ObjectiveSystemic lupus erythematosus (SLE) can affect virtually any organ and can thus be a predisposing factor for secondary complicating conditions. The aim is to present a case of a severe ...multiorgan SLE complicated by tuberculosis in a young woman.MethodsA 42-year-old woman with a known history of SLE was referred for a rheumatological consultation due to deterioration of her general condition. Her medical history had started ten years earlier with thrombocytopenia in HELLP syndrome and preeclampsia in late pregnancy. Four years later she developed pancytopenia, with elevated anticardiolipin levels. Antiphospholipid syndrome and SLE were suspected and she was started on prednisone, azathioprine, and hydroxychloroquine. A year later she suffered an ischemic stroke and developed epilepsy as a sequela. After another three years, she suffered an ST-elevation myocardial infarction and underwent coronary intervention, and two years later, she had an identical episode. Echocardiography showed mitral regurgitation due to Libman-Sacks endocarditis. One year later she was hospitalized due to the development of pulmonary tuberculosis, was treated with antimycobacterial treatment, and dismissed after they were discontinued.ResultsShe had been lost to rheumatological follow-up for three years. At the visit, she complained of poor general condition. She had noticeable hyperpigmentation of the face and forehead. The remaining physical examination was unremarkable. Chest X-ray showed several infiltrates and hilar lymphadenopathy. Workup was scheduled, including sputum cultures, thorax CT, echocardiography, cardiological consultation, and rheumatology blood profile. Workup results confirmed an exacerbation of pulmonary tuberculosis. Antituberculotics were reintroduced. At the latest follow-up her primary disease and infectious complications remain under control.ConclusionsSystemic lupus erythematosus flares are serious and infrequently fatal. Discerning between them and secondary complications can be challenging, particularly if follow-up is inadequate.
Hidradenitis suppurativa is a chronic inflammatory disorder characterized by occlusion of the follicular pilosebaceous units of the skin. The treatment options are sometimes very limited and ...unpleasant odor and abundant drainage complicate the disease. Ankylosing spondylitis is a form of seronegative spondyloarthritis with predominantly axial but also peripheral joint involvement. Both of the conditions lower the patient's quality of life and affect everyday activities. We describe a 39-year-old male patient with both diseases treated with different medications with only a modest result. After the initiation of a tumor necrosis factor α (TNF-α) inhibitor (adalimumab) the patient experienced first the musculoskeletal and later on the skin improvement. The introduction of TNF-α inhibitors should be considered early in the treatment of overlapping hidradenitis suppurativa and the spondyloarthritis spectrum of conditions. Available medical data confirm the positive results and beneficial effect on disease course, activity and, most importantly, quality of life.
Eosinophilia is characterized by more than 0.5
x 10
eosinophils per liter in the full blood count. A wide
range of conditions, from asthma to parasitic infections,
autoimmune diseases, and certain ...forms of cancer, have
been known to trigger abnormally high amount of eosinophils.
It is essential to reach the correct diagnosis and
treat the underlying disease aggresively. Definition of the
eosinophilia-myalgia syndrome was offered in 1980s by
Centers for Disease Control and Prevention for surveillance
purposes, and criteria were revised in 2001, with
high specificity.
We report a case of 59-year old female who started a
special weight-reducing diet regimen that included excessive
cashew nut ingestion. Several months after she has
presented with periferal blood eosinophilia and constitutional
symptoms. Detailed work-up has not found elements
for haematological, systemic autoimmune, neoplastic or
infectious disease. She was diagnosed with eosinophilia-
myalgia syndrome due to extreme L-tryptophan intake,
a compound found in the cashew nut’s oil. She responded
well to cashew nut withdrawal and steroid therapy. In the
follow-up period she remained stable with normal eosinophil
count and there was not a need for any specific
therapy
PET/CT is starting to play an important role in evaluating fever of unknown origin (FUO), due to its ability to localize and delineate areas of high metabolic activity, such as neoplastic ...proliferation and inflammation, including vasculitis. We present a case of giant cell arteritis (GCA) in a 72-year-old female patient admitted to our department with a 4-month history of FUO, weight loss and fatigue, without specific symptoms or signs. Laboratory investigations suggested acute phase response, with a pronounced erythrocyte sedimentation rate, high CRP level and microcytic anemia. A thorough diagnostic evaluation was performed to exclude an unknown primary tumor, which was initially suspected due to a positive family history of cancer. Surprisingly, PET/CT revealed large vessel vasculitis affecting the ascending, descending and abdominal aorta, as well as subclavian, proximal brachial and carotid arteries bilaterally. Biopsy of the superficial temporal artery confirmed the diagnosis of GCA. Treatment with methylprednisolone and azathioprine led to resolution of clinical symptoms and normalization of laboratory parameters. In addition to the use of PET/CT in the evaluation of FUO, its value as a method complementary to temporal artery biopsy is also discussed.
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EMUNI, FIS, FZAB, GEOZS, GIS, IJS, IMTLJ, KILJ, KISLJ, MFDPS, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, SBMB, SBNM, UKNU, UL, UM, UPUK, VKSCE, ZAGLJ
Eosinophilia is characterized by more than 0.5 × 109 eosinophils per liter in the full blood count. A wide range of conditions, from asthma to parasitic infections, autoimmune diseases, and certain ...forms of cancer, have been known to trigger abnormally high amount of eosinophils. It is essential to reach the correct diagnosis and treat the underlying disease aggresively. Definition of the eosinophilia-myalgia syndrome was offered in 1980s by Centers for Disease Control and Prevention for surveillance purposes, and criteria were revised in 2001, with high specificity. We report a case of 59-year old female who started a special weight-reducing diet regimen that included excessive cashew nut ingestion. Several months after she has presented with periferal blood eosinophilia and constitutional symptoms. Detailed work-up has not found elements for haematological, systemic autoimmune, neoplastic or infectious disease. She was diagnosed with eosinophilia-myalgia syndrome due to extreme L-tryptophan intake, a compound found in the cashew nut’s oil. She responded well to cashew nut withdrawal and steroid therapy. In the follow-up period she remained stable with normal eosinophil count and there was not a need for any specific therapy.
Although the SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis) syndrome was defined as a distinct entity more than 20 years ago, its classification within the spectrum of inflammatory ...rheumatic diseases and the proper therapeutic approach are still a matter of debate. We present four patients diagnosed with the SAPHO syndrome treated and followed-up in our Department, demonstrating the diversity of their clinical courses and their responses to different therapeutic approaches. We also review the clinical, laboratory, and imaging features of the SAPHO syndrome described in the relevant literature. Despite the growing quantity of published data on the clinical features of the syndrome and the recognition of two disease patterns (inflammatory and bone remodeling disease), it is still not clear whether these possible disease subsets require different therapeutic strategies. Tumor necrosis factor-alpha (TNF-α) inhibitors have been suggested to be effective in patients with the inflammatory pattern, whereas bisphosphonates seem to be effective in patients with bone remodeling disease; however, this is still a hypothesis not yet confirmed by adequately designed clinical studies. Further research is needed to assess disease features predicting favorable response to the two therapeutic modalities beyond the first line of therapy - TNF-α inhibitors and bisphosphonates.
Subcutaneous Sarcoidosis of the Face Bosnic, Dubravka; Barešic, Marko; Bagatin, Dinko ...
Internal Medicine,
01/2010, Volume:
49, Issue:
6
Journal Article
Peer reviewed
Open access
Subcutaneous sarcoidosis (previously referred to as Darier-Roussy sarcoidosis) is an unusual and atypical form of sarcoidosis. It is characterized by formation of the noncaseating granulomas in the ...subcutaneous tissue with no other systemic manifestations present. Sometimes subcutaneous granulomas are the first sign of systemic sarcoidosis. We report a case of a 51-year-old woman with an isolated form of subcutaneous sarcoidosis with no apparent sign of any other systemic manifestations. The final diagnosis should be made by excluding all other more common diseases. As spontaneous remissions have been reported, regular follow-ups for the first three to six months are advisable. If the remission does not take place within the given period of time or the symptoms progress rapidly, glucocorticoids are the drugs of choice. After improvement is seen, slow tapering of glucocorticoids is advisable.
Cogan's syndrome is characterized by nonsyphilitic interstitial keratitis and an audiovestibular disorder resembling Meniere disease. We report a patient with progressive congestive heart failure due ...to massive aortic and mitral insufficiency coupled with aortitis leading to an ascending aortic aneurysm. The patient underwent successful aortic root replacement and mitral valve repair.
Drug-induced vasculitis is a known side effect of prolonged treatment with several drugs. It is characterized by inflammation and cellular infiltration of small vessels and presence of ...anti-neutrophil cytoplasmic antibodies (ANCA). Propylthiouracil and hydralazine (anti-thyroid and antihypertensive drugs) are the drugs most commonly associated with drug-induced vasculitis. Small vessels of the skin are most frequently affected, while affection of the vessels of the kidneys, central nervous system and lungs make the diagnosis life-threatening. When drug-induced vasculitis is suspected, quick and punctual diagnostic procedure should be carried out to exclude systemic manifestations. Treatment comprises of elimination of the causative drug, which is sufficient in most cases, but sometimes oral or parenteral glucocorticoids and even immunosuppressants are indicated. A case is presented of an 18-year-old male with a history of Graves disease treated with standard dose of propylthiouracil. Approximately 2.5 years after starting therapy he noticed formation of shallow skin ulcerations on both of his ear lobes and elbows. Detailed hospital work-up found high titers of perinuclear-staining anti-neutrophil cytoplasmic antibodies/myeloperoxidase (pANCA/MPO, 1:1024). Biopsy of the affected skin revealed leukocytoclastic vasculitis. Additional tests excluded systemic vasculitis. The patient was diagnosed with propylthiouracil-induced vasculitis, a form of drug-induced vasculitis. Propylthiouracil was discontinued and the skin lesions disappeared over time without the need of any specific therapy (such as glucocorticoids).
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