First question post - sigmadx

[ArchivalUser]

Hi everyone, I've been a longtime reader on this forum and now that I'm finally in med school (boring story), I'll try to contribute more so here's my first question post:

A 37-year-old woman with Raynaud™s phenomenon
complains of progressive weakness with inability to
arise out of a sitting position without assistance. On examination,
the patient has swollen œsausage-like fingers,
alopecia, erythematous patches on the knuckles, facial telangiectasias, and proximal muscle weakness. Laboratory
evaluation includes a normal CBC and serum chemistries,
except for creatine phosphokinase, 4.5
kat/L (270 U/L),
and aldolase, 500 nkat/L (30 U/L). The following serologic
profile is found: rheumatoid factor is positive at 1:
1600; ANA is also positive at 1:1600 with a speckled
pattern and very high titers of antibodies against the ribonuclease-sensitive ribonucleoprotein component of extractable nuclear antigen. This patient probably has

(A) early rheumatoid arthritis
(B) systemic sclerosis
© systemic lupus erythematosus
(D) dermatomyositis
(E) mixed connective-tissue disease (MCTD)

[ArchivalUser]

bb..welcome to the forum

[ArchivalUser]

second thoughts...is it ee?

[ArchivalUser]

ee?

[ArchivalUser]

gj guys

The answer is E. MCTD is a syndrome characterized by high titers of
circulating antibodies to the ribonucleoprotein component of extractable nuclear antigen
in association with clinical features similar to those of SLE, systemic sclerosis, polymyositis,
and rheumatoid arthritis. The average patient with MCTD is a middle-aged woman
with Raynaud™s phenomenon who also has polyarthritis, sclerodactyly (including swollen
hands), esophageal dysfunction, pulmonary fibrosis, and inflammatory myopathy. Cutaneous
manifestations include telangiectasias on the face and hands, alopecia, a lupuslike
heliotropic rash, and erythematous patches over the knuckles. Myopathy may involve
severe weakness of proximal muscles associated with high levels of creatine phosphokinase
and aldolase. Both pulmonary involvement and esophageal dysmotility are common
but are frequently asymptomatic until quite advanced. Almost all patients have high titers
of rheumatoid factor and antinuclear antibodies. Such antibodies are directed toward the
ribonuclease-sensitive ribonucleoprotein component of extractable nuclear antigen.

[ArchivalUser]

ee

[ArchivalUser]

thank u for the question, sigmadx.
welcome to forum.