Rheumatologic manifestations of other diseases
1. Endocrinology
Diabetes
Diabetic Cheiroarthropathy
It is characterized by a progressive resistance to passive and active range of motion, often
beginning in the hands. Contractures and fibrosis with waxy skin on the hands. It is associated
with the duration and severity of diabetes, but can be seen in prediabetes and can be out of
proportion to the HbA1C. They may also have dysesthesia due to concomitant diabetic
polyneuropathy. Contractures mostly occur in the PIP and MCP joints and tend to progress over
time from the fourth and fifth digits radially toward the second and third digits. On clinical
examination, patients will be unable to flatten their palms together (positive prayer sign).
Treatment: physiotherapy and diabetes control.
Dx from Dupuytren’s contracture: no thickening or nodularity of the palmar fascia.
Dx from palmar fasciitis with polyarthritis syndrome: also nodular like Dupuytren, but
this has arthritis and is paraneoplastic (ovarian cancer).
Scleredema
Type III, scleredema diabeticorum: hyperglycemia leads to enzymatic glycosylation of collagen
that becomes resistant to breakdown, leading to an accumulation of abnormal collagen in the
skin. There’s no fibroblast proliferation. It begins with non-pitting edema that progresses to skin
thickening, typically begins on the neck and later spreads to the shoulders and upper part of the
trunk – ‘cape-like’. Unlike SSc, there is no involvement of the hands and fingers (“inverse” in
comparison with SSc). Skin is thickened but not shiny or waxy. It usually does not usually involve
sclerosis or hardening of the internal organs, but the heart, lungs and tongue can still be
affected. Pigmentary changes, which we would expect to see in SSc, would also be absent.
Diabetic Myonecrosis
also known as diabetic muscle infarction. Rare (<200 cases). Patients present with acute-onset,
painful swelling of an affected muscle. The thigh is most commonly involved and a palpable
mass may be present. Myonecrosis is distinct from diabetic amyotrophy (ex. Bruns-Garland
syndrome) in which there is muscle wasting, weakness and pain due to diabetic neuropathy,
such as at the lumbosacral plexus. Myonecrosis may be confused with IIM, given the presence of
fever in 10%, ↑ESR in >50%, ↑ CPK and indistinguishable changes on MRI (“patchwork pattern”
of multifocal areas that demonstrate interfascial and intramuscular edema and muscle
enlargement).
Dx from IIM: diabetic myonecrosis tends to be acute in onset, with only mild
elevations in CPK and it is often unilateral, sparing the upper extremities. Autoantibodies should
be negative and there will be no additional signs or symptoms of CTD.
Treatment: supportive, painkillers, physiotherapy.
Diabetic amyotrophy (Bruns-Garland syndrome)
Charcot’s arthropathy: pain and swelling, midfoot, ‘rock-bottom’ deformity. Treatment: rest,
no weight bearing of the joint.
, Stiff person syndrome: progressive axial stiffness may mimic spine ankylosis. Straightening of
the lumbar lordosis. “Startle” response (marked hardening of the muscle group in response to
fright or surprise) present in most patients. Association anti-GAD antibody.
Medication: thiazolidinediones – secondary osteoporosis (due to PPAR-γ and promotion of
mesenchymal → adipocyte differentiation).
Associated with diabetes: DISH, adhesive capsulitis ('frozen shoulder’), Dupuytren’s
contracture, stenosing flexor tenosynovitis (trigger finger), carpal tunnel syndrome
Thyroid
Hypothyroidism
PMR-like
Myopathy, if severe it’s called ‘Hoffman syndrome’.
Carpal tunnel syndrome
Myxedematous arthropathy
Hyperthyroidism
Pretibial myxedema
Thyrotoxic periodic paralysis (myopathy)
a rare but serious complication associated with hyperthyroidism. Sudden and temporary muscle
weakness or paralysis, typically involving the limbs. The episodes can occur without warning and
may last anywhere from hours to days. TPP is most commonly seen in young men of Asian
descent. The exact mechanism of TPP is not completely understood, but it is believed to involve
an abnormal shift of K+ from the blood into muscle cells during periods of hyperthyroidism. This
shift is likely due to the increased sensitivity of muscle cells to thyroid hormones, which leads to
abnormal ion channel function and K+ depletion in the bloodstream. Treatment: (IV) K+
supplementation but be cautious because it’s not a real hypokalemia, rather the K+ has entered
into the cells. Also treat hyperthyroidism (methimazole, 5-PTU, β-blockers).
Thyroid acropachy (clubbing): unlike pulmonary osteoarthropathy, the bony reaction has a
speculated lacy appearance and is limited to the midportion of the diaphysis with sparing of the
long bones.
Osteoporosis (high bone turnover)
Medication: 5-FU → ANCA vasculitis (p-ANCA [+]), methimazole → DILE.
Hyperparathyroidism
“bones, stones, abdominal groans and psychiatric overtones”
Osteitis fibrosa cystica: high turnover condition, rugger-jersey spine
Osteoporosis: mainly of cortical bone (distal radius, femoral diaphysis – not as much in
trabecular bone).
DEXA in distal radius
CPPD
1. Endocrinology
Diabetes
Diabetic Cheiroarthropathy
It is characterized by a progressive resistance to passive and active range of motion, often
beginning in the hands. Contractures and fibrosis with waxy skin on the hands. It is associated
with the duration and severity of diabetes, but can be seen in prediabetes and can be out of
proportion to the HbA1C. They may also have dysesthesia due to concomitant diabetic
polyneuropathy. Contractures mostly occur in the PIP and MCP joints and tend to progress over
time from the fourth and fifth digits radially toward the second and third digits. On clinical
examination, patients will be unable to flatten their palms together (positive prayer sign).
Treatment: physiotherapy and diabetes control.
Dx from Dupuytren’s contracture: no thickening or nodularity of the palmar fascia.
Dx from palmar fasciitis with polyarthritis syndrome: also nodular like Dupuytren, but
this has arthritis and is paraneoplastic (ovarian cancer).
Scleredema
Type III, scleredema diabeticorum: hyperglycemia leads to enzymatic glycosylation of collagen
that becomes resistant to breakdown, leading to an accumulation of abnormal collagen in the
skin. There’s no fibroblast proliferation. It begins with non-pitting edema that progresses to skin
thickening, typically begins on the neck and later spreads to the shoulders and upper part of the
trunk – ‘cape-like’. Unlike SSc, there is no involvement of the hands and fingers (“inverse” in
comparison with SSc). Skin is thickened but not shiny or waxy. It usually does not usually involve
sclerosis or hardening of the internal organs, but the heart, lungs and tongue can still be
affected. Pigmentary changes, which we would expect to see in SSc, would also be absent.
Diabetic Myonecrosis
also known as diabetic muscle infarction. Rare (<200 cases). Patients present with acute-onset,
painful swelling of an affected muscle. The thigh is most commonly involved and a palpable
mass may be present. Myonecrosis is distinct from diabetic amyotrophy (ex. Bruns-Garland
syndrome) in which there is muscle wasting, weakness and pain due to diabetic neuropathy,
such as at the lumbosacral plexus. Myonecrosis may be confused with IIM, given the presence of
fever in 10%, ↑ESR in >50%, ↑ CPK and indistinguishable changes on MRI (“patchwork pattern”
of multifocal areas that demonstrate interfascial and intramuscular edema and muscle
enlargement).
Dx from IIM: diabetic myonecrosis tends to be acute in onset, with only mild
elevations in CPK and it is often unilateral, sparing the upper extremities. Autoantibodies should
be negative and there will be no additional signs or symptoms of CTD.
Treatment: supportive, painkillers, physiotherapy.
Diabetic amyotrophy (Bruns-Garland syndrome)
Charcot’s arthropathy: pain and swelling, midfoot, ‘rock-bottom’ deformity. Treatment: rest,
no weight bearing of the joint.
, Stiff person syndrome: progressive axial stiffness may mimic spine ankylosis. Straightening of
the lumbar lordosis. “Startle” response (marked hardening of the muscle group in response to
fright or surprise) present in most patients. Association anti-GAD antibody.
Medication: thiazolidinediones – secondary osteoporosis (due to PPAR-γ and promotion of
mesenchymal → adipocyte differentiation).
Associated with diabetes: DISH, adhesive capsulitis ('frozen shoulder’), Dupuytren’s
contracture, stenosing flexor tenosynovitis (trigger finger), carpal tunnel syndrome
Thyroid
Hypothyroidism
PMR-like
Myopathy, if severe it’s called ‘Hoffman syndrome’.
Carpal tunnel syndrome
Myxedematous arthropathy
Hyperthyroidism
Pretibial myxedema
Thyrotoxic periodic paralysis (myopathy)
a rare but serious complication associated with hyperthyroidism. Sudden and temporary muscle
weakness or paralysis, typically involving the limbs. The episodes can occur without warning and
may last anywhere from hours to days. TPP is most commonly seen in young men of Asian
descent. The exact mechanism of TPP is not completely understood, but it is believed to involve
an abnormal shift of K+ from the blood into muscle cells during periods of hyperthyroidism. This
shift is likely due to the increased sensitivity of muscle cells to thyroid hormones, which leads to
abnormal ion channel function and K+ depletion in the bloodstream. Treatment: (IV) K+
supplementation but be cautious because it’s not a real hypokalemia, rather the K+ has entered
into the cells. Also treat hyperthyroidism (methimazole, 5-PTU, β-blockers).
Thyroid acropachy (clubbing): unlike pulmonary osteoarthropathy, the bony reaction has a
speculated lacy appearance and is limited to the midportion of the diaphysis with sparing of the
long bones.
Osteoporosis (high bone turnover)
Medication: 5-FU → ANCA vasculitis (p-ANCA [+]), methimazole → DILE.
Hyperparathyroidism
“bones, stones, abdominal groans and psychiatric overtones”
Osteitis fibrosa cystica: high turnover condition, rugger-jersey spine
Osteoporosis: mainly of cortical bone (distal radius, femoral diaphysis – not as much in
trabecular bone).
DEXA in distal radius
CPPD
