Ashima Makol

Pancreatitis in Systemic Lupus Erythematosus: Frequency and Associated Factors — A Review of the Hopkins Lupus Cohort

Objective. Pancreatitis is a rare but potentially life-threatening complication of systemic lupus erythematosus (SLE). Vasculitis of the gastrointestinal tract is the most commonly proposed mechanism. We determined the frequency of SLE-related pancreatitis in the Hopkins Lupus Cohort. Methods. A large prospective cohort of 1811 patients with SLE was reviewed and clinical and laboratory measures of SLE patients who developed pancreatitis were compared to patients who did not develop pancreatitis. Results. Four percent of patients with SLE had pancreatitis due to SLE. The best multivariate model of clinical and laboratory associations included hypertriglyceridemia, psychosis, pleurisy, gastritis, and anemia. Conclusion. Hypertriglyceridemia appears to be a strong associate of pancreatitis in SLE, but antiphospholipid antibodies are not. SLE patients with psychosis and pleurisy are at increased risk for pancreatitis.

Etanercept and venous thromboembolism: a case series

IntroductionThe treatment with antitumor necrosis factor agents has often been associated with the induction of autoantibodies (antinuclear antibodies, anti-double stranded DNA antibodies and antiphospholipid antibodies). The clinical significance of these antibodies remains unclear, but they may predispose to antiphospholipid syndrome with thromboembolic complications. The association of etanercept with thromboembolic events has not been reported previously in the literature.Case presentationWe describe the cases of three patients with rheumatoid arthritis, psoriatic arthritis and seronegative inflammatory arthritis who were treated with etanercept. They developed deep vein thrombosis and/or pulmonary embolism one to three years after the initiation of etanercept therapy. All three patients had a prolonged activated partial thromboplastin time with a positive lupus anticoagulant that persisted even after 12 weeks.ConclusionAlthough the clinical significance of antiphospholipid antibodies during treatment with antitumor necrosis factor agents remains unclear, they may predispose patients to develop antiphospholipid syndrome when associated with prolonged activated partial thromboplastin time, lupus anticoagulant positivity, or the presence of anti-β2 glycoprotein I. Clinicians must keep this in mind during therapy with antitumor necrosis factor agents in order to prevent, detect and treat potential consequences such as deep vein thrombosis and pulmonary embolism.

Predictors of relapse and treatment outcomes in biopsy-proven giant cell arteritis: a retrospective cohort study

OBJECTIVE To evaluate characteristics of relapse, relapse rates, treatment and outcomes among patients with biopsy-proven GCA in a large, single-institution cohort. METHODS We conducted a retrospective review of all patients with biopsy-proven GCA from 1998 to 2013. Demographic, clinical, laboratory and treatment data at presentation and during follow-up were collected. Comparisons by relapse rate were performed using chi-square tests. Prednisone discontinuation by initial oral dose ≤40 and >40 mg/day was compared using Cox models. RESULTS The cohort included 286 patients [74% female, mean age at diagnosis 75.0 years (s.d. 7.6), median follow-up 5.1 years). During follow-up, 73 patients did not relapse, 80 patients had one relapse and 133 had two or more relapses. The first relapse occurred during the first year in 50% of patients, by 2 years in 68% and by 5 years in 79%. More patients with established hypertension (P = 0.007) and diabetes (P = 0.039) at GCA diagnosis were in the high relapse rate group ( ≥ 0.5 relapses/year) and more females were in the low or high relapse groups than in the no relapse group (P = 0.034). Patients receiving an initial oral prednisone dose >40 mg/day were able to reach a dose of <5 mg/day [hazard ratio (HR) 1.46 (95% CI 1.09, 1.96)] and discontinue prednisone [HR 1.56 (95% CI 1.09, 2.23)] sooner than patients receiving ≤40 mg/day without an increase in observed glucocorticoid-associated adverse events. CONCLUSION Females and patients with hypertension or diabetes at GCA diagnosis have more relapses during follow-up. Patients treated with an initial oral prednisone dose >40 mg/day achieved earlier prednisone discontinuation.

Fecal incontinence in women: causes and treatment

Fecal incontinence is a common problem in women, which often enforces life changes owing to embarrassment and social stigma. It is frequently not reported or diagnosed. Age, obstetric trauma, pelvic surgery, chronic diarrhea, obesity and other medical conditions, such as diabetes and stroke, increase the risk of fecal incontinence. Preventive strategies include avoiding diarrheal triggers, discouraging the routine use of episiotomies, early recognition and management of obstetric injuries and possibly pelvic floor muscle exercises after childbirth. Treatment options are available and should be discussed with the patient. These, in order of progression, are education and medications for diarrhea or constipation, supportive care, biofeedback training and surgery.

Adalimumab induced mononeuritis multiplex in a patient with refractory rheumatoid arthritis: a case report

BackgroundAnti tumor necrosis factor agents are a valuable addition to the armamentarium against rheumatoid arthritis but have some serious side effects which clinicians should be aware about.Case presentationWe present a case of a 54 year old Caucasian male with refractory rheumatoid arthritis who developed mononeuritis multiplex four weeks after starting adalimumab therapy.ConclusionComplete resolution of neurological and electromyography findings was seen upon stopping therapy. These agents can be a double-edged sword in the management of rheumatological illnesses. Though they help treat refractory disease, their potential side effects can include mononeuritis multiplex which can be recognized by means of clinical features, electromyography and nerve biopsy as depicted in our case.

Lymphomatoid granulomatosis masquerading as interstitial pneumonia in a 66-year-old man: a case report and review of literature

Lymphomatoid granulomatosis (LG) is a rare, Epstein-Barr virus (EBV)-associated systemic angiodestructive lymphoproliferative disorder that may progress to a diffuse large B cell lymphoma. Pulmonary involvement may mimic other more common lung pathologies including pneumonias. Therapeutic standards have not been established for LG, but rituximab, interferon-α2b (INF-α2b), and chemotherapy have shown to improve symptoms and long term prognosis.We report a case of rapid respiratory deterioration in a 66-year-old man with clinical presentation, chest radiography, pulmonary function testing and high resolution computed tomography (HRCT) findings consistent with idiopathic interstitial pneumonia, but very poor response to antibiotics and low dose steroids. Lung biopsy showed histopathology consistent with LG that was confirmed by a positive in situ hybridization for Epstein - Barr virus encoded RNA (EBER). The patient was treated with rituximab and combination chemotherapy and showed significant initial clinical improvement with gradual resolution of abnormal findings on imaging. However, the patient developed pancytopenia as a complication of chemotherapy and died secondary to septic shock and renal failure that were refractory to medical management. Autopsy showed diffuse alveolar damage but no evidence of any residual LG within the lungs.This case demonstrates that an open lung biopsy or video-assisted thoracoscopic surgical (VATS) biopsy is often necessary to rule out the presence of LG in order to determine the appropriate therapeutic strategy early in the course of illness to improve prognosis.

Articular Involvement in Disseminated Histoplasmosis in a Kidney Transplant Patient Taking Azathioprine

To the Editor: Histoplasmosis can manifest itself in the joints of immunocompromised patients. It is a potentially fatal opportunistic infection and should be considered when these patients present with unexplained joint swelling, erythema nodosum, or sepsis. An 81-year-old farmer from Minnesota developed acute-onset painful swelling over the ulnar aspect of the right palm. He denied work-related injury or trauma. Hand radiographs did not show evidence of a foreign body. Cephalexin was started for presumed cellulitis, but 4 days later the hand lesion evolved into a localized area of purple discoloration, 3 × 2.5 cm, with central clearing. He developed generalized weakness, myalgias, large-joint arthralgias, worsening hand erythema, productive cough, fever (101.2°F), and hypotension (88/59 mm Hg), and was hospitalized for presumed sepsis. He was treated empirically with vancomycin and ceftriaxone. Physical examination showed a nontender, nonfluctuant, nonindurated ecchymotic lesion on the ulnar aspect of his right hand distal to the hypothenar eminence, with full active and passive range of motion of wrist and fingers and no tenderness along the digital tendon sheaths. There was no synovitis in the metacarpophalangeal, interphalangeal, or radiocarpal joints. His right knee and ankle were warm and swollen with limited range of motion but no erythema. He had a small palatal ulcer. His history was pertinent for hypertension, hyperlipidemia, ischemic cardiomyopathy, gout, and degenerative arthritis of the knees. He underwent a cadaveric kidney transplant in 1982 because of endstage renal disease from chronic interstitial nephritis and was maintained on azathioprine 100 mg and prednisone 5 mg daily with excellent allograft function. Surgical history was pertinent for splenectomy in … Address correspondence to Dr. A. Makol, Mayo Clinic, Division of Rheumatology, Department of Internal Medicine, 200 First St. SW, Rochester, MN 55905, USA. E-mail: makol.ashima{at}mayo.edu

An unusual case of autoimmune pancreatitis presenting as pancreatic mass and obstructive jaundice: a case report and review of the literature

BackgroundAutoimmune pancreatitis is a rare chronic inflammatory pancreatic disease that is increasingly being diagnosed worldwide. As a result of overlap in clinical and radiological features, it is often misdiagnosed as pancreatic cancer. We report the case of a patient with autoimmune pancreatitis that was initially misdiagnosed as pancreatic cancer.Case presentationA 31-year-old Caucasian man presented to our hospital with epigastric pain, jaundice and weight loss. His CA 19-9 level was elevated, and computed tomography and endoscopic ultrasound revealed a pancreatic head mass abutting the portal vein. Endoscopic retrograde cholangiopancreaticography showed narrowing of the biliary duct and poor visualization of the pancreatic duct. Fine-needle aspiration biopsy revealed atypical ductal epithelial cells, which raised clinical suspicion of adenocarcinoma. Because of the patients unusual age for the onset of pancreatic cancer and the acuity of his symptoms, he was referred to a tertiary care center for further evaluation. His immunoglobulin G4 antibody level was 365 mg/dL, and repeat computed tomography showed features typical of autoimmune pancreatitis. The patients symptoms resolved with corticosteroid therapy.ConclusionAutoimmune pancreatitis is a rare disease with an excellent response to corticosteroid therapy. Its unique histological appearance and response to corticosteroid therapy can reduce unnecessary surgical procedures. A thorough evaluation by a multidisciplinary team is important in rendering the diagnosis of autoimmune pancreatitis.

Calcinosis Cutis in Systemic Sclerosis

A 74-year-old man presented for evaluation of severe calcinosis cutis that had progressed to involve his fingers, hands, toes, right thorax, and right thigh, resulting in clinically significant functional limitation.

Clinical characteristics of inflammatory ocular disease in anti-neutrophil cytoplasmic antibody associated vasculitis: a retrospective cohort study

Objective To characterize the clinical correlates and outcome of inflammatory ocular disease (IOD) among patients with ANCA-associated vasculitides (AAV). Methods Medical records of potential cases of AAV seen at Mayo Clinic from 2003 to 2013, inclusive, were reviewed to identify confirmed cases meeting the diagnosis of AAV using the Chapel Hill Consensus Conference 2012 descriptors. Records of confirmed cases of AAV were then further reviewed for IOD, and clinical characteristics, treatment and outcomes abstracted. Results A total of 1171 confirmed cases of AAV were identified of which 183 patients (mean age 49.0 years; 51% female; 95% Caucasian) had IOD. The most common manifestation of IOD was injection of the eye (57%) followed by eye pain (46%) and visual acuity loss (18%). Scleritis was the most common type of IOD (22%) followed by episcleritis (21%), orbital inflammation (18%), lacrimal duct stenosis (10%) and uveitis (9%). Oral glucocorticoids were used to treat IOD in the majority of patients (96%). CYC and rituximab were the most frequently used immunosuppressive agents (54 and 36%, respectively). Of those with orbital inflammation, 52% underwent therapeutic surgical intervention. Clinical remission of IOD was achieved in 91% of patients but relapses were seen in 23%. Significant visual acuity loss was observed in only six patients. Conclusion IOD is a common manifestation of AAV and seen in about 16% of patients with AAV. Scleritis, episcleritis and orbital inflammation are the most common subtypes. Most patients respond well to glucocorticoids and immunosuppression, but relapse of IOD is common.