Improvement of overlapping hidradenitis suppurativa and ankylosing spondylitis after the introduction of adalimumab.

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.

Clinical features of the SAPHO syndrome and their role in choosing the therapeutic approach: report of four patients and review of the literature.

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.

Eosinophilia-myalgia syndrome induced by excessive L-tryptophan intake from cashew nuts.

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.

Fever of unknown origin: large vessel vasculitis diagnosed by PET/CT.

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.

Aortic root vasculitis associated with Cogan's syndrome.

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.

Propylthiouracil-induced anti-neutrophil cytoplasmic antibodies (ANCA) skin vasculitis: the first case reported in Croatia.

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).

Rare zoonosis (hemotrophic mycoplasma infection) in a newly diagnosed systemic lupus erythematosus patient followed by a Nocardia asteroides pneumonia.

Systemic lupus erythematosus (SLE) is per se a disease characterized by suppressed immune response and thus susceptibility to various opportunistic infections. We describe the case of a 21-year old woman who developed a rare zoonosis - hemotrophic mycoplasma infection in the initial stage of SLE, complicated with Nocardia asteroides pneumonia afterwards. Nocardia infection coincided with initiation of glucocorticoids and cyclophosphamide therapy for SLE. After the treatment she recovered completely. To our knowledge the only case of human hemoplasmosis (then referred to as eperythrozoonosis) in medical literature was the one described by a group of Croatian authors 22 years ago. No cases of a hemotrophic mycoplasma infection in a SLE patient have been published up to now.

Subcutaneous sarcoidosis of the face.

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.

Rare zoonosis (hemotrophic mycoplasma infection) in a newly diagnosed systemic lupus erythematosus patient followed by a Nocardia asteroides pneumonia

Systemic lupus erythematosus (SLE) is per se a disease characterized by suppressed immune response and thus susceptibility to various opportunistic infections. We describe the case of a 21-yearold woman who developed a rare zoonosis - hemotrophic mycoplasma infection in the initial stage of SLE, complicated with Nocardia asteroides pneumonia afterwards. Nocardia infection coincided with initiation of glucocorticoids and cyclophosphamide therapy for SLE. After the treatment she recovered completely. To our knowledge the only case of human hemoplasmosis (then referred to as eperythrozoonosis) in medical literature was the one described by a group of Croatian authors 22 years ago. No cases of a hemotrophic mycoplasma infection in a SLE patient have been published up to now.

[The history of systemic lupus erythematosus]. / Povijest sistemskog eritemskog lupusa.

In this review paper the history of systemic lupus erythematosus is presented. History of systemic lupus is summarized in three periods, the classical period of discovering the visible manifestations of the disease, the neoclassical period discovering the concept of systemic disease, and modern times, which provided insight into disease mechanisms and facilitated the formation of objective diagnostic approach.

Non-MS autoimmune demyelination.

Connective tissue diseases can be characterised by central nervous system (CNS) involvement, in some patients manifested by demyelination areas in the white matter of the brain and spinal cord, which are difficult to differentiate from multiple sclerosis (MS) and other demyelinating processes, such as transverse myelitis and optic neuritis. Demyelinating process may be the feature of nervous impairment in systemic lupus erythematosus, Behcet's disease (BD), Sjoegren's syndrome (SS), systemic sclerosis (SSc) or very rarely other systemic autoimmune diseases. An acute isolated neurological syndrome, as the most common symptom of MS can sometimes be the only feature or even first manifestation of nervous impairment in connective tissue disease, hence presenting the diagnostic problem. Although the white matter abnormalities seen by magnetic resonance imaging may be similar in non-MS autoimmune demyelination and MS, it is the most important diagnostic tool in the differential diagnosis of the mentioned conditions. Investigating the presence of various autoantibodies potentially involved in the pathogenesis of demyelinating lesions as well as cerebrospinal fluid (CSF) analysis can be helpful.

[Cryptococcal meningitis as a diagnostic problem in a patient with SLE--case report]. / Kriptokokni meningitis kao dijagnosticki problem u bolesnika sa sustavnim eritemskim lupusom- prikaz bolesnika.

Systemic erythematosus lupus (SLE) is a disease with wide range of clinical manifestations, signs and symptoms. Disease outcome depends mostly on the affection of kidneys and central nervous system by the disease. Very important cause of death in patients with SLE is infection. Infections are very common among these patients due to aggressive immunosuppressive treatment that is needed for the disease inflammatory activity control. In this case report we have presented a patient with SLE who initially had severe renal affection, but also complications of immunosuppressive therapy that was administered. Even though the disease was accidentally diagnosed, it had a severe clinical progress. Because of lupus nephropathy, in the early phase of the disease we administered aggressive immunosuppressive therapy (combined parenteral therapy of glucocorticoides and cyclophosphamide). As an outcome of the combined effect of disease and immunosuppressive agents used in the treatment of the disease, the patient had increased infective diathesis (repeated infections caused by S. enteritidis--urinary infections and sepsis). During one of the disease flares the patient was hospitalized an opportunistic infection developed. It was meningitis caused by C. neoformans. This opportunistic mycosis infection presented with clinically totally nonspecific signs and symptoms of CNS affection. Therefore, we suspected affection of CNS with SLE. Even though all diagnostic procedures were made on time and that adequate antifungal and supportive agents were applied very early after the infection onset, the outcome was fatal. Because of infective diathesis in patients with SLE, which present with common and opportunistic infections, and due to high mortality rates caused by these infections, we have tried to emphasise the importance of taking adequate specimens early after infection outcome for these rare infective agents like C. neophormans. In recent medical literature are dominant cases reported in Asia. Reports from Europe are very rare, and this case is the one of that kind in Croatia.

[Renal lupus]. / Lupus nefritis renal lupus.

The nephritis in systemic lupus erythematosus is presented.

[Hip in inflammatory rheumatic diseases]. / Upalne reumatske bolesti i promjene kuka.

The hip changes in inflammatory rheumatic diseases are presented.

[Rheumatoid arthritis and pregnancy]. / Reumatoidni artritis i trudnoca.

The problems of pregnancy in rheumatoid arthritis are presented in this paper.

[Myopathy with rhabdomyolysis as an adverse effect of simultaneous treatment with cerivastatin and gemfibrozil]. / Miopatija s rabdomiolizom kao nuspojava istodobnog lijecenja cerivastatinom i gemfibrozilom.

Drug-induced myopathy and/or rhabdomyolysis have been reported with use of some statins and fibric acid derivatives. The risk of adverse effects is increased with their concomitant use. We report the case of myopathy with rhabdomyolysis during the concomitant use of cerivastatin and gemfibrozil.