Patient: [Patient Name]
MRN: [Medical Record Number]
Date: [Date of Encounter]
Chief Complaint:
New onset of skin thickening and hardening, particularly involving the extremities
(arms and legs).
(Depending on severity) Joint pain, stiffness, restricted movement.
History of Present Illness:
Onset, duration, and progression of skin thickening and hardening.
Location (typically starts on the extremities) and distribution of skin changes.
Associated symptoms (e.g., joint pain, stiffness, pruritus (itching), redness).
Recent exposure to gadolinium-based contrast agents (GBCAs) for imaging studies (MRI scans). Specify the type of GBCA used (linear vs. macrocyclic) if known.
Underlying kidney function (known or suspected).
History of chronic kidney disease (CKD) or prior episodes of acute kidney injury (AKI).
Past Medical History:
Underlying medical conditions, particularly those affecting kidney function (e.g., diabetes, hypertension, autoimmune diseases).
Previous surgeries or procedures involving GBCA use.
Social History:
Occupational exposures to gadolinium (rare).
Family History:
No familial association with NSF.
Physical Exam:
Skin examination: Assess for thickening and hardening of the skin, particularly on the extremities (extensor surfaces of forearms, hands, legs, feet).
Describe the texture, color, and any associated rash or ulcerations.
Musculoskeletal examination: Evaluate for joint pain, stiffness, and restricted movement (range of motion).
Neurological examination: Assess for sensory or motor deficits if suspected
(rare in NSF).
Laboratory Tests:
Kidney function tests: Serum creatinine, Blood Urea Nitrogen (BUN), electrolytes.
Deranged kidney function may be present, but is not diagnostic of NSF.
Gadolinium levels: Serum gadolinium level may be elevated in some cases
of NSF, but the test is not routinely used due to limitations in interpretation.
Imaging Studies:
Skin biopsy: Histopathological examination of a skin sample is essential for diagnosis of NSF. Biopsy findings typically show collagen deposition in the dermis and subcutaneous tissue.
MRI scan (without gadolinium): May be used to assess the extent of skin thickening and involvement of deeper tissues, but is not diagnostic for NSF.
Assessment:
Nephrogenic systemic fibrosis (suspected or confirmed): Based on clinical presentation (skin thickening, joint involvement) and supportive findings on skin biopsy.
Severity of NSF: Classified based on the extent of skin involvement and functional limitations.
Risk factors: Recent exposure to gadolinium-based contrast agents (GBCA) and underlying kidney dysfunction are the main risk factors for NSF.
Differential Diagnoses:
Consider other conditions that can cause skin thickening and hardening, especially early in the disease course:
Scleroderma (systemic sclerosis)
Localized scleroderma (morphea)
Erythema nodosum leprosum (associated with leprosy)
Elephantiasis (lymphatic obstruction)
Plan:
There is no cure for NSF, but the focus is on managing symptoms and preventing progression. Possible elements include:
Avoidance of further GBCA exposure: Crucial to prevent worsening of NSF.
Skin care: Emollients and moisturizers to improve skin hydration and reduce itching.
Physical therapy: Maintain joint mobility and function.
Medications: Corticosteroids or other medications may be used to suppress the inflammatory process, but their effectiveness is limited.
Consultations: Consider referral to a dermatologist and/or nephrologist for specialized care.
Prognosis:
The prognosis of NSF varies depending on the severity of involvement. Early diagnosis and avoidance of further GBCA exposure are crucial to slow progression.
Follow-up:
Regular follow-up appointments to monitor disease progression and manage symptoms.
Skin care education and ongoing support for the patient