A case of TAFRO syndrome maintained in remission for 5 years after discontinuation of tocilizumab.

TAFRO syndrome is a subtype of idiopathic multicentric Castleman's disease (iMCD) that is characterised by thrombocytopenia, generalised oedema, fever, bone marrow fibrosis, renal failure, and organ enlargement and has a poor prognosis. The prognosis of TAFRO syndrome is worse than that of iMCD-not otherwise specified, with a high mortality rate. There are only a few long-term follow-up reports after remission of TAFRO syndrome with tocilizumab (TCZ) treatment in a patient in whom all drugs were discontinued after attaining sustained remission. Here, we report a case in which interleukin-6 negativity was confirmed and remission was maintained without relapse for 5 years after all drug treatments, including TCZ, were discontinued.

Recent Outpatient Services in General Internal Medicine: 6.4% of Symptomatic Patients Were Post-Acute COVID-19.

PURPOSE: It is important to understand how COVID-19 is affecting general outpatient services, since hospitals see a wide variety of patients. This study aimed to evaluate the incidence and clinical picture of COVID-19 in general outpatient services. Currently, the diagnosis of COVID-19 depends strongly on the results of polymerase chain reaction (PCR) assays. However, since the sensitivity of PCR tests for SARS-CoV-2 is not high enough to assure confidence. On the other hand, the SARS-CoV-2 antibody (Ab) test is highly sensitive after 2 weeks of symptom onset, and might complement the PCR test. Therefore, we measured Ab in addition to PCR to obtain a more accurate clinical profile of COVID-19, which might be helpful in building future practice strategies. PATIENTS: The study patients were those who visited our department for the first time between November 2020 and March 2021 and fulfilled the enrolment criteria. METHODS: All patients underwent total SARS-CoV-2 Ab testing, and PCR tests were performed in only some of them; patients were classified according to the performance of PCR tests for comparisons. RESULTS: Ninety-four patients who underwent Ab testing during the study period were eligible for study enrolment, and the PCR test was performed in 40 of them. Only one patient was diagnosed with acute stage COVID-19 based on a positive PCR test. Antibodies were positive in six (6.4%) of the 94 patients. Five of the six Ab-positive patients were negative for PCR, and the test was not performed in the sixth patient. All the six patients had prior symptoms suggestive of infection, and respiratory symptoms were more noticeable than fever. The Ab positivity rate was significantly higher than published data. CONCLUSION: COVID-19 is not rare in general outpatient services and can be missed based on PCR tests alone. The diagnosis should be made from a comprehensive perspective.

Exacerbation of Ulcerative Colitis with Tocilizumab: A Report of Two Cases, One with Takayasu Arteritis and the Other with Relapsing Polychondritis.

Tocilizumab (TCZ), a biologic that blocks the signal transduction of interleukin-6, has been used for the treatment of various autoimmune diseases. Many of these cases are sometimes complicated by ulcerative colitis (UC). However, the effect of TCZ on UC is unclear. We experienced two cases with concomitant UC that were treated with TCZ, one for Takayasu arteritis (TAK) and the other for relapsing polychondritis (RP). TCZ did not improve UC in either of these cases. TCZ might have adverse effects on the intestinal tract, since interleukin-6 signaling plays an important role in intestinal epithelium maintenance. Treatment with TCZ should therefore be carefully provided in patients complicated with UC.

Acute Urinary Retention Induced by Chemical Meningitis Which Occurred Due to a Ruptured Dermoid Cyst.

Meningitis retention syndrome (MRS), a rare complication of aseptic meningitis, can present with acute urinary retention. The rupture of a dermoid cyst, which is a benign intracranial tumor, can sometimes induce chemical meningitis. We herein present a case of chemical meningitis and acute urinary retention that was induced by the rupture of a dermoid cyst. The patient experienced urinary retention for approximately 60 days, and then made a complete recovery thereafter. This is the first reported case of acute urinary retention due to the rupture of a dermoid cyst.

Familial Mediterranean fever with P369S/R408Q exon3 variant in pyrin presenting as symptoms of PFAPA.

Familial Mediterranean fever (FMF) can be classified into typical and incomplete/atypical types. Periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis (PFAPA) syndrome-like symptoms have been found in atypical type carrying P369S-R408Q mutations in the responsible gene MEFV. A 28-year-old female with recurrent fever and her young sisters and mother, all of whom had tonsillectomy for tonsillitis, carried heterozygous alterations involving E148Q/P369S/R408Q. A diagnosis of atypical FMF, MEFV exon3 variants with PFAPA syndrome-like symptoms, was made.

A case of chronic active Epstein-Barr virus infection mimicking adult-onset Still's disease.

An 83-year-old man was diagnosed with adult-onset Still's disease (AOSD) based on clinical and laboratory findings. However, glucocorticoid had little effect. Epstein-Barr virus (EBV)-DNA was detected in peripheral blood, and autopsy findings confirmed a diagnosis of chronic active EBV infection (CAEBV). CAEBV mimics AOSD, and the presence of articular involvement and leukocytosis does not exclude the possibility of CAEBV. CAEBV should be included in the differential diagnosis of AOSD, and measurement of EBV-DNA is essential.

Pulmonary and gastric metastatic calcification due to milk-alkali syndrome: a case report.

The incidence of metastatic calcification is influenced by high serum calcium and phosphate concentrations and local physicochemical conditions, such as pH. A high pH accelerates tissue calcification. Patients with milk-alkali syndrome typically present with renal failure, hypercalcemia, and metabolic alkalosis, which are caused by the ingestion of calcium and absorbable alkali. Among patients with impairment of renal function, milk-alkali syndrome is a major cause of hypercalcemia. Long-term use of furosemide will lead to hypokalemia, metabolic alkalosis, and eventually renal failure (i.e., pseudo-Bartter syndrome). Even if the level of calcium ingestion is relatively low, the renal failure caused by long-term furosemide use can readily lead to milk-alkali syndrome. We describe a case of a 45-year-old woman who was admitted with cough and dyspnea and presented with pulmonary and gastric metastatic calcification. She had been taking alfacalcidol and oral alkaline medications such as sodium bicarbonate and calcium carbonate as well as oral furosemide for a long time. The patient was found to have hypercalcemia, chronic renal failure, and metabolic alkalosis, so milk-alkali syndrome was diagnosed. Saline was administered and oral medications were discontinued. Serum creatinine levels subsequently decreased, but pulmonary metastatic calcification was not diminished. In this case, the milk-alkali syndrome that caused the severe metastatic calcification was exacerbated by multiple factors, including oral alkaline medications such as sodium bicarbonate and calcium carbonate. In addition, metabolic alkalosis and renal failure were affected by long-term furosemide use (i.e., pseudo-Bartter syndrome).

Churg-Strauss syndrome concomitant with chronic symmetrical dacryoadenitis suggesting Mikulicz's disease.

A case of Churg-Strauss syndrome complicated by chronic symmetrical dacryoadenitis suggestive of Mikulicz's disease is herein presented. A 72-year-old Japanese man, who had been previously diagnosed with asthma, presented with weakness of the left leg and purpura on the lower extremities. A neurological examination showed multiple mononeuropathies and a laboratory examination revealed elevated eosinophil counts, IgE levels and the presence of Myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCAs). Churg-Strauss syndrome was diagnosed, although the patient also exhibited bilateral swelling of the lachrymal glands. Furthermore, elevated serum IgG4 levels, an infiltration of a relatively large number of IgG4-positive plasmacytes in the nasal mucosa and hypocomplementemia were also observed. These findings were consistent with a diagnosis of Mikulicz's disease (MD). Oral prednisolone (30 mg) was administered and the swelling of the lachrymal glands resolved. Churg-Strauss syndrome may be accompanied by Mikulicz's disease (an IgG4-related disease), and common pathogeneses between Churg-Strauss syndrome and IgG4-related disease may exist.