A Unusual Rare Case of Kikuchi-Fujimoto
Horizon Hospital Successfully Treats A Young Woman With Extremely Rare Disease named Kikuchi-Fujimoto
Thane based Horizon Group of Hospitals, recently treated a 40-year-old young woman who was affected with an extremely rare Kikuchi-Fujimoto disease. Priya Awasthi (name changed) from Ambernath which is an eastern suburban city located in Thane district, a part of Mumbai was admitted to Horizon Hospital and was recently diagnosed with Kikuchi – Fujimoto disease, a very rare acute disorder of lymph nodes. The disease was first reported in Japan in 1972. Since then, it has infected only around 110 people across the world.
Dr. Hrushikesh Vaidya, India’s Leading Pulmonologist and Chairman Horizon Group of Hospitals said “Kikuchi-Fujimoto Disease is a rare diagnosis which presents with a constellation of symptoms including fevers, malaise, weight loss, and cervical lymphadenopathy. Because the disease process is uncommon and the symptoms are non-specific, the diagnostic process can be lengthy and challenging”.
Ms. Priya Awasthi, 40 year old unmarried woman is the third daughter amongst the four daughters of Awasthi family. Priya used to work in a private firm in Mumbai and lives with her father and two sisters. Post her mother’s death two and half years ago due to heart failure, the family was just slowly gaining strength that Priya suddenly fell sick. Initially, Priya first started losing weight from June 15, 2024, despite of having proper diet. She was weighing 45 kgs and in 15 days’ time she lost almost 5 kgs. Then, she developed intermittent fever that was ranging between 101-102 degree.
Post this, in the next 15 days loose motion also started. Then immediately she consulted a local General Physician Dr. Sameer Kulkarni who treated her for a week’s period but the fever was not coming down. Along with fever, appeared redness in the tip of the nose. Little coughing was there but because of fever, loosemotions etc she actually couldn’t eat anything much. She was tested for Malaria, Dengue, Typhoid and all tests came negative.
As there was no improvement in her health, Priya consulted another doctor for second opinion. Dr. Sushil Sabnis initially treated her for the first three days by giving injections for malaria. Simultaneously her X-Ray was done and it showed lower respiratory system bacterial infection. Medication was prescribed for a week but unfortunately there was no respite from the disease. The disease only kept progressing and no doctors were able to identify it. In the meanwhile, she consulted her family physician Dr Kalmath Basavaraj who also prescribed medicines for malaria but her fever was only shooting up and it ranged between 102-103 degree. Finally Dr Kalmath suggested that she needs to be admitted.
Thus, finally she was admitted to Horizon Hospital on July 19, 2024 with chief complaints of Fever, Loose motions, vomiting and Weight Loss.
Various diagnostic procedures were done during this one month stay at hospital. LRTI – Sputum, Systemic Lupus Erythematosus, Secondary HLH and Kikuchi Lymphadenitis. Various radiology procedures also were done that included USG abdomen and pelvis, HRCT chest plain, 2D echo, CT abdomen, PET scan- whole body. Besides this, two procedures also were conducted Cervical Lymph Node Biopsy that was done by Dr Sunil Bangera on August 4th and Bone Marrow Aspiration was done by Dr. Ashray Kolhe on August 7th.
As soon as, Priya was admitted with complaints of intermittent fever associated with loose motion and vomiting for 15 days, the hospital performed all routine investigations leukopenia and thrombocytopenia, fever profile and all reports were found negative. Priya was attended by Dr. B. C. Kalmath and was examined thoroughly. She was initially advised USG abdomen and pelvis and medical management. The test showed right ovarian cyst with minimal free fluid in pelvis. 2 D echo results found were (Left Ventricular Ejection Fraction) LVEF- 60%, No RWMA seen, No MS (Mitral Stenosis) /MR (Mitral Regurgitation). Priya had fever and persistent cough, so HRCT chest done on July 23rd to find out infection’s etiologies. So Sputum culture was taken and the report showed E. coli growth. According to the reports, antibiotics were started. But still the fever was shooting up but that was associated with development of rash at the tip of the nose firstly and then the redness was found in thumb finger, index finger and both the ears and hence dermatologist opinion was also taken. Priya was attended to by Dr. Ruchita Hasija, Consultant Dermatologist and was examined thoroughly. She was prescribed medications and was advised specific tests ANA Profile (anti-immune, anti-nuclear and anti-body) to rule out SLE (systemic lupus erythromitus), SOS biopsy. Priya’s ANA report showed ANA blot positive-SM/RNP, SM and SM -70 positive. So accordingly, Dematologist prescribed medications.
Post this, she was also seen by Rheumatologist Dr. Milind Ghare. She was advised CT abdomen and Pelvis with Sr. TB Gold and Medical management and in view of ANA reports, patient was started on IV steroids.
CT abdomen with contrast was done subjective of exophytic subserosal uterine fibroid, Right ovarian haemorrhagic cyst. In view of persistent high grade fever spikes even after steroidal doses, Infectious disease specialist Dr. K. Dawda’s opinion was also taken and he advised investigations to conduct cytopenia and axillary lymph node core biopsy and refer to a Haematologist.
Post which Haematologist Dr. Ashray Kolhe’s opinion was taken and after examining the patient thoroughly, he advised lymph node biopsy and (SOS) PET CT scan. Post that General Surgeon Dr. Sunil Bangera opinion was taken and he advised cervical or axillary lymph node biopsy. After informing the patient and seeking her written consent, cervical lymph node biopsy was done by Dr. Sunil Bangera on August 4, 2024 and sample was sent for investigation. On examination of cervical lymph-nod, caseating lymph node where found. Dr. Dawda post seeing the reports advised to start AKT from August 5, 2024. As Priya still had persistent fever spikes, CBC subject to low TLC count was found. Hence Dr. Ashray Kolhe opinion was taken again and he immediately advised bone marrow aspiration and biopsy. To take neutropenic precaution, Priya was immediately shifted to isolation room and hertemperature’s were monitored continuously.
Since Priya was having throat pain, after examining she was diagnosed with Oral candidiasis. Hence anti-fungal medications also were started immediately.
Post informing her and taking her written consent, bone marrow aspiration was performed on August 7th and the samples were sent for further investigations. Besides, she was also advised PET scan subject to infective etiology in lung, Mild bilateral pleural effusion, Mild pericardial effusion, low to mild grade metabolically active bilateral cervical and bilateral axillary lymph nodes subjective of infective aetiology, minimal ascites.
Bone marrow aspiration reports were shown to Gene expert and there was nothing detected. Her culture reports were still awaited. The Cervical biopsy was also showed to Gene expert and nothing was found and her TB- gold reports too were negative. Irrespective of starting AKT and awaiting bone aspiration report patient complaint of fever, hence HLH was suspected by Haematologist and sr. fibrinogen, sr. triglycerides, SGOT, SGPT and sr. ferritin tests were done and reports were sent. Priya’s Sr. ferritin and triglycerides reports showed increased level. Hence Hemophagocytic lymphohistiocytosis (HLH) was diagnosed. Cervical biopsy histopath showed Kikuchi lymphadenitis. Post reviewing the reports with Rheumatologist and Haematologist, patient was started with T. Naprosyn and steroids and AKT was withheld.
Dr. Hrushikesh Vaidya said “Since Priya’s case was difficult to be identified, we had to take a multi-disciplinary treatment model approach. In the hospital, she was attended by 6 other consultants at various stages viz., Dermatologist, Rheumatologist, Chest Physician, Infectious Disease Specialist, General Surgeon and Haematologist. She was finally discharged nearly a month after on August 12, 2024”.
Priya’s condition started improving gradually and slowly her vitals were stabilised. Priya did not have fever that was recorded by the hospital for 48 hours. She was slowly gaining mobility out of bed and hence discharged in haemodynamically stable state.
Dr Hrushikesh Vaidya said “Priya has been advised Hyperbaric Oxygen Therapy (HBOT) which is a type of treatment that is used to speed up healing of wounds that won’t heal. It is also used for infections in which tissues are starved for oxygen”.
Dr Hrushikesh Vaidya said “Kikuchi is a very rare disorder of lymph nodes. There are no regular statistics on the number of Kikuchi-Fujimoto infected patients in our country. I have never treated such a case in my professional career spanning over two decades. Her symptoms were a little confusing, so she was advised to go for a biopsy. The test showed that it is a case of rare Kikuchi-Fujimoto disease, The disease might be fatal if it’s not detected and treated on time. She was treated in the hospital for nearly a month and has been discharged recently. We couldn’t find any specific reason that might have caused the disease. Therefore, we opted for only symptomatic treatment. And she requires to follow-up for at least six months from now”.
Kikuchi–Fujimoto disease (KFD), which is also known as histiocytic necrotizing lymphadenitis, is a rare cause of lymphadenopathy, that was first recorded in Japan in 1972 by Kikuchi and Fujimoto. It is an extremely rare benign, self-limited disease. The condition is also characterized by fever, night sweats, fatigue and to a lesser extent skin rash and joint pain. The exact cause of KFD is still obscure. Kikuchi and Fujimoto originally identified this pathology among Japanese and other Asiatic women; the condition has since been defined worldwide in both genders and in a variety of ethnic backgrounds, with higher prevalence among females. Although KFD is considered a benign condition, the significant morbidity with frequent hospitalization may be attributed to mistaken diagnosis with other lymphoproliferative disorders, connective tissue diseases or infectious etiologies.
Affected patients with KFD may need to be followed for years due to the possibility of association with systemic lupus erythematosus. In addition, recurrences of Kikuchi disease can occasionally continue for many years. Early detection and treatment of KFD to control active inflammation is crucial, owing to the presumptive reduction in the risk of consequent limb swelling and permanent lymphedema.
In conclusion, KFD may still be under-diagnosed in our part of the world, likely due to the non-specific mixed symptoms at presentation as well as due to the lack of awareness among general practitioners and health care providers. Early diagnosis with tissue biopsy and treatment is essential to reduce associated morbidity.
