All About Antibodies
And Now Presenting ...
So Long Sjogren's
Much Ado About MCTD
CTD Potpourri
100

This antibody at high titer is a serologic hallmark of MCTD but may be present in low titers in other diseases.

What is RNP?

100

Renal disease in MCTD is ______ (more/less/as) common than in lupus.

What is "less"?

100

This neurologic diagnosis is relatively unique to Sjogren's Syndrome

What is a purely sensory neuropathy?

100

Common rashes at onset of MCTD are: _______

What is rashes of cSLE or JDM?

100

This type of white blood cell is increased in tissues affected by IgG4-Related Disease.

What is the eosinophil?

(also plasma cells)

200

This antibody (or antibodies) is/are associated with neonatal lupus.

What are SSA (anti-Ro) and SSB (anti-La)?

200

The most common clinical manifestation of MCTD at presentation is ...

What is arthritis?

200

These proteins are associated with poor prognosis in Sjogren's Syndrome.

What are cryoglobulins?

200

When children with MCTD have arthritis, it is associated with which protein.

What is rheumatoid factor (RF) seropositivity?

200

This is the most common clinical manifestation of IgG4-related disease in children.

What is orbital disease? (44%)

Other common clinical manifestations include:

- Pancreatitis/autoimmune pancreatitis type 1 (12%)

- Cholangitis (8%)

- Pulmonary disease (8%)

300

This antibody is associated with disease persistence in MCTD

What is RF (rheumatoid factor)?

300

This rheumatic disease is associated with a 44 fold increased risk of developing lymphoma.

What is Sjogren's Syndrome?

300

This is the type of lymphoma that is most common for patients with Sjogren's.

What is non-Hodgkin lymphoma?

Or what is extranodal B-cell lymphoma of the MALT cell type?

300

This is the major source of morbidity and mortality among adults with MCTD.

What is pulmonary disease?

Pulmonary hypertension is the most frequent serious complication

300

This is the term for patients with some features of CTD who lack adequate clinical or diagnostic features to fit a recognizable clinical syndrome.

What is undifferentiated connective tissue disease?

Not to be confused with overlap syndrome: patients with two or more distinctly recognizable rheumatic diseases.

400

According to the Sharp criteria, this antibody CANNOT be present for a diagnosis of MCTD.

What is anti-Smith (Sm)?

400

_____ should be on your differential for a patient with retro-orbital pseudotumor.

What is IgG4 related diseases?

400

Name one (or more!- up to 4) clinical features thought to be predictive of lymphoma development in Sjogren's patients.

What is: persistent enlargement of parotid glands, lymphadenopathy, splenomegaly and palpable purpura?

400

This HLA class II specificity is associated with MCTD.

What is HLA-DR4?

400

Name 1 (or more! - up to 3) lab features that are associated with a predisposition to lymphoma in patients with Sjogren Syndrome.

What is mixed monoclonal cryoglobulinemia, low level of C4, monoclonal bands in serum or urine?

500

IgG4-Related Disease is characterized by serum levels of IgG greater than ____

What is 140 mg/dL?

500

Neuromyelitis optica spectrum disorder is associated with this rheumatologic diagnosis

What is Sjogren's Syndrome?

500

Patients with Sjogren Syndrome treated with belimumab (Benlysta) reported improvement in this symptom.

What is dryness?

No improvement in pain or fatigue

500

This hematologic abnormality when it occurs can be resistant to conventional therapy.

What is thrombocytopenia?

500

This cytokine is thought to contribute to the pathogenesis of Sjogren Syndrome.

What is IFNalpha?

IFN signature detected in PBMC of SS patients and correlated with anti-SSA and anti-SSB levels. There is increased expression of IFN-regulated genes in the salivary glands of SS patients.

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