volgende had:	csq_cpk_adu_q_39_g	csq39g	1C	18+

¹⁾ , ²⁾

Karlson, E. W.et al. (1995). A connective tissue disease screening questionnaire for population studies. Annals of epidemiology 5(4), 297-302

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Connective Tissue Disease (CSQ)

Background

Diagnostic criteria

Validation

Variables

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Table of Contents

Connective Tissue Disease (CSQ)

Background

Diagnostic criteria

Validation

Variables

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