Case Report: Systemic Sclerosis After Covid-19 Infection.

The coronavirus disease (COVID-19) is a respiratory tract infection caused by the new virus SARS-CoV-2. The acute phase of the infection may in certain individuals be followed by another longer phase of disease (long COVID) of unknown etiology probably associated in certain cases with autoimmune activation. It has been shown that COVID-19 can trigger autoantibody production and in genetically predisposed patients may cause the onset or exacerbation of autoimmune diseases. We are reporting a case of mild COVID-19 infection complicated by autoantibody production and cutaneous and gastrointestinal symptoms and subsequently diagnosed with systemic sclerosis (SSc). A 47-year-old man with no history of any autoimmune diseases and in good health became sick together with his family on the 12th of November with mild symptoms: tiredness, fever, cough, and sore throat. Oropharyngeal swab for SARS-CoV-2 tested positive. He was isolated at home and did not require hospitalization. Three weeks later he presented with clinical manifestation compatible with suspicion of SSc. He briefly presented with skin rush, periorbital edema and conjunctivitis, vomiting, dysphagia, burning sensation in the skin, above all in the fingertips and around the mouth, puffy fingers, Raynaud's phenomenon, pain at the fingertip of the middle finger where a depressed area was noticed without a clear ulceration. ANA showed a strongly positive nucleolar pattern. Anti-PM/Scl 75 and PM/Scl 100 resulted positive. High-resolution computed tomography (HCRT) showed early stage of interstitial lung disease (ILD). The patient was diagnosed with SSc based on the persistence of autoantibodies and the clinical and radiological pictures according to the ACR/EULAR classification (scores: puffy finger, 2; ILD, 2; Raynaud's phenomenon, 3; SSc related antibodies, 3; total 10). There are several cases described in the medical literature of possible new onset of SLE after COVID-19 infection. This is the first case that describes a possible new onset of SSc. Conclusion: SARS-CoV-2 may trigger systemic sclerosis.

Case of primary Sjogren's syndrome preceded by dystonia.

There are only six cases in literature that describe development of dystonia with Sjogren's syndrome (SS). We describe a case of a 43-year-old woman who presented with symptoms including movement disorder, sensory neurogenic bladder, sensory loss and neuropathic pain, migraine like headaches, musculoskeletal pain, Raynaud's phenomenon and dysautonomia. Symptoms started in 2000, with weakness that progressed to dystonia in 2003. Diagnostic work-up was inconclusive with negative inflammatory serologies, cerebrospinal fluid and MRI for many years. After patient developed sicca syndrome with dry eyes and mouth in 2009, her rheumatoid factor titre was elevated (550 IU/mL), erythrocyte sedimentation rate, anti-Sjogrens syndrome-related antigen A (anti-Ro/SSA) and anti-SSB/La: anti-Sjogrens syndrome-related antigen B (anti-La/SSB) became positive. Lip biopsy confirmed diagnosis of SS. She was diagnosed with primary SS with neurological involvement. Her symptoms responded well to intravenous methylprednisolone. Symptoms stabilised with trials of immune-suppressive therapy. This is a case that demonstrates the delay of diagnosing SS with preceding unique neurological association.

[Systemic scleroderma in children. Apropos of 5 cases. A review of the literature]. / Sclérodermie systémique de l'enfant. A propos de 5 observations. Revue de la littérature.

Systemic scleroderma is a rare disease in childhood. 62 cases are analyzed. Cutaneous manifestations are identical to those seen in adults. However Raynaud phenomenon is much more frequently missing but follow-up of patients is only of 4 years' duration. We want to draw attention on possible worsening of clinical signs during intercurrent infectious episodes. We report exceptional roentgenological bone anomalies. Gastro-intestinal tract is frequently involved, particularly the oesophagus. We want to draw attention on latent small intestine involvement. A normal thoracic X-ray examination cannot rule out involvement of the lungs; systematic respiratory functional tests are absolutely necessary. All parts of the cardiac wall can be involved: we underline the particular seriousness of this involvement as it was exclusively responsible of death in 10 cases out of 21. Renal involvement is rare. We are reporting 2 cases where a staturo-ponderal retardation remains unexplained; 7 other cases in the literature report on isolated weight retardation. Biological anomalies are similar to those reported in adult. Treatment is not well-codified; we can hope that a better understanding of the disease's physiopathology will lead to the discovery of an efficient therapy.

[Occupational Raynaud's phenomenon in a dentist: a case report]. / Fenomeno di Raynaud occupazionale in dentista: presentazione di un caso.

After more than 39 years of practice, a dentist showed Raynaud's phenomenon of the hand exposed to vibrations generated by dental instruments. Other possible occupational, pharmacological and systemic disease factors that could cause Raynaud's phenomenon were excluded. Since it has been reported in the literature that dentists and dental technicians could exceed the permissible limits for vibrations, an occupational etiology of this case is suggested on the basis of logical reasoning.

Dermatologic manifestations of Sjögren syndrome.

BACKGROUND: Sjögren syndrome (SS) is a chronic autoimmune inflammatory disease that involves primarily the exocrine glands, resulting in their functional impairment. SS typically presents as dry eyes (xerophthalmia) and dry mouth (xerostomia). This process can manifest either as the independent phenomenon of primary SS or as secondary SS when found in the context of another autoimmune process, most commonly rheumatoid arthritis or systemic lupus erythematosus. Nearly half of the patients with SS develop cutaneous manifestations, which may include dry skin (xeroderma), palpable and nonpalpable purpura, and/or urticaria-like lesions. These cutaneous manifestations have been underemphasized because they are often overshadowed by the more prominent sicca symptoms. However, certain skin findings are of paramount clinical and prognostic importance as they confer an increased risk for the development of life-threatening conditions, including multisystem vasculitis and non-Hodgkin lymphoma.OBJECTIVE AND CONCLUSIONS:In this review, the cutaneous manifestations of primary SS are discussed, with an emphasis on those findings that portend an increased risk of mortality.

Primary Sjogren's syndrome with tuberculous arthritis of left knee.

A 56-year-old woman presented with recurrent pain and swelling of left knee, Raynaud's phenomenon and dry mouth. She was initially diagnosed with primary Sjögren's syndrome and was put on prednisone, which substantially relieved her complaints. But 8 months later, pain and swelling of left knee recurred with spiking fever, chills and shortness of breath. Escalation of prednisone did not improve the pain and swelling of left knee. CT of chest revealed pulmonary interstitial changes with coexisting infection. MRI of left knee was highly consistent with tuberculous arthritis, which was further confirmed by positive blood culture of Mycobacterium tuberculosis and acid-fast stain of synovial fluid.

Interferon-gamma in the treatment of systemic sclerosis: a randomized controlled multicentre trial.

We report the results of a randomized controlled multicentre study on interferon-gamma (IFN-gamma) treatment of systemic sclerosis as determined by skin sclerosis, renal and other organ involvement, global assessment, subjective symptoms and quality of life. Forty-four patients were enrolled into the trial, 27 in the treatment group and 17 in the control group. All patients presented with type I or type II scleroderma. Twenty-nine patients (64%) finished the study. The mean duration of Raynaud's phenomenon and skin sclerosis was 15.3 and 10.8 years, respectively. The skin scores tended to improve in the treatment group (P > 0.05). Mouth aperture increased significantly from 38.5 to 47.7 mm in the treatment group (P < 0.001). Subanalysis of IFN-gamma treated patients with normalized skin sclerosis scores >/=1 showed significant improvement in both skin involvement and subjective symptoms (P < 0.05). Organ involvement improved in eight of 18 treatment patients and in three of 11 control patients. It worsened in three of 18 treatment patients and in four of 11 control patients. One control patient died due to cardiorespiratory failure during the study. No deterioration of renal function occurred during IFN-gamma treatment. There was a significant improvement in quality of life parameters in the control group but not in the treatment group. Plasma levels of neopterin increased significantly during IFN-gamma treatment but not in the control group, whereas N-terminal procollagen III peptide levels did not change in either group. There was a high frequency of mild to moderate influenza-like adverse events during IFN-gamma treatment. Only four of nine drop-out patients, however, experienced symptoms most probably associated with IFN-gamma treatment. We conclude that IFN-gamma therapy has mild beneficial effects on skin sclerosis and disease-associated symptoms in type I and II scleroderma. IFN-gamma treatment was associated with acceptable tolerability and did not induce major renal dysfunction in our patients.

O que o cirurgião-dentista deve sobre o síndrome de Raynaud / What the dentist must knows concerning Raynaud's syndrome

A síndrome, fenômeno ou doença de Raynaud, embora de patogênese desconhecida, caracteriza-se por ser uma desordem vaso espástica definida por quadro isquêmico transitório causado, principalmente,pela vaso constrição das artérias digitais, pré-capilares e plexo artériovenoso subcutâneo. As manifestações clínicas ocorrem principalmente nas mãos (dedos), porém, pode ocorrer em pés, ponta do nariz, língua e orelha, sendo que o órgão afetado pode apresentar como características, a pele branca e isquêmica, com duração variável, seguida de fase cianótica e, posteriormente, hiperêmica. O fenômeno é particularmente importante porque pode representar uma indicação de desenvolvimento futuro (média de 10,4 anos a partir do aparecimento da doença de Raynaud) de doenças secundárias como esclerose sistêmica, artrite reumatóide, dermatopolimiosite, lúpuseritematoso sistêmico, síndrome de Sjögren, entre outras. O caso relatado é de paciente do sexo feminino, 32 anos, leucoderma, com a classificação mais alta na escala de Taylor-Pelmear (estágio: 4; graduação: muito severa). A anamnese e exame físico revelaram a condição com evolução de 3 anos, com sinais e sintomas clássicos. A paciente apresentava importantes cicatrizes nas extremidades digitais de ambas as mãos, referente a quadros severos de isquemia. Exames hematológicos e sorológicos mostraram-se dentro dos limites normais e a paciente não apresentava indícios de doença secundária, entretanto, a observação em longo prazo é imperativa