Temporal arteritis in the young

Temporal arteritis in the form of giant cell arteritis (GCA) is common in the elderly but is extremely rare in patients less than 50 years of age. We describe two male patients: one who presented at the age of 31 years with painful, nodular swellings of both temporal arteries and whose temporal artery biopsy demonstrated a non-giant cell panarteritis with mixed inflammatory cell infiltrate typical of juvenile temporal arteritis (JTA); another one, aged 40 years, who presented with headache and cerebral angiography consistent with an intracranial vasculitis and whose temporal artery biopsy confirmed an authentic multinucleated GCA. The first patient spontaneously improved after biopsy and the second patient has responded well to corticosteroid therapy. These two cases exemplify well two distinct but extremely rare forms of temporal arteritis in young patients. A 3rd subset is that associated with a systemic vasculitis. Few cases of JTA have been reported and, to our knowledge, we describe in this report one of the only cases of GCA with central nervous system involvement in the young.     

Headache caused by giant cell arteritis

Opinion statement Giant cell arteritis (GCA) is the most common primary systemic vasculitis in older adults. Patients usually are older than 50 years and have an erythrocyte sedimentation rate (Westergren) greater than 50 mm/h. Headache is a common symptom, occurring in approximately 90% of patients. However, the most serious complications of GCA, blindness and stroke, may occur in the absence of headache. Nonspecific constitutional symptoms such as weight loss, fever, and malaise may dominate the clinical presentation. Currently, corticosteroids are the mainstay of therapy for GCA. Treatment is initiated at 0.7 to 1 mg/kg mg of prednisone (or equivalent) per day as soon as the diagnosis is suspected. The medication is tapered based on laboratory parameters and symptoms. Relapse is common, especially during the first year of therapy. Side effects from steroids in the elderly are common and often serious. Steroid resistance (manifesting as continued high dose requirements after 3 to 6 months) may complicate therapy and place patients at increased risk of side effects. Methotrexate and azathioprine have been used as steroid-sparing agents based on anecdotal evidence. More recently, evidence is emerging that antitumor necrosis factor-α agents may be efficacious and act as steroid-sparing agents. New-onset headache or worsening headache in a patient older than 50 years should raise the possibility of GCA and appropriate therapeutic and diagnostic measures should be begun promptly.     

Cryptococcal myositis and vasculitis: an unusual necrotizing soft tissue infection

BACKGROUND: Cryptococcus neoformans var. neoformans is an opportunistic yeast that typically infects immunocompromised patients. METHODS: A case report and review of the pertinent English-language literature are presented. RESULTS: Necrotizing vasculitis associated with cryptococcal invasion was identified in 1986. Until now, only 24 cases of cryptococcal cellulitis have been reported, including one case of cryptococcal necrotizing fasciitis and one case of necrotizing vasculitis. We report an unusual case of occult disseminated cryptococcosis presenting as necrotizing cellulitis, fasciitis, and myositis. CONCLUSIONS: Cryptococcal soft tissue infection serves as a marker of disseminated cryptococcosis in immunocompromised hosts. Owing to its rarity as a cause of soft tissue infections, diagnosis is difficult and mortality is high.     

Innumerable bilateral pulmonary arteriovenous malformations: percutaneous transcatheter embolization using amplatzer vascular plug

We report an unusual and distinct case of innumerable bilateral pulmonary arteriovenous malformation (PAVM) in a 42-year-old patient presenting with significant right-to-left shunt resulting in severe dyspnea and with a history of stroke due to paradoxical embolization related to his PAVM. Because it was impossible to treat all his PAVMs, our aim was to treat those with a feeding artery measuring 3 mm or more in diameter, aiming to improve the patient's dyspnea and decrease the risk of paradoxical embolization. We safely and successfully embolized 8 of the patient's PAVMs, using a single Amplatzer vascular plug for each PAVM. The procedure did not result in significant improvement of the patient's symptoms. In similar cases with innumerable PAVMs, the expectations of symptom relief should be lowered and the patient should be informed that treatment will only offer protection from paradoxical embolization. To our knowledge, there are no similar cases previously described in the literature.     

Vasculitis working group: Selected unanswered questions related to giant cell arteritis and anti-neutrophil cytoplasmic antibody-associated vasculitis

Evidence to guide assessment and management of patients with vasculitis is lacking for many important clinical questions. The evidence surrounding several common questions about management of vasculitis was reviewed. Patients with giant cell arteritis (GCA) are at risk for developing extra-cranial large vessel inflammation. Clinicians should be aware of this complication and search for large vessel involvement in patients with GCA who have ischemic symptoms. Research is needed to define optimal strategies to identify patients with such complications. Because of the hazards of chronic corticosteroid use, alternative therapies for patients with GCA have been sought but thus far no clear alternatives have been identified. Anti-neutrophil cytoplasmic antibodies (ANCA) are associated with small-vessel vasculitis, including Wegener's granulomatosis and microscopic polyangiitis, but changes in ANCA titers should not be used as a surrogate biomarker for disease activity. Several immunosuppressive agents can be used for maintenance therapy after induction of remission in patients with ANCA-associated vasculitis, with no firm evidence that one agent is superior to others. Collectively, this review shows that more research is needed to provide a firmer body of evidence to support clinical decision-making for patients with vasculitis.     

Amaurosis fugax

This is an overview of publications concerning the risk of amaurosis fugax (AF) as a sign of cerebrovascular insufficiency and the risk of cerebral infarction in patients presenting with this symptom. AF is characterized by a sudden, painless, monocular loss of vision that typically lasts between 1 and 15 min. The patient frequently describes his vision as “dark” or “dim” and, generally, vision returns to normal immediatly after such an attack. The most common causes of AF are embolism, hemodynamics, or vasospasm. In 66% of cases the source of embolism was not detectable, in 27% it was likely to be caused by a carotid artery lesion, whereas a cardioembolic event was likely to be the source in only 2%. Most recent studies show that in patients with severe carotid stenosis (70% – 99%) and AF as the first presenting symptom, there is a 16% risk of stroke within a period of 2 years. This stroke risk is lower than in patients with transient ischemic attack as the presenting symptom, who are at a risk of 43% of stroke within 2 years. The risk of early mortality in older patients with AF is three to five times higher than in age-matched controls, with myocardial infarction as the major cause of death. In conclusion, the occurrence of AF carries a considerable risk of cerebral stroke and AF is a marker of generalized vascular disease.     

Psoriatic arthritis in two patients with an inadequate response to treatment with tocilizumab

Psoriatic arthritis (PsA) is considered as one of the seronegative spondylarthropathies. Like rheumatoid arthritis (RA), the increased production of interleukin (IL)-6 suggests a pathogenic role of IL-6 in PsA. However, whether humanized anti-IL-6 receptor antibody such as tocilizumab (TCZ) might be effective for PsA as well as RA has yet to be determined. We report herein two cases of PsA treated using TCZ. Although, TCZ treatment resulted in disappearance of serum CRP in both patients, arthritis and skin lesions were not improved despite 6-month administration of TCZ. In contrast, tumor necrosis factor (TNF) inhibitor proved effective against arthritis and skin lesions in these patients. Collectively, these findings not only indicate that IL-6 has distinct pathological roles in RA and PsA, but also suggest that TNF inhibitor therapy (but not TCZ) is effective for arthritis and skin lesions of PsA.     

Correlates for nocturia: a review of epidemiological studies

Traditionally, nocturia was considered to be one of the symptoms of prostatism. However, this symptom has unusual characteristics compared with other lower urinary tract symptoms; for example, there is only a mild benefit for nocturia from treatment for benign prostatic hyperplasia. In the past decade, nocturia has been investigated as a separate symptom in epidemiological studies. These types of studies have shown various correlates for nocturia other than benign prostatic hyperplasia, as well as the prevalence of this symptom. These correlates include age, race/ethnicity, medical problems (such as hypertension, diabetes and stroke) psychological aspects, tasting habits, quality of life and even mortality. The cause-and-effect associations are not always obvious between nocturia and its correlates. In the present article, the author attempts to comprehensively review the correlates for nocturia shown by epidemiological studies.     

Tocilizumab Treatment for Nephrotic Syndrome Due to Amyloidosis in Behcet’s Disease

Renal involvement is an unusual but significant Behcet´s disease (BD) complication and AA amyloidosis appears to be the most common etiology. IL-6 is a pro-inflammatory cytokine with an important role in AA amyloidosis development. Tocilizumab (TCZ) is a humanized anti-IL-6 receptor antibody that has emerged as an effective and specific treatment in AA amyloidosis secondary to chronic inflammatory disorders. We report on a patient diagnosed with BD who developed nephrotic syndrome caused by renal AA amyloidosis with an excellent response to TCZ therapy.     

Spontaneous non-aneurysmal rupture of the renal pedicle artery due to acute vasculitis in polyartheritis nodosa--with survival.

The first published case is presented of survival following a ruptured renal artery due to acute vasculitis in polyarteritis nodosa (PAN). Spontaneous, massive retroperitoneal hemorrhage was the presenting symptom in this case. Emergency nephrectmoy was carried out as a life-saving procedure. Postoperative studies revealed that only the kidneys and joints were involved in the disease.     

Acute hemiballismus after carotid endarterectomy--a case report.

A 72-year-old woman who experienced a postoperative stroke after a carotid endarterectomy with the presenting symptom of hemiballismus is described. This unusual presentation was likely the result of a hypotensive episode coupled with a predisposing anatomic variant in the circle of Willis, which compromised blood flow in the posterior cerebral circulation. She responded well to treatment with haloperidol with complete resolution of hemiballistic movements.     

Intestinal obstruction caused by splenic volvulus: Report of a case

Torsion of a wandering spleen is rare, usually presenting as acute abdomen, and is commonly misdiagnosed. In special cases, ultrasonography, arteriography, and additional scintigraphy are extremely valuable in the preoperative diagnostic management. We herein present an unusual case of torsion of a wandering spleen in a 19-year-old female. The presenting symptom was acute gastrointestinal obstruction due to pressure of the enlarged and ptotic spleen in the pelvis. The intestinal obstruction with signs of peritonitis made a laparatomy with removal of the infarcted spleen imperative. After the operation, the patient made a complete recovery.     

Polyarteritis nodosa, presenting as life-threatening gastrointestinal hemorrhage in a liver transplant recipient.

This unique case reports the first recorded episode in the medical literature of vasculitis post-liver transplantation, presenting as life-threatening gastrointestinal hemorrhage. A 52-year-old Caucasian woman underwent orthotopic liver transplantation (OLT) for autoimmune cirrhosis complicated by hepatoma and portal vein thrombosis. Late hepatic artery thrombosis led to a second liver graft. Following recovery from an episode of acute rejection, the patient presented with large volume hematemesis, melena, and hemochezia (passage of fresh blood from the rectum). Following upper and lower gastrointestinal endoscopy and surgery, angiography illustrated the presence of polyarteritis nodosa (PAN), which was successfully treated with high-dose steroid therapy. Gastrointestinal hemorrhage is an unusual presentation of vasculitis, especially PAN. The occurrence of this phenomenon post-OLT, in the presence of immunosuppression is previously unreported.     

Kienb?ck's disease: two unusual cases

Two unusual cases of Kienb?ck's disease are reported. The first is of a young woman whose presenting feature was spontaneous rupture of the flexor pollicis longus tendon. This is the first reported case in which such a rupture was the presenting feature of the condition. The second is of a 71-year-old woman with scleroderma and Raynaud's disease who did not develop her Kienb?ck's disease until late in life. The literature contains no older patient with this condition and a likely association is made between the avascular necrosis and the vasculitis associated with this connective tissue disorder.     

Prostatic involvement in Wegener's granulomatosis

Two cases of Wegener's granulomatosis presenting with prostatic involvement are described and compiled with the five previously detailed cases. Each of these patients presented with obstructive symptoms, proteinuria, leukocyturia, and hematuria. The urinary sediment normalized with treatment of the underlying granulomatous vasculitis. Wegener's granulomatosis is a rare cause of prostatic obstructive symptoms, but should be considered whenever the relatively unusual entity of granulomatous prostatitis is diagnosed. One patient was initially treated exclusively with trimethoprim-sulfamethoxazole (TMP-SMX). He responded, but noted recurrence during the 15th month of treatment. We also report on this patient's antineutrophil cytoplasmic antibody (ANCA) titers, which correlated with clinical assessment and predicted recurrence 2 months before elevation of the Westergren sedimentation rate (WSR) and clinical diagnosis.     

Infected Postcarotid Endarterectomy Pseudoaneurysms: Retrospective Review of a Series

The development of infected pseudoaneurysms (PAs) following carotid endarterectomy (CEA) is extremely rare. We retrospectively reviewed the data from 13 such aneurysms (in 12 patients) repaired over a period of 35 years at the Texas Heart Institute (THI) and included an overview of published cases to analyze the epidemiology, mode of presentation, methods of repair, and outcome. The diagnosis of PA should be considered when a patient develops a persistent hematoma, recurrent bleeding from the wound, or late wound infection. Sepsis is occasionally a presenting symptom. Surgical therapy for infected PAs can be challenging; excision of the aneurysm followed by autologous grafting constitutes the favored approach. Traditionally, Dacron patch repair has been associated with a high incidence of reinfection. Carotid artery ligation is required in a large percentage of cases (22.7%) and is associated with a prohibitive rate of death/major stroke (50%) compared with a low (12%) risk following arterial reconstruction. Prevention and early diagnosis of infected PAs are essential to limit complications and mortality.     

Crusted Norwegian scabies, an opportunistic infection, with tocilizumab in rheumatoid arthritis

Background

Crusted Norwegian scabies is an extremely rare hyperkeratotic variant of scabies infestation. We report herein a case of crusted scabies in a woman with severe rheumatoid arthritis (RA) treated by tocilizumab (TCZ), a monoclonal antibody blocking the interleukin-6 receptor.

Observation

An 80-year-old female with severe RA developed erythroderma followed by hyperkeratosis, widespread scaling over the trunk, arms, hands and limbs, and crusted lesions on her scalp. She was on TCZ (8 mg/kg per month) with prednisone (20 mg/d). Several hypotheses were evoked (i.e., vasculitis, psoriasis or paraneoplasic syndrome) but a microscopic examination of the skin surprisingly demonstrated numerous scabies mites and eggs leading to the diagnosis of crusted (Norwegian) scabies. After repeated use of ivermectin and application of topical piperonyl butoxide cream, scabies disappeared. However, there had been outbreak of scabies among two individuals in the staff and two patients. TCZ was stopped after four infusions because of a severe infectious pneumonia and failure.

Conclusion

Crusted scabies should be evoked in case of pruritus or erythroderma occurring under TCZ therapy and it may be explained by the interleukin-6 blockade.     

IgA glomerulonephritis associated with microscopic polyangiitis or Churg-Strauss syndrome.

When renal insufficiency occurs in classical antineutrophil cytoplasm antibody- (ANCA) associated vasculitides, histological examination usually finds pauci-immune focal segmental glomerulonephritis. We report on 2 cases of histologically proven necrotizing vasculitis associated with IgA nephropathy. Concomitant vasculitis and IgA nephropathy has only rarely been reported but this joint occurrence may not be coincidental as its pathophysiology is not known. Among vasculitides, IgA nephropathy has more frequently been associated with Henoch-Schoenlein purpura: one microscopic polyangiitis unusual because the patient simultaneously presented ANCA and microaneurysms, and the other Churg-Strauss syndrome associated with mild renal insufficiency. This uncommon association might represent a possible overlap syndrome between these ANCA-associated vasculitides and IgA nephropathy or simply a new type of glomerulonephritis that must be taken into account in these vasculitides.     

AL amyloidosis with temporal artery involvement simulates giant-cell arteritis

Light-chain (AL) amyloidosis may present with features suggesting vasculitis, including giant-cell arteritis (GCA). We describe a case of an 80-year-old man, who initially presented with bilateral jaw claudication, bi-temporal headache and arthralgia, however a temporal-artery biopsy then revealed AL amyloidosis. A diagnosis of AL amyloidosis complicating multiple myelome simulates GCA and polymyalgia rheumatica was established. The patient was successfully treated with melphalan and dexamethasone: the free kappa light chains decreased, the patient's jaw claudication and headache disappeared. Then we discuss similarities between GCA and AL amyloidosis and potential confusion in diagnosis. We suggest that, in patients with clinical features of GCA without any temporal-artery typical findings, specimens are stained with Congo red, which then results in a different diagnosis and treatment.     

Squamous cell carcinoma arising in a giant condyloma acuminatum (Buschke-Lowenstein tumour)

Giant condyloma acuminatum (GCA) is a tumour that primarily affects the genital and perianal areas. Despite the histologically benign appearance, it behaves in a malignant fashion, destroying adjacent tissues, and is regarded as an entity intermediate between an ordinary condyloma acuminatum and squamous cell carcinoma. Primary anorectal lesions account for only a small number of GCA cases and, as with squamous cell carcinoma, the human papilloma virus is the causative agent. The hallmark of GCA is the high rate of local recurrence and transformation into squamous cell carcinoma. We describe a case of GCA complicated by malignant transformation, where locoregional control was achieved with combined chemoradiotherapy.