Summary about Disease
Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a rare, benign, self-limiting inflammatory disorder that primarily affects cervical (neck) lymph nodes. It is often characterized by fever, lymphadenopathy (enlarged lymph nodes), and sometimes skin rash. Systemic Lupus Erythematosus (SLE) is a chronic autoimmune disease in which the body's immune system attacks its own tissues and organs. The coexistence of KFD and SLE is rare but reported. The combination poses diagnostic challenges as their symptoms can overlap, and KFD can sometimes be mistaken for lupus or lupus flare.
Symptoms
Kikuchi-Fujimoto disease (KFD):
Enlarged, tender lymph nodes, usually in the neck
Fever
Night sweats
Fatigue
Skin rash (less common)
Headache
Sore throat
Systemic Lupus Erythematosus (SLE):
Fatigue
Joint pain, stiffness, and swelling
Butterfly-shaped rash on the face
Skin lesions that worsen with sun exposure (photosensitivity)
Fever
Chest pain
Shortness of breath
Headaches, confusion, memory loss
Dry eyes
Hair loss
Raynaud's phenomenon (fingers and toes turn white or blue in cold temperatures)
Causes
Kikuchi-Fujimoto disease (KFD): The exact cause of KFD is unknown. However, viral infections (such as EBV, HHV-6, parvovirus B19), autoimmune conditions, and genetic predispositions have been suggested as possible triggers. It is considered an inflammatory response to an unknown agent.
Systemic Lupus Erythematosus (SLE): SLE is an autoimmune disease. The immune system attacks healthy tissues. The exact cause is not fully understood, but genetic factors, environmental triggers (such as sunlight and infections), and hormones are believed to play a role.
Medicine Used
4. Medicine used
Kikuchi-Fujimoto disease (KFD):
Pain relievers (analgesics) such as acetaminophen or ibuprofen for fever and pain
Nonsteroidal anti-inflammatory drugs (NSAIDs) for inflammation
Corticosteroids (such as prednisone) may be used in severe cases to reduce inflammation
Systemic Lupus Erythematosus (SLE):
Hydroxychloroquine (an antimalarial drug)
Corticosteroids (such as prednisone)
Immunosuppressants (such as methotrexate, azathioprine, mycophenolate mofetil, cyclophosphamide)
Belimumab (a biologic drug)
Anifrolumab (a type I interferon receptor antagonist)
NSAIDs for pain and inflammation
Is Communicable
Neither Kikuchi-Fujimoto disease (KFD) nor Systemic Lupus Erythematosus (SLE) is communicable. They are not infectious diseases that can be spread from person to person.
Precautions
Kikuchi-Fujimoto disease (KFD):
Rest and adequate hydration
Avoidance of potential triggers (if any are identified)
Follow medical advice regarding medication and follow-up
Systemic Lupus Erythematosus (SLE):
Sun protection (wear protective clothing, use sunscreen)
Stress management
Regular exercise (as tolerated)
Healthy diet
Avoiding smoking
Vaccinations (after consulting with a doctor)
Regular medical check-ups
How long does an outbreak last?
Kikuchi-Fujimoto disease (KFD): KFD is usually self-limiting, with symptoms typically resolving within 1 to 4 months.
Systemic Lupus Erythematosus (SLE): SLE is a chronic condition with periods of flares (increased disease activity) and remission (periods with few or no symptoms). Flares can last for weeks, months or even years, and the duration varies greatly among individuals.
How is it diagnosed?
Kikuchi-Fujimoto disease (KFD):
Lymph node biopsy is the gold standard for diagnosis. Histopathological examination of the biopsy sample reveals characteristic features of KFD.
Medical history and physical examination
Blood tests (complete blood count, erythrocyte sedimentation rate, C-reactive protein) to assess inflammation.
Systemic Lupus Erythematosus (SLE):
Medical history and physical examination
Blood tests:
Antinuclear antibody (ANA) test (positive in most SLE patients)
Anti-dsDNA antibody test
Anti-Smith (Sm) antibody test
Complement levels (C3, C4)
Complete blood count (CBC)
Kidney and liver function tests
Urinalysis
Imaging studies (chest X-ray, echocardiogram)
Kidney biopsy (if kidney involvement is suspected)
The American College of Rheumatology (ACR) criteria and/or Systemic Lupus International Collaborating Clinics (SLICC) criteria are used to help diagnose SLE based on a combination of clinical and laboratory findings.
Timeline of Symptoms
9. Timeline of symptoms
Kikuchi-Fujimoto disease (KFD):
Initial: Enlarged lymph nodes (often in the neck), fever.
Progression: Possible night sweats, fatigue, skin rash, sore throat.
Resolution: Symptoms gradually subside over 1-4 months.
Systemic Lupus Erythematosus (SLE):
SLE's timeline varies widely. Some people experience a gradual onset of symptoms over years, while others have a more sudden and severe presentation. Flares can occur unpredictably throughout the course of the disease.
Symptoms can appear and disappear, or change in severity over time.
Important Considerations
The coexistence of KFD and SLE can complicate diagnosis and management.
KFD can mimic lupus or lupus flare, leading to misdiagnosis or delayed diagnosis of SLE.
Patients with KFD should be monitored for the development of autoimmune diseases, including SLE.
When both conditions are present, treatment strategies need to be individualized, considering the specific symptoms and severity of each disease.
A rheumatologist and other specialists may be involved in the care of patients with KFD and SLE.