Using Middle Cerebral Artery Doppler Ultrasound to Predict Clinical Chorioamnionitis After Preterm Prelabor Rupture of Membranes.

BACKGROUND: In neonates, blood flow to the brain as measured by peak systolic velocity (PSV) in the middle cerebral artery (MCA) is altered in pregnancies affected by chorioamnionitis. OBJECTIVE: We aim to determine whether PSV and other measures of flow in the MCA in the fetus are altered prior to the development of clinical chorioamnionitis following preterm prelabor rupture of membranes (PPROM). METHODS:  This was a prospective observational study. Fifty patients from one institution were recruited after being diagnosed with PPROM between 23 weeks zero days and 33 weeks six days gestation. We performed measurements of the PSV in the fetal MCA on a weekly basis following PPROM and used the value taken closest to the time of delivery for our statistical analysis. The primary outcome assessed was clinical chorioamnionitis, and the exposure of interest was MCA PSV. Additional independent variables of interest were other Doppler measures of the MCA. Secondary outcomes included histological chorioamnionitis and other measures of neonatal health, including sepsis, days in the neonatal intensive care unit (NICU), and death. RESULTS: Of the 50 patients recruited to our study, eight (16%) developed clinical chorioamnionitis, similar to previously reported values in the general population. The PSV in the MCA was not significantly associated with the development of clinical chorioamnionitis. However, an elevated MCA pulsatility index (PI), a measure of resistance to flow, was associated with a higher probability of developing clinical chorioamnionitis. CONCLUSION:  There does not appear to be a difference in the PSV of the MCA of fetuses in pregnancies following PPROM with impending chorioamnionitis. However, elevated PI in the MCA could be a marker of impending chorioamnionitis in PPROM. Larger studies are needed to confirm these findings.

Oral Tongue Spontaneous Tumor Regression after Biopsy: A Case Report and Genomic Profile.

Spontaneous regression of a neoplasm is a rare oncologic phenomenon. Certain neoplasms, such as melanomas and neuroblastomas, display this phenomenon. To date, spontaneous regression of oral cavity squamous cell carcinomas has been documented in only a handful of case reports. We present a novel case of spontaneous regression of an oral tongue squamous cell carcinoma following biopsy. We discuss the tumor's unique genetic profile, immune response to cancer, and review the literature on possible mechanisms of spontaneous regression. Small-volume persistent cancer in our patient reinforces that tissue confirmation remains crucial to avoid missing remaining tumor. Further investigation is required to understand mechanisms of spontaneous regression and how these may be exploited to improve head and neck squamous cell carcinoma treatment.

A 24-Year-Old Man With Dyspnea and a Broken Left Femur.

CASE PRESENTATION: A 24-year-old White man presented with 1-day complaints of progressive shortness of breath and fever. He recently underwent an open reduction and internal fixation of a left midshaft femur fracture from a skiing accident 4 days ago. He denied chest pain, skin rashes, hemoptysis, hematemesis, melena, or surgical site bleeding. On arrival, the patient appeared in mild respiratory distress with a respiratory rate of 23 breaths/min, temperature of 37.8°C, heart rate of 97 beats/min, BP of 95/54 mm Hg, and peripheral saturation of 97% on 6-L/min nasal canula. His initial peripheral saturation on room air was 67%. Physical examination was unremarkable, except for diffuse rhonchi on chest auscultation. Chest radiograph on admission showed alveolar opacities predominantly in bilateral lower lobes. A chest CT angiography revealed no evidence for pulmonary embolism. However, there were findings of diffuse bilateral ground-glass opacities with areas of patchy consolidation and innumerous micronodules in both lungs (Fig 1). Laboratory examination was significant for a drop of hemoglobin by 3 g/dL and hematocrit level by 7% since his hospital discharge 4 days earlier. His renal function and urine analysis were normal. Venous blood gas on admission showed pH of 7.39 and Pco2 of 43 mm Hg. Because of unexplained acute anemia, nonspecific CT chest findings and progressive dyspnea, a bronchoscopy with BAL was performed. Four aliquots of 60 mL saline solution were injected for lavage with fluid return (Fig 2). BAL fluid showed WBC count of 0.411 × 103/mm3, RBC count of 318 × 103/mm3, 100% fresh RBCs, 73% neutrophil, 24% lymphocytes, 1% monocytes, and 2% eosinophils. BAL fluid cytologic condition is shown in Figure 3. A full vasculitis workup by rheumatology was unremarkable. Ophthalmologic and skin examination were unrevealing.

Lipoid Pneumonia.

Lipoid pneumonia occurs due to the accumulation of lipids within the lung tissue. Autopsy series have reported an incidence of 1.0-2.5% in adult and 8.8% in children. Lipoid pneumonia can be from an exogeneous or an endogenous source. Exogenous lipoid pneumonia is often associated with aspiration of fatty materials, whereas endogenous lipoid pneumonia is associated with an accumulation of lipid-rich debris from destroyed alveolar cells. We describe a 75-year-old man who presented with spiculated lung nodules found incidentally on abdominal CT. Reviews of systems were positive for weight loss, and a history of constipation. A PET/CT revealed spiculated nodules with positive fluorodeoxyglucose (FDG) uptakes. A wedge resection was performed with histopathologic findings consistent with exogenous lipoid pneumonia with granulomatous reaction. We report clinical, radiological, and pathological features of exogenous lipoid pneumonia secondary to chronic aspiration mimicking invasive adenocarcinoma. A high index of suspicion for exogenous lipoid pneumonia should be maintained, especially when evaluating patients with abnormal chest radiographic findings and risk factors for aspirations.

Scleroderma Renal Crisis Associated With Microangiopathic Hemolytic Anemia in a Patient With Seronegative Scleroderma and Monoclonal Gammopathy.

Systemic sclerosis with negative serology, particularly that complicated by scleroderma renal crisis (SRC), is rarely encountered. We describe a patient with seronegative systemic sclerosis who developed acute kidney injury, proteinuria, and hypertensive emergency following motor vehicle-related trauma and in the setting of nonsteroidal anti-inflammatory drug use. Findings on physical examination, imaging, and skin biopsy led to a clinical diagnosis of scleroderma, despite the lack of supportive laboratory data. IgG lambda paraproteinemia was detected on workup. Bone marrow biopsy showed plasmacytosis and trace lambda-restricted plasma cells consistent with monoclonal gammopathy of undetermined significance. Chemotherapy was initially started given concern for myeloma with cast nephropathy but was later stopped after a kidney biopsy revealed thrombotic microangiopathy (TMA). The SRC associated with TMA was ultimately diagnosed, though atypical hemolytic uremic syndrome (aHUS) induced perhaps by monoclonal gammopathy or hypertension was also possible. Captopril and eculizumab were initiated for SRC and aHUS, respectively. Despite therapy, renal function did not recover, and the patient required hemodialysis indefinitely. This case highlights clinical features common to both SRC and aHUS as well as points out a few ways to differentiate between them.

Sarcoidosis associated pleural effusion: Clinical aspects.

A sarcoidosis associated pleural effusion (SAPE) is a pleural effusion caused by active granulomatous inflammation from sarcoidosis. We describe the epidemiology, clinical features, diagnostic approach, treatment strategies and outcome of this condition. SAPE occurs in approximately 1% of sarcoidosis patients. The condition most commonly occurs at the initial presentation of sarcoidosis or within the first year. Dyspnea is the most common presenting symptom. Although a definitive diagnosis of SAPE requires a pleural biopsy, the diagnosis may be established on the basis of clinical features alone provided that alternative conditions can be reliably excluded. Pleural fluid analysis is essential in establishing the clinical diagnosis of SAPE. Corticosteroids are the drugs of choice for SAPE, and they are usually rapidly effective with courses of therapy often lasting less than two months. SAPE tends to have a low rate of recurrence that appears be to lower than for many other forms of sarcoidosis.

A 67-Year-Old Woman With Progressive Dyspnea and Innumerous Pulmonary Nodules.

CASE PRESENTATION: A 67-year-old woman, who recently immigrated to the United States from Afghanistan, presented to the hospital after sustaining a mechanical fall. She had no significant medical history and was not on any medication routinely. She denied any fever, night sweats, weight loss, shortness of breath, or hemoptysis. The patient had no prior personal history or exposure to TB. Results of a previous purified protein derivative skin test upon immigration were negative.

Disseminated Histoplasmosis with Concurrent Hairy Cell Leukemia.

Histoplasmosis is an endemic fungal infection that can lead to disseminated disease, especially in immunosuppressed patients. Hairy cell leukemia is a rare, slow-growing hematological malignancy. Concurrence of histoplasmosis and hairy cell leukemia is extremely rare. We describe a 69-year-old male who presented with fever, dry cough, pancytopenia, multiple pulmonary nodules, and massive splenomegaly. Histoplasma urinary antigen was positive and disease was confirmed by biopsy of lung lesions. Peripheral smear showed 'hairy cells', and bone marrow biopsy revealed findings of hairy cell leukemia. The patient was treated with intravenous amphotericin, followed by oral itraconazole. After the initial treatment of infection, treatment for hairy cell leukemia was started with cladribine. We will discuss the principles of treating disseminated histoplasmosis in the setting of immunosuppression, and hairy cell leukemia with coexisting infection.

Hemodialysis-associated soft tissue amyloidomas of the chest and abdominal wall.

Amyloidoma is a highly unusual presentation of amyloidosis in tumoral or nodular form. Isolated soft tissue amyloidomas in individuals with end-stage renal disease on chronic hemodialysis is exceedingly rare, particularly in the era of advanced dialysis technologies. We report the case of a 55-year-old male with end-stage renal disease due to autosomal-dominant polycystic kidney disease, on HD for over 30 years, who was found to have soft-tissue, dialysis-related (ß2 -microglobulin) amyloidomas (DRA). He presented with painful, palpable masses within the thoracic and abdominal walls. Serum ß2 -microglobulin level was only mildly elevated at 24.9 mg/L. Biopsy confirmed amyloidosis with positivity for Congo Red staining and apple-green birefringence under polarized light. Amyloid subtyping with immunohistochemistry showed positive ß2 -microglobulin staining within the deposits. Conservative therapy involving pain management and close monitoring resulted in eventual improvement in symptoms and thus proved to be a viable option for treatment.

Loss of Histone H3 K79 Methyltransferase Dot1l Facilitates Kidney Fibrosis by Upregulating Endothelin 1 through Histone Deacetylase 2.

BACKGROUND: The progression rate of CKD varies substantially among patients. The genetic and epigenetic contributions that modify how individual patients respond to kidney injury are largely unknown. Emerging evidence has suggested that histone H3 K79 methyltransferase Dot1l has an antifibrotic effect by repressing Edn1, which encodes endothelin 1 in the connecting tubule/collecting duct. METHODS: To determine if deletion of the Dot1l gene is a genetic and epigenetic risk factor through regulating Edn1, we studied four groups of mice: wild-type mice, connecting tubule/collecting duct-specific Dot1l conditional knockout mice (Dot1lAC ), Dot1l and Edn1 double-knockout mice (DEAC ), and Edn1 connecting tubule/collecting duct-specific conditional knockout mice (Edn1AC ), under three experimental conditions (streptozotocin-induced diabetes, during normal aging, and after unilateral ureteral obstruction). We used several approaches (colocalization, glutathione S-transferase pulldown, coimmunoprecipitation, yeast two-hybrid, gel shift, and chromatin immunoprecipitation assays) to identify and confirm interaction of Dot1a (the major Dot1l splicing variant in the mouse kidney) with histone deacetylase 2 (HDAC2), as well as the function of the Dot1a-HDAC2 complex in regulating Edn1 transcription. RESULTS: In each case, Dot1lAC mice developed more pronounced kidney fibrosis and kidney malfunction compared with wild-type mice. These Dot1lAC phenotypes were ameliorated in the double-knockout DEAC mice. The interaction between Dot1a and HDAC2 prevents the Dot1a-HDAC2 complex from association with DNA, providing a counterbalancing mechanism governing Edn1 transcription by modulating H3 K79 dimethylation and H3 acetylation at the Edn1 promoter. CONCLUSIONS: Our study confirms Dot1l to be a genetic and epigenetic modifier of kidney fibrosis, reveals a new mechanism regulating Edn1 transcription by Dot1a and HDAC2, and reinforces endothelin 1 as a therapeutic target of kidney fibrosis.

Chronic, Silent Microaspiration Masquerading as Interstitial Lung Disease.

Chronic, silent microaspiration is a common but underrecognized pathologic process in pulmonary medicine. The clinical presentation is variable and diagnosis can be challenging. We present the case of a 55-year-old woman with known emphysema, who was referred to us for progressive respiratory failure that was unresponsive to therapy. The patient had 9 hospital admissions in the preceding 5 months and was treated with multiple courses of antibiotics and systemic steroid therapy for a diagnosis of cryptogenic organizing pneumonia. The steroid therapy was complicated by 51 pounds of weight gain. She had conversational as well as profound exertional shortness of breath. Physical examination revealed a woman in moderate distress and bilateral diffuse wheezing and rhonchi. Computed tomography of the chest revealed areas of bronchocentric consolidation and bronchial wall thickening in the bilateral lower lobes. She underwent surgical lung biopsy and the histopathology was consistent with chronic aspiration pneumonia.

Rare case of parathyroid gland sarcoidosis presenting with hypercalcaemia.

Sarcoidosis of the parathyroid gland is a rare occurrence. Parathyroid sarcoidosis is usually associated with parathyroid adenomas, and, therefore, hypercalcaemia is a common presentation of this entity. We present a case of parathyroid sarcoidosis and review the world literature regarding this rare condition. A woman with a history of diffuse large B cell lymphoma underwent a surveillance positron emission tomography scan that showed increased fluorodeoxyglucose uptake in multiple thoracic and abdominal lymph nodes and in a left upper extremity soft tissue mass. Biopsy of the soft tissue mass showed non-caseating granulomas consistent with sarcoidosis. Blood work showed a serum calcium of 11.1 mg/dL with an intact serum parathyroid hormone of 92 pg/dL. Primary hyperparathyroidism was suspected. A neck ultrasound and sestamibi parathyroid scintigraphy demonstrated a parathyroid nodule. She underwent surgical resection, and the histopathology revealed a parathyroid adenoma and non-caseating granulomata consistent with a diagnosis of sarcoidosis.

ANCA associated vasculitis: experience of a tertiary care referral center / Vasculite associada a ANCA: experiência de um centro de referência de atendimento terciário

ABSTRACT Background and objectives: Anti-neutrophil cytoplasmic autoantibodies (ANCA) associated vasculitis is a small vessel vasculitis with insufficient epidemiologic estimates in the United States. We aimed to determine demographic and clinical features of ANCA associated vasculitis patients presenting to a large tertiary care referral center in Upstate New York. Design, setting, participants, and measurements: A retrospective analysis of cases with pauci-immune GN on renal biopsy and clinical diagnosis of ANCA vasculitis presenting over 11 years was conducted. Outcomes of interest were: demographics, ANCA antibody positivity, patient and renal survival, and regional trends. Results: 986 biopsies were reviewed, 41 cases met the criteria for inclusion: 18 GPA, 19 MPA, and 4 double positive (anti-GBM disease plus ANCA vasculitis). Mean age at presentation was 52.4 years (SD 23.7), 23 (56%) were male and median creatinine was 2.6 mg/dL. The median patient follow up was 77 weeks (IQR 10 - 263 weeks), with a 3-month mortality rate of 5.7% and a 1-year estimated mortality rate of 12%. Thirteen patients required hemodialysis at the time of diagnosis; 7 patients came off dialysis, with median time to renal recovery of 4.86 weeks (IQR 1.57 - 23.85 weeks). C-ANCA positivity (p < 0.001) and C-ANCA plus PR3 antibody pairing (p = 0.005) was statistically significant in GPA versus MPA. P-ANCA positivity was observed in MPA versus GPA (p = 0.02) and double positive versus GPA (p = 0.002), with P-ANCA and MPO antibody pairing in MPA versus GPA (p = 0.044). Thirty-seven of the 41 cases were referred locally, 16 cases were from within a 15-mile radius of Albany, Schenectady, and Saratoga counties. Conclusions: ANCA vasculitis is associated with end stage renal disease and increased mortality. Our study suggests the possibility of higher regional incidence of pauci-immune GN in Upstate New York. Further studies should investigate the causes of clustering of cases to specific regions.

RESUMO Introdução e objetivos: A vasculite associada a anticorpos anticitoplasma de neutrófilo (ANCA) é uma vasculite de pequenos vasos com estimativas epidemiológicas insuficientes nos Estados Unidos. Nosso objetivo foi determinar características demográficas e clínicas de pacientes com vasculite associada à ANCA, apresentando-se a um grande centro de referência de atendimento terciário em Upstate New York. Formato, cenário, participantes e medidas: Foi realizada uma análise retrospectiva dos casos de GN pauci-imune em biópsias renais e diagnóstico clínico de vasculite ANCA por mais de 11 anos. Os resultados de interesse foram: dados demográficos, positividade de anticorpos ANCA, sobrevidas renal e de pacientes e tendências regionais. Resultados: 986 biópsias foram revisadas, 41 casos preencheram os critérios de inclusão: 18 GPA, 19 PAM, e 4 duplo-positivos (doença anti-MBG com vasculite ANCA). A média de idade na apresentação foi de 52,4 anos (DP 23,7), 23 (56%) eram do sexo masculino e mediana de creatinina de 2,6 mg/dL. O acompanhamento mediano dos pacientes foi de 77 semanas (IQR 10 - 263 semanas), com uma taxa de mortalidade de 3 meses de 5,7% e uma taxa de mortalidade estimada em 1 ano de 12%. Treze pacientes necessitaram de hemodiálise no momento do diagnóstico; 7 pacientes saíram da diálise, com tempo médio para recuperação renal de 4,86 semanas (IQR 1,57 - 23,85 semanas). A positividade para C-ANCA (p < 0,001) e o pareamento de anticorpos C-ANCA mais PR3 (p = 0,005) foram estatisticamente significantes em GPA versus PAM. A positividade de P-ANCA foi observada em PAM versus GPA (p = 0,02) e duplo positivo versus GPA (p = 0,002), com pareamento de anticorpos P-ANCA e MPO em PAM versus GPA (p = 0,044). Trinta e sete dos 41 casos foram encaminhados localmente, 16 casos foram de dentro de um raio de 15 milhas dos condados de Albany, Schenectady e Saratoga. Conclusões: A vasculite por ANCA está associada à doença renal terminal e aumento da mortalidade. Nosso estudo sugere a possibilidade de maior incidência regional de GN pauci-imune no norte do estado de Nova York. Novos estudos devem investigar as causas do acúmulo de casos em regiões específicas.

Coexistent Takayasu arteritis and sarcoidosis: a case report and review of the literature.

BACKGROUND: Takayasu Arteritis (TAK) is a granulomatous large vessel vasculitis that predominantly affects the aorta, major aortic branches and pulmonary arteries resulting in pulselessness. Sarcoidosis is a systemic granulomatous disease of unknown etiology that can affect any organ. Numerous cases of coexistence of both these rare diseases have been described, suggesting that their association may be by more than chance alone. OBJECTIVE: To describe a case of coexistent TAK and sarcoidosis and review the world literature concerning this condition. METHODS: The clinical presentation and diagnostic approach is described of a woman with TAK who developed sarcoidosis. The world literature was reviewed by searching the PubMed and Google Scholar database for the terms 'Takayasu arteritis' and 'sarcoidosis'; 'Takayasu arteritis' and 'granuloma'; 'vasculitis' and 'sarcoidosis'; and 'vasculitis' and 'granuloma.' The identified individual articles were reviewed, and the bibliography of these articles were scrutinized to identify more cases. The pertinent clinical features of these cases were summarized. RESULT: A 36-year-old Caucasian woman, who was diagnosed with histologically confirmed TAK at 22 years of age, was referred for evaluation of mediastinal lymphadenopathy. The diagnosis of sarcoidosis was established on histopathology of a mediastinal lymph node biopsy. A literature review identified 23 additional cases of coexisting sarcoidosis and TAK, and the clinical features of these cases is described. CONCLUSION: TAK and sarcoidosis may occur in the same patient. Given the prevalence of these diseases, concomitant development of these two diseases is unlikely to be by chance alone and probably reflects a unifying mechanism. Clinicians should be aware of this association in patients in order to make a timely diagnosis and optimize patient care.

ANCA associated vasculitis: experience of a tertiary care referral center.

BACKGROUND AND OBJECTIVES: Anti-neutrophil cytoplasmic autoantibodies (ANCA) associated vasculitis is a small vessel vasculitis with insufficient epidemiologic estimates in the United States. We aimed to determine demographic and clinical features of ANCA associated vasculitis patients presenting to a large tertiary care referral center in Upstate New York. Design, setting, participants, and measurements: A retrospective analysis of cases with pauci-immune GN on renal biopsy and clinical diagnosis of ANCA vasculitis presenting over 11 years was conducted. Outcomes of interest were: demographics, ANCA antibody positivity, patient and renal survival, and regional trends. RESULTS: 986 biopsies were reviewed, 41 cases met the criteria for inclusion: 18 GPA, 19 MPA, and 4 double positive (anti-GBM disease plus ANCA vasculitis). Mean age at presentation was 52.4 years (SD 23.7), 23 (56%) were male and median creatinine was 2.6 mg/dL. The median patient follow up was 77 weeks (IQR 10 - 263 weeks), with a 3-month mortality rate of 5.7% and a 1-year estimated mortality rate of 12%. Thirteen patients required hemodialysis at the time of diagnosis; 7 patients came off dialysis, with median time to renal recovery of 4.86 weeks (IQR 1.57 - 23.85 weeks). C-ANCA positivity (p < 0.001) and C-ANCA plus PR3 antibody pairing (p = 0.005) was statistically significant in GPA versus MPA. P-ANCA positivity was observed in MPA versus GPA (p = 0.02) and double positive versus GPA (p = 0.002), with P-ANCA and MPO antibody pairing in MPA versus GPA (p = 0.044). Thirty-seven of the 41 cases were referred locally, 16 cases were from within a 15-mile radius of Albany, Schenectady, and Saratoga counties. CONCLUSIONS: ANCA vasculitis is associated with end stage renal disease and increased mortality. Our study suggests the possibility of higher regional incidence of pauci-immune GN in Upstate New York. Further studies should investigate the causes of clustering of cases to specific regions.

Microscopic ileitis in diverted and nondiverted enteric segments: an underrecognized condition with a multifactorial etiology.

Microscopic ileitis has been infrequently reported in the literature with the few reported cases usually associated with concurrent microscopic colitis. Having encountered a case of collagenous ileitis involving the diverted distal limb of a loop ileostomy and sparing the proximal limb, we examined additional cases of loop ileostomy, end ileostomy, colostomy, and the accompanying diverted colorectal segment for features of microscopic ileitis and colitis. A total of 101 cases of diverted and nondiverted enteric segments were examined from 37 loop ileostomies, 16 end ileostomies, and 12 colostomies status post-Hartmann's procedure. The patients' clinical histories, including demographics and risk factors for microscopic colitis, were obtained from electronic medical records. The index case and an additional case showed collagenous ileitis: the former in the diverted distal limb, and the latter in the nondiverted proximal limb of the loop ileostomy. The latter was associated with high ileostomy output with watery diarrhea. Two additional cases showed lymphocytic ileitis: one in the nondiverted proximal limb of loop ileostomy and the other in the end ileostomy. All 4 patients had one or more risk factors for microscopic colitis. The etiology of microscopic ileitis seems to be multifactorial, and microscopic ileitis may be underdiagnosed. The diverted enteric segment may be involved by microscopic enteritis, suggesting that additional factors other than fecal stasis and altered bacterial flora may be contributing to its pathogenesis. When microscopic ileitis is encountered, identifying associated risk factors, recognizing incipient clinical symptoms of microscopic colitis, and considering other associated diseases or conditions are warranted.

The role of serum amyloid A staining of granulomatous tissues for the diagnosis of sarcoidosis.

BACKGROUND: Previous studies demonstrated that SAA staining of sarcoidosis granulomas was qualitatively and quantitatively different from other granulomatous diseases. These data suggest that positive SAA staining of granulomatous tissue may have adequate specificity to establish a diagnosis of sarcoidosis. Our objective was to determine the diagnostic specificity of SAA staining for sarcoidosis relative to other granulomatous disorders. METHODS: Pathological specimens demonstrating granulomatous inflammation were retrospectively identified at one institution, plus 4 specimens were obtained from New York City firefighters with biopsy-confirmed World Trade Center "sarcoidosis-like" pulmonary disease. Specimens were analyzed if specific diagnoses related to the granulomatous inflammation were confirmed through medical record review. SAA staining was performed using previously developed methods. Two pathologists, blinded to each other and the diagnoses, determined if the stained material was SAA positive or negative. Discordant results were adjudicated by the two pathologists. MEASUREMENTS AND MAIN RESULTS: 106 specimens were analyzed from 100 patients, with 36 biopsies (34%) from sarcoidosis tissues and 70 (66%) from other granulomatous disorders. The Cohen Kappa correlation between the two pathologists for SAA staining positivity was excellent (0.85, 0.73-0.98). The overall specificity of SAA staining for the diagnosis of sarcoidosis was 84% (59/70). The sensitivity was 44% (16/36). CONCLUSIONS: Although SAA staining of various granulomatous tissues was fairly specific for the diagnosis of sarcoidosis, the specificity was inadequate for SAA staining to be used as a diagnostic test for sarcoidosis in isolation. These data suggest that SAA production may not be a universal mechanism in the development of sarcoidosis.

JC polyomavirus nephropathy, a rare cause of transplant dysfunction: Case report and review of literature.

JC polyomavirus-associated nephropathy (JC-PVAN) is a rare but challenging cause of renal dysfunction. We report JC-PVAN in a renal allograft recipient and highlight the obstacles in definitive diagnosis of this disease entity. A deceased-donor renal transplant recipient was diagnosed with JC polyomavirus nephritis 4 years after transplantation. Immunosuppressive agents were subsequently reduced, resulting in an initial stabilization of renal function. We present this interesting case and discuss the challenges with diagnosing and treating this rare entity.

Sunitinib-Induced Acute Interstitial Nephritis in a Thrombocytopenic Renal Cell Cancer Patient.

Sunitinib, a multitargeted tyrosine kinase inhibitor (TKI), is currently the standard of care for patients with metastatic renal cell carcinoma. Renal adverse events associated with sunitinib include proteinuria, renal insufficiency secondary to focal segmental glomerulosclerosis (FSGS), and thrombotic microangiopathy. We describe the second reported instance of biopsy-proven sunitinib-induced acute interstitial nephritis (AIN), in a challenging case complicated by thrombocytopenia. The case illustrates the importance of early diagnosis and intervention in ensuring long-term recovery from renal complications. Four other cases of AIN reported along with inhibition of the vascular endothelial growth factor (VEGF) by either TKI (sunitinib and sorafenib) or antibodies (bevacizumab) suggest a possible class effect. Given our experience, we recommend monitoring renal function with VEGF inhibition, and in the case of renal failure in the setting of an unclear diagnosis, we recommend prompt biopsy.