====== 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 [[https://en.wikipedia.org/wiki/Connective_tissue_disease|Connective Tissue Disease]] (CTD).\\ Note that musculoskeletal [[musculoskeletal symptoms|symptoms]] and [[musculoskeletal conditions|diseases]] were also assessed in general within [[start|Lifelines]] ([[sections|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 CTDs((Karlson, E. W.et al. (1995). A connective tissue disease screening questionnaire for population studies. Annals of epidemiology 5(4), 297-302)). ===== Diagnostic criteria ===== An algorithm for identifying potential CTD based on the [[https://www.rheumatology.org|ACR]] or published clinical criteria was used.\\ ^ CTD ^ Diagnostic criteria ^ | [[https://en.wikipedia.org/wiki/Rheumatoid_arthritis|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.// | | [[https://en.wikipedia.org/wiki/Systemic_lupus_erythematosus|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*// | | [[https://en.wikipedia.org/wiki/scleroderma|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 | | [[https://en.wikipedia.org/wiki/polymyositis|polymyositis]] or [[https://en.wikipedia.org/wiki/dermatomyositis|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. | | [[https://en.wikipedia.org/wiki/sjogrens_syndrome|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. | | [[https://en.wikipedia.org/wiki/Raynaud_syndrome|Raynaud’s phenomenon]] | cold sensitivity plus white, blue, or purple color changes of the fingers on exposure to cold | | [[https://en.wikipedia.org/wiki/Mixed_connective_tissue_disease|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 ===== See((Karlson, E. W.et al. (1995). A connective tissue disease screening questionnaire for population studies. Annals of epidemiology 5(4), 297-302))\\ **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+ |