ABSTRACT
We report a case of IgG4-related disease with marked eosinophilia. A 79-year-old woman was admitted due to diarrhea, and weight loss. Cervical lymphadenopathy, bilateral submandibular glands swelling, anemia (Hb8.5g/dl), hypereosinophilia (9,750/µL), and elevated serum creatinine (1.57 mg/dL), pancreatic amylase (191 IU/L), and IgG4 (3,380 mg/dL) were found. Diffusion-weighted image on MRI showed high intensity signals inside of both the pancreas and the kidney. The echogram of submandibular glands revealed cobblestone pattern. Kidney biopsy revealed acute tubulointerstitial nephritis. Biopsies of lip, gastrointestinal tract and bone marrow showed infiltration of lymphoplasmacytic cells and IgG4 positive plasma cells (30-67/HPF). Gastrointestinal and bone marrow biopsies also showed eosinophilic infiltration. Adrenal insufficiency, rheumatic disease, tuberculosis, parasite infection, drug induced eosinophilia, and eosinophilic leukemia were all ruled out. We started treatment with 40mg of prednisolone and her general condition rapidly improved. The eosinophil count, serum IgG4, and serum creatinine decreased. We gradually tapered prednisolone and maintained 5mg/day. During the 5 years of treatment, she had no recurrence of the symptom. According to the 2019 American College of Rheumatology/European League Against Rheumatism classification criteria for IgG4-related disease, eosinophils > 3000/µL is one of the exclusion criteria. If we comply this criterion, the diagnosis of IgG4-related disease should be avoided. However, our case fit the diagnostic criteria of type I autoimmune pancreatitis, IgG4-related sialadenitis and global diagnosis of IgG4-related disease. We finally diagnosed our case as IgG4-related disease with secondary hypereosinophilic syndrome. This case suggests that IgG4-related disease with eosinophils > 3000/µL does exist in the real world.
ABSTRACT
On 31 December 2019, cases of pneumonia whose cause was later identified as SARS-CoV-2 were detected in Wuhan City, Hubei Province of China, and now COVID-19 has spread worldwide. On March 1, 2020, a 69-year-old Japanese man who had been on hemodialysis for 3 years was diagnosed as having COVID-19 pneumonia and hospitalized at our Medical Center. Pulmonary CT revealed bilateral multiple consolidation with bilateral pleural effusion. Aggressive weight reduction was needed to improve the patient's respiratory condition. Hemodialysis therapy was performed in isolation with hydroxychloroquine administration, but the formation of a dialysis membrane clot forced the withdrawal of dialysis therapy. Changing the dialysis membrane material and anticoagulant enabled the resumption of dialysis therapy, allowing the body weight to correct downward. On the 5th hospitalization day, the patient's fever dropped and he showed improved oxygenation and chest X-ray. He was eventually discharged. The hydroxychloroquine and appropriate fluid management may have contributed to the patient's recovery. Clinicians should pay close attention to avoid dialysis-related problems when treating a patient with COVID-19.
