Kikuchi-Fujimoto Disease With Systemic Lupus Erythematosus and Systemic Sclerosis Overlap: A Unique Clinical Presentation.

Kikuchi-Fujimoto Disease (KFD), or histiocytic necrotizing lymphadenitis (HNL), is a rare self-limiting disorder presenting with fever and swollen lymph nodes. It is characterized by the focal proliferation of reticular cells, the presence of nuclear debris, and histiocytes. In advanced cases, it can present with hepato-splenomegaly and generalized lymphadenopathy. Historically, it has been associated with viral infections, as it frequently was found to be associated with upper respiratory symptoms. Alternative explanations include the immune response of T-cells leading to alteration in CD8-positive T-cell-mediated cell apoptosis. It is also speculated that KFD can be associated with rheumatological autoimmune diseases. We present a case of a 21-year-old African American female with a known diagnosis of systemic lupus erythematosus (SLE)-systemic sclerosis (SS) overlap presented with febrile lymphadenopathy and was diagnosed to have HNL on histological exam of lymph node biopsy.

A Cavitary Lesion in a Patient with Antineutrophilic Cytoplasmic Antibody (ANCA) Associated Vasculitis: A Case Report and Review of the Literature.

Patients with antineutrophilic cytoplasmic antibody (ANCA) associated vasculitis who were on immunosuppressive therapy with corticosteroids may be susceptible to cavitary lesions.1 Only a few cases have been reported in the literature to date. Immunosuppression was shown to improve prognosis in patients with vasculitis. However, adverse therapy events and the risk of opportunistic infections become a major cause of morbidity and mortality in this specific patient population. We present a case of a 75-year-old female who was diagnosed and treated in our hospital for ANCA-associated vasculitis and returned within a few weeks of medical therapy and was found to have developed cavitation concerning for worsening vasculitis or an opportunistic fungal infection or combination of both. Given the risk of severe complications from opportunistic fungal infections, close monitoring and prophylactic antifungal therapy should be considered. Further studies are needed to evaluate the benefit of prophylaxis in this patient population.

Tattoo-Associated Sarcoidosis With Severe Uveitis Successfully Treated With Mycophenolate Mofetil: A Report of Two Cases.

Tattooing is an increasing trend among Western countries, with about 18% of the population undergoing the procedure once in their lifetime. The process looks simple; introduce exogenous pigment into the dermis layer of the skin, altering the skin color permanently. However, this simple procedure leads to several health issues and medical complications, both acute and chronic, and some are difficult to cure. Sarcoidosis is high on the list of severity involving almost all body organs. Multiple organ involvement makes this condition more difficult to treat. Lungs and lymphatics are the leading sites of involvement, followed by an inflammatory disease of the eye called uveitis. An additional problem is the limited confirmatory diagnostic tests and treatment options for sarcoidosis. Each patient must be considered unique based on their age, clinical presentation, and severity of involvement. Proper treatment must be tailored for better outcomes with minimum side effects and rapid cure. Here we describe two case reports of tattoo-associated sarcoidosis with severe uveitis successfully treated with mycophenolate mofetil.

A Case of Rapidly Proliferative Glomerulonephritis Secondary to Syphilis That Responded to Treatment With Penicillin.

The involvement of kidneys in syphilis has been reported in the literature with the majority of cases presenting with nephrotic-range proteinuria. We report a case of rapidly proliferative glomerulonephritis in a patient with secondary syphilis. A 40-year-old male with a history of human immunodeficiency virus (HIV), chronic hepatitis B virus, and chronic kidney disease stage 2 presented with fatigue, anorexia, weight loss, arthralgia, chills, and rash throughout the body. The patient was non-compliant with HIV medication and had unprotected sexual intercourse. Labs showed blood urea nitrogen of 57 mg/dL (range: 7-23 mg/dL), creatinine 8.2 mg/dL (range: 0.5-1.3 mg/dL), and high titers of rapid plasma reagin. The biopsy showed crescentic glomerulonephritis with c3 deposition in mesangium and basement membrane. The patient responded to treatment with penicillin therapy with gradual improvement in kidney function.

Macrophage Activation Syndrome Upon Initiation of Adalimumab in a Patient With Longstanding Rheumatoid Arthritis.

Macrophage activation syndrome (MAS) is a subset of hemophagocytic lymphohistiocytosis (HLH) described in patients with rheumatological disorders. Some triggers of MAS and HLH include infection, malignancy, rheumatological disease, HIV, and rarely medications such as immunosuppressants. In recent medical literature, biologic agents are increasingly recognized as a potential trigger, but the mechanism behind this remains poorly understood. We describe the case of a patient who developed MAS after initiating adalimumab and propose a potential pathophysiological link between biologics and this syndrome.

Intestinal Pseudo-Obstruction as the Initial Clinical Presentation in Systemic Lupus Erythematosus: A Rare and Severe Disorder.

Intestinal pseudo-obstruction (IPO) is a rarely recognized complication of systemic lupus erythematosus (SLE). We present a 36-year-old African American female, with only known past medical history of anemia, admitted for frequent vomiting, abdominal distension, abdominal pain, diarrhea, and fever that had been ongoing for 5 days. Laboratory results revealed leukopenia and thrombocytopenia. Imaging revealed dilated small bowel loops, abdominal ascites, as well as mild bilateral hydroureteronephrosis without obstructing calculus. Serologic testing confirmed a diagnosis of SLE. The patient was placed on immunosuppressive therapy and responded well. IPO has previously been described as a rare finding in patients with SLE, with bilateral hydroureteronephrosis and lupus interstitial cystitis having been noted as common concomitant factors. One must have a high level of suspicion to recognize it as being one of the initial clinical presentations. Early recognition and appropriate management preclude unnecessary invasive procedures that do not take into account the pathophysiology of the condition and allow for appropriate management and return of peristaltic function.

Sustained Remission of Lupus Panniculitis Treated With Hydroxychloroquine in a Patient With Crohn's Disease: A Case Report.

Extraintestinal manifestations (EIM) in inflammatory bowel disease (IBD) are common including cutaneous manifestations that either precede or follow manifestations of IBD. Cutaneous manifestations of IBD include erythema nodosum, pyoderma gangrenosum, oral lesions, and Sweet's syndrome. Cutaneous manifestations of IBD tend to recur and extensive cases may require maintenance management with immunomodulators or biologics. However, the complications and adverse effects of long-term therapy with immunosuppressive agents are numerous and need to be considered before their initiation. We report a case of a Crohn's disease patient with recurrent and debilitating cutaneous manifestation of lupus panniculitis that had sustained remission with hydroxychloroquine.

Monckeberg Medial Calcific Sclerosis of the Temporal Artery Masquerading as Giant Cell Arteritis: Case Reports and Literature Review.

Monckeberg medial calcific sclerosis (MCS) is an infrequent finding in the temporal artery and can clinically present almost indistinguishably from giant cell arteritis (GCA). To our knowledge, there have been only two case reports of suspected GCA found to be MCS only after a temporal artery biopsy (TAB). Herein, we present two cases. The first case is a 69-year-old female with hypertension, type-2 diabetes mellitus, and chronic headaches who presented with left temporal headaches and scalp tenderness. She had a prominently dilated, tortuous, and tender left temporal artery. Initial labs showed a leukocyte count of 11.1x103/L, erythrocyte sedimentation rate (ESR) of 29 mm/hr, and C-reactive protein (CRP) of 5.8 mg/L. The patient was started on prednisone 60 mg for presumptive GCA. Left TAB was negative for inflammatory changes, with findings consistent with MCS. Steroids were discontinued, and symptoms resolved. The second case is a 67-year-old male with hypertension, asthma, hyperlipidemia, status-post left eye cataract phacoemulsification, with intraocular lens insertion one-month prior, who presented with left eye blurriness in the inferior visual field and intermittent headache for 15 days. Left ophthalmoscopy showed retinal pallor and edema. Initial labs revealed ESR of 25 mm/hr, CRP of 11.2 mg/L, leukocyte count of 13.01x103/L. The patient was given solumedrol 120 mg once and prednisone 70 mg daily for presumptive GCA. Left TAB was negative for GCA but reported damaged elastic fibers by calcification consistent with MCS. Partial visual blurriness remained, and steroids were discontinued. This report accentuates the importance of MCS as a temporal GCA simulator. Physicians should be aware that TAB potentially changes management and may help surface underlying conditions.

A Case of Atorvastatin-Associated Necrotizing Autoimmune Myopathy, Mimicking Idiopathic Polymyositis.

Statin-induced necrotizing autoimmune myopathy (SINAM) is a rare side effect of statin use which manifests as progressive muscle weakness. Because statins are a widely prescribed medication for coronary artery disease, hyperlipidemia, and many other diseases, many patients are at risk of developing SINAM or one of the many other statin-induced myopathies. Due to identification of an antibody specific to this disease, we were able to diagnose SINAM in a patient whose symptoms had progressed to the extent that they were debilitating. Our case describes SINAM in a patient undergoing treatment with a statin for an extended period of time, diagnosis of the disease process, treatment, and resolution of symptoms.

Drug-Induced Lupus Erythematosus Associated with Antiretroviral Therapy in a Patient with Human Immunodeficiency Virus: A Case Report.

Antiretroviral medications are the mainstay of human immunodeficiency virus (HIV) therapy and some have been in use for over 20 years. To date, there have been no reported cases of antiretroviral therapy (ART) induced drug-induced lupus erythematosus (DILE). We present a case of a 35-year-old woman who received a combination of emtricitabine, rilpivirine, and tenofovir disoproxil fumarate for HIV treatment. Three years later, she developed an extensive rash and polyarthralgia in her extremities with laboratory findings significant for positive antinuclear antibody (ANA), anti-double stranded deoxyribonucleic acid (DNA) antibody (anti-dsDNA), and anti-histone antibody titers. Her systemic symptoms and rash improved with ART discontinuation. She was later restarted on her original ART due to difficulty in tolerating a different combination therapy. A few months after restarting ART, she developed new dermatologic symptoms, worsening arthralgias, tenderness of the metacarpophalangeal and proximal interphalangeal joints of the hand, and an increase in anti-dsDNA titers to 286 IU/ml. ART was then discontinued, which led to complete resolution of her symptoms and her anti-dsDNA decreased significantly. She had no further recurrence of symptoms. Awareness of the possibility of ART-induced DILE in the right clinical setting would prompt early recognition and management of this condition.

A Case of Systemic Lupus Erythematosus Presenting as Guillain-Barré Syndrome.

Systemic lupus erythematosus (SLE) is an autoimmune systemic disease with multiple organ involvement with high morbidity and mortality rate. Among the severe potential fatal complications are those of the central and peripheral nervous system which usually develop during the course of the disease and very rarely from the outset of the disease. We are reporting a rare case of Miller-Fisher (MFS) variant of Guillain-Barré syndrome (GBS) as the first manifestation of SLE in a 41-year-old female who progressed to flaccid paralysis with no neurological improvement with initial immunosuppressive therapy, plasmapheresis, and first cycle of intravenous immunoglobulin (IVIG) but with remarkable and complete recovery after the second 5-day course of IVIG.

Mixed Connective Tissue Disease and Papillary Thyroid Cancer: A Case Report.

BACKGROUND: Mixed connective tissue disease (MCTD) is a connective tissue disorder characterized by high titers of distinct antibodies: U1 ribonucleoprotein with clinical features seen in systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), dermatomyositis (DM), polymyositis, and scleroderma. The association of SLE and DM with various cancers of the thyroid has been reported in the literature. However, there have been no reports associating MCTD with thyroid cancer. CASE REPORT: We present a 58-year-old woman diagnosed with MCTD with co-morbid interstitial lung disease that has remained stable for 10 years, who developed papillary thyroid carcinoma (PTC) 10 years after initial diagnosis. CONCLUSIONS: We theorize that: 1) MCTD may have been a primary diagnosis complicated by PTC, or 2) MCTD may have been an initial presentation of paraneoplastic syndrome of silent PTC, because her symptoms of MCTD significantly improved after total thyroidectomy. To the best of our knowledge, this is the first case report to associate MCTD with PTC. It highlights the importance of maintaining a high index of suspicion for thyroid malignancy in MCTD patients.

Non-Systemic Vasculitic Neuropathy: An Enigmatic Clinical Entity.

BACKGROUND: Non-systemic vasculitic peripheral neuropathy is a rare condition characterized by necrotizing inflammation resulting in luminal narrowing of the vasa nervorum, leading to ischemic injury to peripheral nerves. Here, we present the case of 63-year-old woman with subacute onset of severe hyperesthesia of the lower extremities accompanied by foot drop. CASE REPORT: A 63-year-old woman with prolonged history of uncontrolled diabetes mellitus presented with subacute onset of severe bilateral lower extremity hyperesthesia and motor weakness along with left-sided foot drop. She had multiple emergency room visits with no relief of her symptoms. High doses of analgesics were insufficient to control pain. Laboratory tests were positive only for high erythrocyte sedimentation rate and C-reactive protein. A skin biopsy obtained 5 cm above the left lateral malleolus revealed medium-sized dermal vasculitis with dense mononuclear infiltrate. Electromyography showed peripheral neuropathy. A nerve biopsy was needed to reveal the exact diagnosis. CONCLUSIONS: Diagnosis of non-systemic vasculitic peripheral neuropathy can be delayed or missed in patients with uncontrolled diabetes mellitus, leading to significant morbidity. Elevated markers of inflammation in the absence of a possible explanation should prompt the clinician to perform a nerve biopsy; however, it is an invasive procedure and is associated with complications of post-neuropathic pain and delayed wound healing. Magnetic resonance angiography of the lower limbs, if combined with skin biopsy, can save the patient from undergoing nerve biopsy.

Rheumatoid arthritis-associated corneal ulceration with superimposed infection by methicillin-resistant Staphylococcus aureus: a complicated type of corneal melt.

BACKGROUND: Severe extra-articular manifestations of rheumatoid arthritis usually occur in advanced stages of the disease. In particular, ocular involvement may lead to inflammatory corneal ulceration, in which therapy is challenging owing to its association with systemic vasculitis. Close collaboration between ophthalmologists and rheumatologists is paramount in providing the best treatment approach in this sight-threatening condition. CASE REPORT: We present a case of seropositive rheumatoid arthritis associated with corneal melting in the absence of other typical clinical manifestations of rheumatoid arthritis flare. The rheumatoid arthritis-associated corneal ulcer was complicated in our case by concomitant infection with methicillin-resistant Staphylococcus aureus, which was treated with intravenous vancomycin after an initial antimicrobial ophthalmic solution proved not to be making adequate improvement in the corneal healing. The recurrent corneal melting appeared to be aggravated by the ophthalmic infection while on immunosuppressive regimen. CONCLUSIONS: In patients on biologic agents, intravenous antibiotics must be considered in addition to ophthalmic eye solution in controlling the infectious process. Excluding concomitant ophthalmic infection is equally important before initiation of high-dose steroid and immunosuppressive regimens.

Lung sarcoidosis in etanercept treated rheumatoid arthritis patient: a case report and review of the literature.

We report a 55-year-old female with seropositive rheumatoid arthritis for 10 years who developed large mediastinal and hilar adenopathy while receiving etanercept therapy. Chest high resolution computed tomography (HRCT) showed mediastinal lymph nodes with size of 2.3 × 3.1 centimeters. Right paratracheal lymph node biopsy showed nonnecrotizing epithelioid granulomata. All infectious studies of pulmonary lymph node tissues were negative. Etanercept was discontinued. Follow-up HRCT 6 months later showed resolution of mediastinal lymph nodes. This report should increase awareness of pulmonary sarcoidosis development in patient treated with tumor necrosis factor-alpha blocking agent, etanercept.

A case of granulomatosis with polyangiitis causing hydroureter and hydronephrosis.

Granulomatosis with Polyangiitis (GPA, formerly known as Wegener) is a systemic vasculitis characterized by granulomatous involving upper and lower respiratory tract and can also cause necrotizing glomerulonephritis (Umemoto et al. 2012 and Takala et al. 2011). GPA is associated with antineutrophil cytoplasmic autoantibodies (ANCA) against serine proteinase 3 (PR3) (Takala et al. 2011, Dufour et al. 2012, and Berthoux et al. 2011). This disease usually starts with involvement of the upper and lower respiratory tracts and also can involve kidney, eyes, skin, central and peripheral nervous systems, and gastrointestinal tract (Umemoto et al. 2012, Takala et al. 2011, and Berthoux et al. 2011). We describe a case of GPA that presented with abdominal pain. Computed tomography (CT) scan with contrast showed right sided moderate hydronephrosis and hydroureter, to the level of the right common iliac artery. There was also mural segmental thickening in common iliac artery which was thought to be the cause of the ureteral obstruction and hydronephrosis. Our case shows that mural segmental thickening in common iliac artery happened due to GPA and caused hydronephrosis. In addition, most of the cases with hydronephrosis due to GPA went through urology intervention such as stent placement but in our case hydronephrosis resolved with medical management.

[Vitamin D levels in ankylosing spondylitis: does deficiency correspond to disease activity?]. / Níveis de vitamina D na espondilite anquilosante: a deficiência corresponde à atividade da doença?

Ankylosing spondylitis (AS) is an inflammatory disorder that presents with arthritis of the axial skeleton, including sacroiliac joints. Vitamin D is a secosteroid hormone with a long-established role in calcium and phosphate homeostasis, and in the regulation of bone formation and resorption. It is now known that vitamin D plays an immunosuppressive role in the body, and there is interest of late in the role of vitamin D in autoimmune diseases. Inflammation may be responsible for some of the loss of bone mineral density seen in AS. We reviewed the literature for studies assessing vitamin D level as a marker of AS disease activity and those examining vitamin D levels in AS in comparison to healthy controls. Four of 7 studies found a significant negative correlation between vitamin D levels and Bath Ankylosing Spondylitis Index (BASDAI), erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). In a review of 8 case-control studies, the mean level of 25-hydroxyvitamin D3 was 22.8 ± 14.1 ng/mL in 555 AS patients versus 26.6 ± 12.5 ng/mL in 557 healthy controls. When compared with a 2-sample t test, vitamin D levels were significantly higher in healthy controls (p < 0.01). We conclude that patients with AS appear to have lower vitamin D levels versus healthy controls; however, the cause is unclear. Existing studies do not demonstrate a consistent link between vitamin D levels and disease activity in AS. Further studies are in need to determine if a causative link exists between vitamin D deficiency and AS.

Rare case of idiopathic lymphocytic interstitial pneumonia exhibits good response to Mycophenolate Mofetil.

Lymphoid interstitial pneumonia (LIP) is a poorly understood lymphoproliferative disorder that can be idiopathic or associated with different conditions. LIP is considered to be steroid responsive. However, its response is mainly unpredictable with a high mortality rate. Here we describe a rare case of idiopathic LIP that shows clinical improvement to Mycophenolate Mofetil after intolerable side effects of high-dose steroids.

Middle cerebral artery involvement in systemic lupus erythematosus presenting as a stroke--an unusual initial presentation of the disease.

Systemic lupus erythematosus (SLE) disproportionately affects the African American population and usually presents with a constellation of symptoms. Along with hematologic, musculoskeletal, serous and skin involvement, the most common causes of morbidity are attributed to altered blood rheology causing accelerated atherosclerotic vascular disease (ASVD). ASVD occurs in predominantly premenopausal women at an age where ASVD is rare or uncommon. Classical central nervous system manifestations are very rare from the outset of the disease. Here we present a 44-year old African American woman with newly diagnosed SLE and no significant atherosclerotic risk factors, who presented initially with symptoms of subacute stroke.