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Rheumatology Secrets > Mixed Connective Tissue Disease (MCTD), Overlap Syndromes, and Undifferentiated Connective Tissue Disease (UCTD) > Flashcards

Mixed Connective Tissue Disease (MCTD), Overlap Syndromes, and Undifferentiated Connective Tissue Disease (UCTD) Flashcards

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1
Q

What is Mixed Connective Tissue Disease (MCTD)?

A

Overlap of SLE, SSc, inflammatory myopathy; high-titer ANA + U1-RNP antibodies.

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2
Q

What suggests a diagnosis other than MCTD?

A

Severe renal/neurologic disease or absence of Raynaud’s.

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3
Q

What are major causes of mortality in MCTD?

A

PAH and ILD.

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4
Q

What percentage of rheumatologic presentations are classified as Undifferentiated CTD (UCTD)?

A

25%.

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5
Q

What features characterize Mixed Connective Tissue Disease (MCTD)?

A

SLE + SSc + inflammatory myopathy; Jaccoud’s deformity possible.

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6
Q

What antibodies are associated with MCTD?

A

High-titer speckled ANA + U1-RNP; absence of anti-Sm, anti-dsDNA.

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7
Q

What is the female to male ratio in MCTD?

A

15:1.

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8
Q

What is the mean age of onset for MCTD?

A

37 years (range 4–80).

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9
Q

What defines Overlap Syndromes?

A

Symptoms of ≥2 classic rheumatic diseases (SLE, SSc, PM/DM, RA, SS).

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10
Q

Name an example of an Overlap Syndrome.

A

MCTD, PM-Scl antibody syndrome, ‘RUPUS’ (SLE + RA).

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11
Q

What are the features of Undifferentiated CTD (UCTD)?

A

Incomplete criteria for defined CTDs; ANA-positive.

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12
Q

What percentage of UCTD cases evolve into defined diagnoses?

A

25% develop SLE, SSc, or SS over 1–3 years.

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13
Q

What are common early symptoms of MCTD?

A

Raynaud’s (>90%), synovitis (>90%), puffy fingers (>70%), myositis (>50%).

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14
Q

What is a symptom that is absent in MCTD?

A

Severe renal/CNS disease.

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15
Q

What gastrointestinal issues are associated with MCTD?

A

Esophageal dysmotility (85%), reflux, rare intestinal vasculitis.

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16
Q

What pulmonary complications are common in MCTD?

A

PAH (mortality risk), ILD (66% on HRCT), restrictive pattern (69%).

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17
Q

What neurological symptoms may occur in MCTD?

A

Trigeminal neuralgia (15%); seizures/psychosis rare.

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18
Q

What lab findings are commonly seen in MCTD?

A

Anemia (65–75%), leukopenia (57–75%), hypergammaglobulinemia (80%), anti-U1-RNP (100%).

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19
Q

What is the prognosis for patients with MCTD?

A

58% remain MCTD; 17% → SSc, 9% → SLE, 2.5% → RA.

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20
Q

What complications dominate mortality in MCTD?

A

PAH complications.

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21
Q

What are common overlaps with Sjögren’s Syndrome (SS)?

A

RA, SLE, SSc, PBC, vasculitis.

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22
Q

What are common symptoms of Undifferentiated CTD (UCTD)?

A

Arthralgia (37–86%), Raynaud’s (33–56%), sicca (7–41%), serositis (5–16%).

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23
Q

What serological findings are typical in UCTD?

A

ANA+; lacks anti-Sm/dsDNA/centromere.

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24
Q

What symptoms predict progression to SLE in UCTD?

A

Fever, serositis, anti-Sm/dsDNA.

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25
What symptoms predict progression to SSc in UCTD?
Raynaud’s + nailfold capillaries + nucleolar ANA.
26
What symptoms predict progression to SS in UCTD?
Xerostomia + anti-SSA/SSB.
27
What are the classification criteria for UCTD?
Symptoms ≥3 years + ANA on 2 occasions.
28
What treatment approaches are used for SLE-like symptoms in MCTD?
NSAIDs, antimalarials, low-dose prednisone.
29
What treatment is used for myositis in MCTD?
High-dose prednisone + methotrexate/azathioprine.
30
What management strategies are used for SSc-like symptoms in MCTD?
Manage Raynaud’s, reflux, PAH (standard therapies ± immunosuppressants).
31
Who defined MCTD?
Sharp et al. (1972).
32
What study provided insights into long-term outcomes of MCTD?
Burdt et al. (1999).
33
What paper discussed MCTD as a distinct entity?
Cappelli et al. (2012).
34
Who reviewed Undifferentiated CTD?
Mosca et al. (2011).
35
Welche Laborbefunde sind typisch für MCTD?
Anemia is usually that of chronic disease. Coombs' positivity is detected in up to 60% of patients, although overt hemolytic anemia is uncommon. Thrombocytopenia is uncommon. The sedimentation rate is usually elevated due to hypergammaglobulinemia. ## Footnote Typische Laborbefunde für MCTD umfassen Anämie, Coombs' Positivität und erhöhte Sedimentationsrate.
36
Was ist U1-RNP und welche Rolle spielt es bei MCTD?
U1-RNP is a uridine-rich small nuclear ribonucleoprotein (snRNP) that consists of U1-RNA and U1-specific polypeptides 70 kD, A, and C. Patients with MCTD form high titers of antibodies against U1-RNP, particularly U1-70kD and U1-RNA. ## Footnote U1-RNP ist entscheidend für die Diagnose von MCTD, da Patienten hohe Antikörpertiter gegen U1-RNP bilden.
37
Wie wird die SLE-ähnliche Komponente von MCTD behandelt?
The SLE-like features of arthritis and pleurisy are treated with NSAIDs, antimalarials, low-dose prednisone (<20 mg/day), and occasionally methotrexate. ## Footnote Behandlungsansätze für die SLE-ähnlichen Merkmale umfassen entzündungshemmende Medikamente und Kortikosteroide.
38
Wie wird die Myositis-Komponente behandelt?
Inflammatory myositis is treated with high doses of prednisone (60 mg/day) and sometimes methotrexate or azathioprine. ## Footnote Hohe Dosen von Prednison sind entscheidend für die Behandlung der entzündlichen Myositis.
39
Was charakterisiert das PM-Scl Overlap-Syndrom?
One specific overlap is characterized by antibody to PM-Scl, a complex of 16 polypeptides located at the site of ribosomal assembly in the nucleolus. ## Footnote Patienten mit PM-Scl-Antikörper zeigen häufig ein nucleoläres Muster in der ANA-Testung.
40
Was sind die Faktoren, die die Entwicklung eines SLE bei UCTD vorhersagen?
Fever, serositis, and/or anti-Sm or anti-dsDNA antibodies—SLE. ## Footnote Diese Faktoren sind entscheidend für die Prognose der Krankheitsentwicklung bei UCTD.
41
Was sind die Faktoren, die die Entwicklung einer SSc vorhersagen?
Raynaud's phenomenon, abnormal nailfold capillaries, and nucleolar ANA—SSc. ## Footnote Diese Merkmale helfen bei der Unterscheidung von SSc.
42
Wie ist die Geschlechterverteilung bei MCTD?
MCTD is 15 times more common in women than in men. ## Footnote Diese Geschlechterverteilung zeigt eine signifikante Prävalenz bei Frauen.
43
In welchem Alter wird MCTD typischerweise diagnostiziert?
The mean age at diagnosis is 37 years, with a range of 4 to 80 years. ## Footnote Das Alter bei der Diagnose von MCTD variiert erheblich, wobei die Mehrheit der Patienten im mittleren Alter diagnostiziert wird.
44
MCTD lab findings
LAB-MCTD: Low Anemia (chronic), Boosted gammaglobulins, Coombs' positive (60%) ## Footnote Mnemonic zur Erinnerung an die typischen Laborbefunde bei MCTD.
45
U1-RNP components
U1-70-CAR: U1-70kD, C, A, RNA ## Footnote Mnemonic zur Erinnerung an die Bestandteile von U1-RNP.
46
MCTD treatment approach
SLE-MYO: SLE-like (NSAIDs, antimalarials), MYOsitis (high-dose steroids) ## Footnote Mnemonic zur Erinnerung an die Behandlungsansätze für MCTD.
47
PM-Scl antibody pattern
NUC-16: Nucleolar ANA from 16-polypeptide complex ## Footnote Mnemonic für das charakteristische Muster bei PM-Scl Antikörpern.
48
UCTD to SLE predictors
FSAD: Fever, Serositis, Anti-Sm/dsDNA ## Footnote Mnemonic zur Erinnerung an die prädiktiven Faktoren für die Entwicklung von SLE aus UCTD.
49
MCTD epidemiology
15-to-1: Women 15x > men, Age 37 mean ## Footnote Mnemonic zur Erinnerung an die epidemiologischen Daten von MCTD.
50
Welche kardialen Komplikationen treten bei MCTD auf?
Myocarditis (Rare), Hypertensive crisis (Rare) ## Footnote Aus Tabelle 21.1
51
Welche Hautveränderungen sind typisch für MCTD?
Sclerodermatous changes (33-67%), Skin rash (38-50%), Mouth sores (45%) ## Footnote Aus Tabelle 21.1
52
Wie häufig ist Lymphadenopathie bei MCTD?
Lymphadenopathy (39-50%) ## Footnote Aus Tabelle 21.1
53
Wie häufig ist Rheumafaktor bei MCTD?
Rheumatoid factor (50%) ## Footnote Aus Tabelle 21.3
54
Was sind die kardialen Komplikationen von MCTD?
MH: Myocarditis & Hypertensive crisis (both Rare)
55
Was sind die Hautveränderungen bei MCTD?
S-S-M: Scleroderma (33-67%), Skin rash (38-50%), Mouth sores (45%)
56
Wie häufig tritt Lymphadenopathie bei MCTD auf?
39-50%: Lymphadenopathy in 39-50% of cases
57
Wie häufig ist der Rheumafaktor bei MCTD?
RF-50: Rheumatoid factor positive in 50%
58
Welche Erkrankungen müssen von MCTD abgegrenzt werden?
Severe renal disease, neurologic disease, or the absence of Raynaud's phenomenon should suggest a disease other than MCTD.
59
Wie wird PAH bei MCTD behandelt?
Standard approaches to the diagnosis and management of PAH are outlined in Chapter 18, Systemic Sclerosis. Some evidence suggests that PAH secondary to MCTD or SLE may benefit from immunosuppressive medication such as cyclophosphamide.
60
Was sind die Kriterien, die UCTD definieren?
Signs and symptoms of a connective tissue disease for ≥3 years but not fulfilling the criteria for any of the defined CTDs. Presence of ANAs on two different occasions.
61
Wie häufig entwickelt sich UCTD zu einer definierten Erkrankung?
Over time (1-3 years), about 25% of these patients will evolve into a defined rheumatologic diagnosis.
62
Welche Antikörper sind mit Myositis-Overlap-Syndromen assoziiert?
Myositis overlap syndromes: antisynthetase antibody syndromes
63
Welche Antikörper sind mit RA-Overlaps assoziiert?
Patients with anti-RA-33 (hnRNP-A2) are most likely to develop an erosive arthritis.
64
Wie ist die Geschlechterverteilung bei UCTD?
The majority of patients with UCTD are female (80%-95%) with disease onset during the fourth decade (30-40 years).
65
Welches ANA-Muster zeigt PM-Scl?
Patients with PM-Scl antibody commonly have a nucleolar pattern ANA on immunofluorescent antibody assay.
66
Wie lautet die Mnemonic für MCTD kardiale Komplikationen?
MH: Myocarditis & Hypertensive crisis (both Rare)
67
Wie lautet die Mnemonic für MCTD Hautveränderungen?
S-S-M: Scleroderma (33-67%), Skin rash (38-50%), Mouth sores (45%)
68
Wie lautet die Mnemonic für MCTD Lymphadenopathie?
39-50%: Lymphadenopathy in 39-50% of cases
69
Wie lautet die Mnemonic für MCTD Rheumafaktor?
RF-50: Rheumatoid factor positive in 50%
70
Wie lautet die Mnemonic für MCTD Differentialdiagnose?
No Raynaud's? Rule Out MCTD!
71
Wie lautet die Mnemonic für MCTD PAH Behandlung?
PAH Rx: Immunosuppressants (Cyclophosphamide) + Standard care
72
Wie lautet die Mnemonic für UCTD Klassifikation?
3-2 Rule: 3 years symptoms + 2 positive ANA tests
73
Wie lautet die Mnemonic für UCTD Progression?
25% in 1-3 years: 25% develop defined CTD
74
Wie lautet die Mnemonic für Myositis Overlap Antikörper?
Anti-SYN: Anti-synthetase syndrome
75
Wie lautet die Mnemonic für RA Overlap Marker?
RA-33: Anti-RA-33 predicts erosive arthritis
76
Wie lautet die Mnemonic für UCTD Geschlechterverteilung?
F-90: Female predominance (80-95%)
77
Wie lautet die Mnemonic für PM-Scl ANA Muster?
NUC-PM: Nucleolar pattern in PM-Scl
78
What 3 diseases overlap in MCTD?
SLE + Systemic Sclerosis + Inflammatory Myopathy ## Footnote Mnemonic: "SSS" - SLE, SSc, MyoSitis
79
What antibodies are pathognomonic for MCTD?
High-titer speckled ANA + anti-U1-RNP ## Footnote Mnemonic: "U1-RNP or bust!"
80
What percentage of MCTD patients have Raynaud's?
>90% ## Footnote Mnemonic: "90° turns make fingers white"
81
What GI symptoms occur in MCTD?
Esophageal dysmotility (85%), reflux (60-70%), dysphagia (40%) ## Footnote Mnemonic: "GERD-85" - GastroEsophageal Reflux Disease in 85%
82
Most feared pulmonary complication?
Pulmonary arterial hypertension (PAH) ## Footnote Mnemonic: "PAH = Poor Airway Health"
83
What percentage develop ILD?
66% on HRCT ## Footnote Mnemonic: "2/3 lungs affected"
84
Universal lab findings?
100% high-titer ANA + anti-U1-RNP ## Footnote Mnemonic: "100% U1-RNP guarantee"
85
How common is hypergammaglobulinemia?
80% ## Footnote Mnemonic: "8-ball gammopathy"
86
What suggests NOT MCTD?
Absence of Raynaud's or severe renal/CNS disease ## Footnote Mnemonic: "No Raynaud's? No MCTD!"
87
How to treat MCTD myositis?
High-dose prednisone (60mg/day) ± methotrexate ## Footnote Mnemonic: "60 mg for 60 muscles"
88
SLE-like features treatment?
NSAIDs, antimalarials, low-dose prednisone (<20mg) ## Footnote Mnemonic: "SLEep with low-dose steroids"
89
Most common overlap disease?
Sjögren's syndrome ## Footnote Mnemonic: "SS is #1 in overlaps"
90
What defines "RUPUS"?
SLE + RA (RF+ + erosions) ## Footnote Mnemonic: "RUPUS = Rheumatoid + lUPUS"
91
UCTD progression rate?
25% develop defined CTD in 1-3 years ## Footnote Mnemonic: "25% in 3 years"
92
Predictors of progression to SSc?
Raynaud's + nailfold capillaries + nucleolar ANA ## Footnote Mnemonic: "RAN - Raynaud's, Abnormal Nailfold"
93
What is the "90-70-50 Rule"?
90% Raynaud's/synovitis, 70% puffy fingers, 50% myositis ## Footnote Mnemonic: "90-70-50 Rule"
94
What is the "PFT-66"?
66% have abnormal PFTs, 66% decreased DLCO ## Footnote Mnemonic: "PFT-66"
95
What is the "LAB-100"?
100% ANA/U1-RNP, 100% specificity ## Footnote Mnemonic: "LAB-100"
96
What is the "UCTD-40"?
40% have arthralgias, 40% have Raynaud's ## Footnote Mnemonic: "UCTD-40"
97
Neurologic manifestation?
Trigeminal neuralgia (15%) ## Footnote Mnemonic: "TN-15" - Trigeminal Neuralgia in 15%
98
MCTD prognosis over 10 years?
58% remain MCTD, 17%→SSc, 9%→SLE ## Footnote Mnemonic: "60-20-10" (rounded percentages)

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