Immune Reconstitution Inflammatory Syndrome Associated with Pneumocystis jirovecii Pneumonia and Cytomegalovirus Colitis in a Patient with Rheumatoid Arthritis.

A 68-year-old woman presenting with rheumatoid arthritis was admitted due to pancytopenia caused by methotrexate. Pneumocystis jirovecii pneumonia was diagnosed based on the abnormal shadows observed on chest computed tomography, the presence of serum ß-D-glucan, and positive P. jirovecii-DNA results in a sputum analysis. Subsequently, after treatment with leucovorin and trimethoprim-sulfamethoxazole, lung consolidation was found to be aggravated, along with a rapidly increasing leukocyte count. In addition, cytomegalovirus colitis was diagnosed. Both conditions were associated with immune reconstitution inflammatory syndrome caused by recovery from leukopenia. The patient was successfully treated with intravenous methylprednisolone pulse therapy and ganciclovir.

Characteristic Renal Histology of a 81-Year-Old Patient with a 30-Year History of Diabetes Mellitus: A Case Report.

A renal histology of an 81-year-old man with a 30-year history of diabetes mellitus (DM), as well as diabetic retinopathy and neuropathy, was examined. The patient's blood pressure was controlled within the normal range (less than 140/75 mmHg) using antihypertensive agents including angiotensin receptor blocker. Edematous management was achieved by a strict salt diet (less than 6 g/per day). However, this patient's glycemic control was poor with HbA1c 8-10%. Serum creatinine was 0.87 mg/dL and estimated globular filtration rate (eGFR) was 64 ml/min/1.73m2. Urinary protein excretion was 1.5 g/day. This patient's renal biopsy showed linear staining for IgG along the GBM by immunofluorescence microscopy, but light microscopy showed almost intact glomeruli, and the GBM was not thickened as revealed by electron microscopy with a width of 288-368 nm (< 430 nm). While arteriolar hyalinosis was severe, and polar vasculosis was observed around the glomerular vascular pole. This case indicates that long-standing hyperglycemia may induce polar vasculosis by the mechanism of angiogenesis, but diabetic glomerulopathy can become minor change, only when hypertension and edematous management could be controlled strictly.

Salmonella enterica Subspecies arizonae Detected from Bilateral Pleural Fluid in a Patient with Systemic Lupus Erythematosus and Malignant Lymphoma.

A 72-year-old woman was admitted to our hospital with bilateral pleural effusions. She had a 31-year history of systemic lupus erythematosus and had been treated with prednisolone and azathioprine. Pleural fluid culture revealed Salmonella enterica subsp. arizonae infection. This pathogen rarely infects humans but is commonly found in the gut flora of reptiles, especially snakes. Our patient had not come in contact with reptiles. Despite antibiotic therapies and negative pleural cultures, the pleural effusion persisted. Colon cancer was detected concomitantly, and she finally died. The autopsy revealed that the pleuritis was due to underlying diffuse large B cell lymphoma.

A Non-smoking Woman with Anti-phospholipid Antibodies Proved to Have Thromboangiitis Obliterans.

A 48-year-old woman with severe pain and numbness of her right leg and foot was admitted to our hospital. She had never smoked and had little exposure to passive smoking. Initially, polyarteritis nodosa with anti-phospholipid antibodies was considered. Combination therapy with methylprednisolone pulse therapy, intravenous cyclophosphamide pulse therapy, vasodilators, antiplatelet agents, and anticoagulants was not effective. Vasculopathy was progressive, and she presented with gangrene of the toes. She required amputation of her right leg. The pathological findings of the amputated leg revealed thromboangiitis obliterans (TAO). TAO should be considered even in non-smoking women. Non-response to immunosuppressant and anticoagulant therapies may be a clue to the diagnosis of TAO.

Arteriovenous fistula-related renal bleeding 5 days after percutaneous renal biopsy.

A 32-year-old Japanese woman was admitted to our hospital for evaluation of microscopic hematuria with a positive family history. Percutaneous renal biopsy was performed under real-time ultrasound guidance using a 16-gauge automated needle and three specimens were obtained. She had no risk factors for hemorrhage. However, macroscopic hematuria developed from 5 days after biopsy and persisted for 4 days. Her Hb decreased markedly from 15.0 to 8.1 g/dL. Enhanced computed tomography revealed urinary tract hematoma, while the early arterial phase showed inflow of contrast medium into the left renal vein from a pseudoaneurysm on a branch left renal artery. Renal transcatheter arterial embolization was performed using platinum microcoils and the arteriovenous fistula was occluded. The patient did not require blood transfusion. Severe renal bleeding that causes urinary tract hematoma usually occurs within 24 h after renal biopsy, but the possibility of late-onset renal bleeding should be kept in mind.

Simultaneous Oculomotor and Facial Nerve Palsies in a Patient with Systemic Lupus Erythematosus and Sjögren's Syndrome.

A 70-year-old man with systemic lupus erythematosus (SLE) presented with simultaneous right oculomotor nerve palsy and right facial nerve palsy. Brain magnetic resonance imaging and cerebrospinal fluid analysis revealed no abnormality. Coexistent Sjögren's syndrome was diagnosed on the basis of anti-SS-A antibody positivity, salivary gland scintigraphy, and histological findings on minor salivary gland biopsy. As there was no obvious cause of multiple cranial neuropathies, we supposed that the palsies were induced by either of the underlying diseases. The patient was treated with a high-dose of prednisolone and intravenous cyclophosphamide, and both palsies recovered almost completely within two weeks.

Eltrombopag Improves Refractory Thrombocytopenia in a Patient with Systemic Lupus Erythematosus.

A 42-year-old woman with systemic lupus erythematosus (SLE) was admitted to our hospital for evaluation of severe thrombocytopenia. She was treated with steroids, intravenous cyclophosphamide, intravenous immunoglobulin, and plasma exchange, but her thrombocytopenia did not improve. Renal biopsy showed class IV-S(C) + V lupus nephritis, according to the classification of the International Society of Nephrology/Renal Pathology Society. The PA-IgG and serum thrombopoietin (TPO) levels were elevated. Her thrombocytopenia responded to off-label administration of eltrombopag, which was discontinued after 42 months. At 18 months after stopping eltrombopag, the platelet count was 19.3 × 104/µL. Eltrombopag may be a therapeutic option for SLE patients with severe thrombocytopenia refractory to conventional therapy.

Multicentric Reticulohistiocytosis with Dermatomyositis-like Eruptions.

A 68-year-old man presented with polyarthritis, proximal muscle weakness, and erythema of the face, arms, neck, and anterior chest that resembled the V-neck sign. Initially, dermatomyositis (DM) was considered because of the erythema, polyarthritis, and muscle weakness. He also had mediastinal and hilar lymphadenopathy on contrast-enhanced computed tomography. Unexpectedly, a biopsy of the forehead skin revealed numerous multinucleated giant cells. A biopsy of a solitary nodule on the dorsum of his right middle finger revealed similar multinucleated giant cells with ground-glass cytoplasm, leading to the diagnosis of multicentric reticulohistiocytosis (MRH). Although MRH is rare, it should be remembered that MRH can mimic DM.