VA Morning Report- The Pulseless Disease

Yesterday, R3 Dr. Brandon Jakubowski presented a case he initially saw in PRIME clinic – a young lady who presented with a papular rash on her chin. The rash was initially treated conservatively, however progressed and involved painful pustular lesions on the patient’s arms. The patient was seen by dermatology and underwent a biopsy, however ended up going to the emergency room prior to the pathology resulting. In the ER, the patient was hypotensive and was noted to have absent upper extremity pulses. A CT scan was performed and aided in diagnosing Takayasu’s arteritis.

 

For research purposes, the American College of Rheumatology developed diagnostic criteria for Takayasu Arteritis in 1990. Patients must meet at least three of the following six criteria:

  • Age of onset ≤ 40 years old
  • Claudication of extremities
  • Decreased brachial artery pulse
  • Blood Pressure difference > 10mmHg between arms
  • Bruit over subclavian arteries or aorta
  • Arteriogram abnormality (narrowing or occlusion of the entire aorta, its primary branches or large arteries in the proximal upper or lower extremities, not due to arteriosclerosis, fibromuscular dysplasia or similar causes; changes usually focal or segmental)

The modified Ishikawa diagnostic criteria is slightly more complicated but has a higher sensitivity and specificity (92.5% and 95%, respectively) than the original ACR criteria and is notable for removing the age criteria.

In the management of Takayasu Arteritis, immunosuppressants are the cornerstone of therapy, however optimal management is undefined. This particular patient received high dose steroids, then transitioned to tocilizumab when side effects were no longer manageable. It is important to understand that laboratory findings do not reliably track disease activity and therapy is essentially guided by patient symptoms.

 

Further reading:

Arend WP, Michel BA, Bloch DA , et al. The American College of Rheumatology 1990 criteria for the classification of Takayasu arteritis. Arthritis Rheum 1990;33:112934.doi:10.1002/art.1780330811

Kim ESH, Beckman J. Takayasu arteritis: challenges in diagnosis and management. Heart. 2018 Apr;104(7):558-565. doi: 10.1136/heartjnl-2016-310848. Epub 2017 Nov 25.

 

 

Cox’s Conference – Sweet’s Syndrome

In last week’s Ambulatory Cox’s Conference, Dr. Stephen Philip presented a case of a female with history of rheumatoid arthritis who presented with a chief complaint of fever and rash. Initial laboratory studies unveiled an active urinary sediment concerning for glomerulonephritis. Our expert discussant, Dr. Biff Palmer guided us through the differential diagnosis for fever and rash with renal involvement.

In a patient who presents with fevers and rash, the differential diagnosis is broad and includes infectious, autoimmune, neoplastic, and drug-related etiologies. Our patient had evidence of renal involvement with an active urinary sediment concerning for glomerulonephritis. Our differential diagnosis in this patient included systemic lupus erythematosus, vasculitis (IgA vasculitis, ANCA-associated vasculitis, or cryoglobulinemia), common viral illnesses (EBV, CMV, and HIV), infective endocarditis, disseminated fungal infection, and cutaneous malignancy. The patient underwent a skin biopsy which confirmed the diagnosis of Sweet’s Syndrome.

Sweet’s Syndrome Pearls:

·     Also known as acute febrile neutrophilic dermatosis

·     Characterized by abrupt onset of painful, erythematous papules, plaques, or nodules which is frequently accompanied by fever and leukocytosis.

·     Neutrophilic infiltration to other organ systems can very rarely occur, including ocular, musculoskeletal, central nervous system, cardiovascular system, pulmonary system, gastrointestinal tract/liver, and kidneys. Renal involvement, as in our patient, is rare but commonly presents with proteinuria, less commonly with hematuria or renal insufficiency.1-2

·     Most commonly associated with infections (after URI or GI infection), inflammatory bowel disease, pregnancy, autoimmune diseases (RA, SLE, dermatomyositis, sarcoid), malignancies (more commonly hematologic malignancies), and drug related (most common is G-CSF).

Clinical Presentation:

·     Patients will have abrupt onset of painful inflammatory papules, plaques, or nodules. The distribution will be asymmetric with the upper extremities most commonly effected.

·     Often associated with fever, arthralgias, headache, myalgias, and malaise.

·     Extracutaneous involvement can occur, frequently ocular and musculoskeletal. However additional organ systems can also become involved.

·     Laboratory studies often show a peripheral leukocytosis with neutrophilia. An elevated erythrocyte sedimentation rate and C-reactive protein is common, along with anemia and platelet abnormalities.

Diagnosis:

·     Diagnostic criteria has been established – both major criteria is required along with two of four minor required.3

o     Major Criteria:

§     Abrupt onset of painful erythematous plaques or nodules

§     Histopathologic evidence of a dense neutrophilic infiltrate without evidence of leukocytoclastic vasculitis

o     Minor Criteria:

§     Fever, > 38 C

§     Association with underlying maligiancy, inflammatory disease, or pregnancy OR preceded by URI, GI infection, or vaccination

§     Excellent response to treatment with systemic glucocorticoids or potassium iodine

§     Abnormal laboratory values at presentation (need 3 of the 4: ESR > 20 mm/hr, positive C-reactive protein, > 8,000 leukocytes, > 70% neutrophils).

Treatment:

·     Corticosteroid therapy is first-line. Typically start with prednisone at 0.5 to 1mg/kg per day.

·     After initiation of steroid therapy, symptoms are expected to improve within 48 hours.

·     Once disease control is obtained, can begin to taper steroids with a plan to discontinue steroids within 4-6 weeks.

·     Other therapies such as colchicine, dapsone, and potassium iodide have been used as steroid-sparing agents.4

References:

1.     Cohen PR, Kurzrock R. Sweet’s syndrome revisited: a review of disease concepts. Int J Dermatol 2003; 42:761-778.

2.     Vignon-Pennanmen MD. The extracutaneous involvement in the neutrophilic dermatoses. Clin Dermatol 2000;18:339–47.

3.     von den Driesch P. Sweet’s syndrome (acute febrile neutrophilic dermatosis). J Am Acad Dermatol 1994; 31:535.

4.     Cohen PR, Kurzrock R. Sweet’s syndrome: a review of current treatment options. Am J Clin Dermatol 2002; 3:117.