A rare syndrome / workup of arthritis

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Learning points

Sandra P. Susanibar Adaniya Chief Resident IM - UAMS

ACD06/12

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What is the diagnosis?

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Sweet syndrome• Sweet syndrome = acute febrile neutrophilic dermatosis• Inflammatory disorder characterized by the presence of inflammatory papules, plaques, or nodules on the skin, systemic symptoms, and neutrophilic infiltration of the skin.• Cutaneous lesions of Sweet syndrome are typically painful,

erythematous to violaceous papules and plaques• Fever, malaise, joint pain, and muscle pain often accompany the

cutaneous lesions.

• Three categories based on etiology: • Classical Sweet syndrome• Malignancy-associated Sweet syndrome (most common acute

myelogenous leukemia)• Drug-induced Sweet syndrome (MCC granulocyte-colony

stimulating factor (G-CSF).

Clinical features of Sweet syndrome

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Sweet syndrome – diagnosis

1. Abrupt onset of painful erythematous plaques or nodules

2. Histopathologic evidence of a dense neutrophilic infiltrate without evidence of leukocytoclastic vasculitis.

3. Pyrexia >38°C

4. Excellent response to treatment with systemic corticosteroids or potassium iodide.

5. Abnormal laboratory values at presentation (three of four): erythrocyte sedimentation rate >20 mm/hr; positive C-reactive protein; >8,000 leukocytes; >70 percent neutrophils

A. Abrupt onset of painful erythematous plaques or nodules.

B. Histopathologic evidence of a dense neutrophilic infiltrate without evidence of leukocytoclastic vasculitis

C. Pyrexia >38°C

D. Temporal relationship between drug ingestion and clinical presentation, or temporally-related recurrence after oral challenge.

E. Temporally-related resolution of lesions after drug withdrawal or treatment with systemic corticosteroids

Classical = The presence of both major criteria (1 and 2), and two of the four minor criteria.

Drug induced = All five criteria (A, B, C, D, and E) are required for the diagnosis.

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LEARNING POINTS

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Does this patient have arthralgia or arthritis?

• Key = presence of inflammation• Cardinal signs of inflammation: erythema, warmth, pain, and swelling. • Severe joint inflammation or systemic disease: fatigue, weight loss, or fever. • Morning stiffness lasting > 1 hour = underlying inflammation

Richie AM, Francis ML. Diagnostic approach to polyarticular joint pain. Am Fam Physician 2003; 68: 1151–60Samanta J, Kendall J. Polyarthralgia. British medical journal. 2003; 326:859.

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Approach to arthritis

Duration?Cut-off=6

weeks

Acute

Infection

Trauma

Crystals

Reactive

Early chronic cause

Chronic

Pocket Medicine. Fifth Edition. Marc S. Sabatine, MD, MPH

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Approach to arthritis

Duration?

Chronic # joints involved

Mono (1) Indolent infection

Oligo (2-4)

Indolent infection

Spondylo-

arthropathy

Poly (≥5)

RA

SLE

Myositis

Systemic sclerosis

CPPD

Acute

Adapted from Pocket Medicine. Fifth Edition. Marc S. Sabatine, MD, MPH

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Evaluation of polyarthritis or polyarthralgia

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