The parents of a child with primary Sjögren syndrome are counseled on long-term risks associated with the disease. Due to chronic inflammation and monoclonal B-lymphocyte proliferation originating within the affected glands, these patients are at a significantly increased lifetime risk of developing which specific type of malignancy?
A 16-year-old female with a multi-year history of Systemic Lupus Erythematosus presents with new-onset hypertension and lower extremity edema. A renal biopsy is performed to stage her lupus nephritis. The pathologist reports diffuse proliferative glomerulonephritis. According to the International Society of Nephrology classification, which histologic class corresponds to this finding and carries the most significant risk for renal morbidity?
A 13-year-old cross-country runner presents with several weeks of bilateral knee pain that is worse with activity and better with rest. When evaluating a child for musculoskeletal pain, which physical examination finding would most strongly suggest a non-inflammatory, mechanical cause like an overuse syndrome rather than an inflammatory arthritis?
A pathologist examining a lymph node biopsy from a child with suspected Sarcoidosis identifies multiple non-caseating granulomas. The formation of these granulomas is a T-cell-mediated process. Which cytokine, released by type 1 helper T cells, is crucial for promoting the proliferation of lymphocytes within the granuloma?
A 9-year-old with known Systemic Juvenile Idiopathic Arthritis develops Macrophage Activation Syndrome (MAS) that is refractory to high-dose corticosteroids. You decide to add a second agent that acts predominantly on T cells and is highly effective in the treatment of MAS. Which medication best fits this description?
A 7-year-old girl is evaluated for difficulty getting out of bed and a new rash. On examination, you note symmetric proximal muscle weakness and a bluish-violet discoloration of her eyelids with some periorbital edema. Which of the following is the name for this pathognomonic rash of the most likely diagnosis?
A 14-year-old female presents with a 2-month history of fatigue and symmetric joint pain involving her wrists, knees, and small joints of the hands. On examination, she has mild synovitis and an erythematous facial rash. Laboratory evaluation reveals a white blood cell count of 3,200/μL (normal 4,500-11,000/μL), positive antinuclear antibody at 1:320, and low C3 and C4. What is the most likely diagnosis?
A 12-year-old girl is diagnosed with Polyarteritis Nodosa after presenting with fever, myalgias, and livedo reticularis. Biopsy of a skin nodule shows necrotizing inflammation of medium-sized arteries. Which of the following findings would be UNEXPECTED in this specific type of vasculitis?
A 3-year-old with Kawasaki Disease has a persistent fever 48 hours after completing an initial infusion of intravenous immunoglobulin (IVIG). You diagnose IVIG-resistant disease. Which of the following is considered a primary therapeutic option for this situation?
A 10-year-old girl presents with a well-circumscribed, waxy, ivory-colored area of indurated skin on her trunk, surrounded by a faint violaceous halo. The lesion appears to be confined to the dermis. What is the most appropriate classification for this type of juvenile localized scleroderma?
